Cervical chordoma. A case report and differential diagnosis

VOLUME 2 - No. 5 - MARCH 15, 2012 CENTAURO S.r.l., BOLOGNAFortnightly ISSN 2239-7493

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AINR - Associazione Italiana di Neuroradiologiaand:The Neuroradiologists of Alpe-AdriaANRS - Albanian Neuroradiological SocietyPANRS - Pan Arab NeuroRadiology SocietyRadiological Society of Saudi Arabia, Division of NeuroradiologyEgyptian Society of NeuroradiologyISNR - Indian Society of NeuroradiologyIndonesian Society of NeuroradiologyNeuroradiology Section of the Radiology Society of IranIsraeli Society of NeuroradiologyCollege of Radiology Malaysia

Neuroradiology Section - Pakistan Psychiatry Research CenterSection of Neuroradiology - Polish Radiological SocietyThe Neuroradiologists of RomaniaSection of Neuroradiology of Serbia and MontenegroSILAN - Sociedad Ibero Latino Americana de NeurorradiologiaNeuroradiology Section of Singapore Radiological SocietySlovenian Society of NeuroradiologyThe Neuroradiological Society of TaiwanTSNR - Turkish Society of Neuroradiology

Vol. 2No. 5

Index RadiologicalFindingsinGliosarcoma. 173

ASingleInstitutionExperienceM.Y. Swaidan, M. Hussaini, I. Sultan, A. Mansour

DiagnosticAbilityofFluid-Attenuated 181InversionRecoveryMRImagingtoDetectRemnantorRecurrentMeningiomasafterResectionH. Mori, A. Kunimatsu, O. Abe, H. Sasaki, H. Takao, T. Nojo, K. Kawai, N. Saito, K. Ohtomo

XanthogranulomaoftheSellarRegion. 190ACaseReportA. Agarwal, K. Agarwal, H.K. Lee

CervicalChordoma.ACaseReport 194andDifferentialDiagnosisR. Conforti, G. Taglialatela, F. Rinaldi, E. Quaranta, M. Cirillo, G. Paolisso, S. Cirillo

PrimarySkullOsteosarcoma:MDCT 197EvaluationandHistopathologicalCorrelationinTwoCasesK. Gangadhar, D. Santhosh

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Instructions for Authors 206

Cover: John Singer Sargent: Daughters of Edward Darley Boit (detail) - Oil painting. 1882. - By deflam - http://www.flickr.com

Official Journal of:AINR - Associazione Italiana di NeuroradiologiaandThe Neuroradiologists of Alpe-AdriaANRS - Albanian Neuroradiological SocietyPANRS - Pan Arab NeuroRadiology SocietyRadiological Society of Saudi Arabia, Division of NeuroradiologyEgyptian Society of NeuroradiologyISNR - Indian Society of NeuroradiologyIndonesian Society of NeuroradiologyNeuroradiology Section of the Radiology Society of IranIsraeli Society of NeuroradiologyCollege of Radiology MalaysiaNeuroradiology Section - Pakistan Psychiatry Research CenterSection of Neuroradiology - Polish Radiological SocietyThe Neuroradiologists of RomaniaSection of Neuroradiology of Serbia and MontenegroSILAN - Sociedad Ibero Latino Americana de NeurorradiologiaNeuroradiology Section of Singapore Radiological SocietySlovenian Society of NeuroradiologyThe Neuroradiological Society of TaiwanTSNR - Turkish Society of Neuroradiology

ISSN 2239-7493

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SUMMARY – Gliosarcomas are rare tumors with a poor prognosis composed of intermingled ma-lignant glial and sarcoma elements with an estimated incidence of 1.8-8.0% of all malignant astro-cytic neoplasms. We aimed to review the imaging findings in eight patients with gliosarcoma who were treated in our center between 2002 and 2010. The diagnosis was confirmed by morphological and immunohistochemical stains. This study, to the best of our knowledge, is the largest describing the imaging manifestations of this tumor. Although our study revealed no unique radiological fea-tures for gliosarcoma, it is important to note that they all demonstrated either dural or ependymal involvement or both. Calcification, hemorrhage or cystic components are described with a tendency for a ring enhancement pattern. Interestingly pre-existing benign looking lesions and associated remotely located small meningiomas are also described.

RadiologicalFindingsinGliosarcomaASingleInstitutionExperience

M.Y.SWAIDAN,M.HUSSAINI,I.SULTAN,A.MANSOURKing Hussein Cancer Center; Amman, Jordan

Key words: gliosarcoma, radiological features, glioblastoma multiforme

Introduction

Gliosarcoma (GSa) is a very rare primary biphasic central nervous system (CNS) neo-plasm, classified by the World Health Organi-zation (WHO) 2007 as a variant of glioblas-toma (GBM), therefore a grade IV tumor 1. It represents approximately 1.8% of all cases of GBM 2. GSa shows a biphasic tissue pattern with glial and mesenchymal differentiation. The age of presentation ranges from the fourth to the sixth decades of life with a mean age of 53 years. However, cases have been described in children with tumors originating in the cer-ebral hemispheres 3-5. Gliosarcoma is usually situated supratentorially 8. The primary site of predilection is reported to be in the temporal lobe 1,2,7-8.

GSa was initially hypothesized to originate from neoplastic transformation of blood vessels in a preexisting glioblastoma 9. Recent immu-nohistochemical and genetic studies failed to support this theory, suggesting a monoclonal origin for both histological components 1.

GBM and GSa are reported to share sig-nificant clinical and genetic similarities 10. No significant differences between GSa and GBM

could be demonstrated with regard to age, sex, pretreatment Karnofsky performance status, tumor location, size, unifocality, median sur-vival time, nor to the rarity of extra-cranial metastasis 11.

None of the treatment regimens used, which included various combinations of radiation therapy and chemotherapy, improved the sur-vival of GSa over GBM 2.

We describe eight cases of pathologically proven GSa that were managed at our insti-tution during the period from 2002 to 2010. Special emphasis is placed on radiological and pathological findings.

Patients and Methods

We reviewed the clinical data and radiologi-cal imaging features of five female and three male patients with ages ranging from 19 to 66 years (median age: 51 years) diagnosed with GSa over a period of eight years (from January 2002 to December 2010). Two radiologists re-viewed the images retrospectively. Our clinical and epidemiological findings are summarized in Table 1.

174

Radiological Findings in Gliosarcoma M.Y. Swaidan

could be identified in only one case, which happened to be the same patient (Figure 2).

MRI showed variable appearance on both T1 and T2 weighted images. In general, the tumors had a tendency for hypointense sig-nal on T1 and hyperintense signal on T2 (Figure 3). Six tumors (75%) showed intense ring-like enhancement (Figure 4) and two heterogeneous enhancement of a mainly solid tumor. Associated dural (62%) and/or epend-ymal involvement (62%) was present in all

Results

Five of eight patients (62%) were females. All lesions were located supratentorially. In five cases (62%) the tumor was located in the pa-rietal lobe (Figure 1). The other three were in the occipital lobe, temporal lobe (Figure 2) and occipitoparietal lobes. Tumors were iso- or hy-perdense on CT with evidence of calcification in three of four cases who had brain CT scan done (Figure 1). Cystic components and hemorrhage

Case 1 Case 2 Case 3 Case 4 Case 5 Case 6 Case 7 Case 8

Age (yrs) 36 19 64 37 66 58 48 54

Sex Female Male Female Female Female Male Male Female

Pre-existingclinical conditions

NF – – – Hypertension DM and IHD

Epilepsy –

Locationof tumor

Parietal Parietal Parietal Parietal Occipital Parietal Temporal Occipital and

parietal

Outline infiltrative well-defined

infiltrative infiltrative infiltrative infiltrative infiltrative infiltrative

Dural involvement

+ – + – + – + +

Ependymal involvement

– + – + – + + +

Edema +++ ++ + ++ ++ +++ +++ ++

Calcification NA + – NA NA + + NA

Hemorrhage – – – – – – + –

Cystic component

– – – – – – + –

Enhancement pattern

Ring Ring Heterogeneous Ring Ring Ring Heterogeneous Ring

Pre-existing lesion

+* – + – – – + –

Associated meningioma

– – – – + – – +

Density on CT

NA Hyper NA NA NA Iso Iso NA

Signal intensity on MRI

T1 Hypo Iso Hypo Hyper Hypo to Hyper

Hypo Hyper Hypo

T2 Hyper Iso Hyper Hypo Hypo to Hyper

Hyper Hypo Hyper

NF: neurofibromatosis, DM: diabetes mellitus, IHD: ischemic heart disease, NA: not available, *: unknown pathology

Table 1 Summary of the epidemiological, clinical and radiological findings in the eight patients diagnosed at the King HusseinCancer Center.

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Pathological Findings

Microscopically, all tumors but one showed a mixture of glial and mesenchymal elements, with mesenchymal elements occupying 30-80% of the slides examined. The most frequent sar-comatous pattern was spindle cell fibrosarcoma (6/8 cases), while two cases showed malignant fibrous histiocytoma pattern, with malignant giant cells scattered. In addition, chondroid-like differentiation and calcification was de-tected in two cases each. One case showed evidence of a pre-existing lesion in the form of benign fibroblastic/meningeal proliferation admixed with blood vessels and calcification suggesting meningoangiomatosis. Mitosis was identified in both glial and sarcomatous com-ponents. Reticulin stain highlighted the differ-ence in both components as it was positive in the sarcomatous but not the glial component.

Immunostains were performed on all cases. The malignant glial component was positive in all cases examined, while the sarcomatous component showed patchy staining with GFAP in only one case, with negative staining in all other cases. Vimentin was positive in all cases in both components. In a single case, focal staining with desmin was detected in the sarco-matous component. P53 was performed on four

cases (Figures 2 and 3) and moderate to se-vere edema was evident in seven (85%) (Fig-ure 1). The outline of the tumor was infiltra-tive in seven cases (85%) (Figure 3). Interest-ingly, three cases had a pre-existing lesion at the same site of the tumor at least two of which had benign radiological appearance (Figures 2 and 3). Two other female patients had associated small meningiomas (Figure 4).

Three of our cases died six, 17, 18 months after diagnosis, three patients are lost to follow-up, two of which after documented tumor progres-sion by brain MRI. The last two patients are still alive five and 12 months after surgery but with evidence of tumor progression on brain MRI.

Data from the Jordan cancer registry were reviewed to get an idea of the incidence of GBM and gliosarcoma in Jordan. Accurate reg-istration of cases could only be obtained since July 2006. The total number of GBM cases in adults for the period July 2006 till June 2010 was 162: 48 (30%) females and 114 (70%) males. On the other hand, the total number of GSa cases in the same period was seven 7: four (57%) females and three (43%) males. The percentage of the GBM cases from the to-tal number of primary brain tumors in adults, which is 462, was calculated to be about 35% for this period and that for GSa about 1.5%.

Figure 1 A 19-year-old male patient (case 2). Non-enhanced CT scan in November 2006 showing a 5 cm deeply seated pa-rietal lobe mass with coarse calcification (black arrow), well-defined outline, moderate surrounding edema (white arrow), sulcal effacement and left lateral ventricular trigonal compres-sion (small arrowhead) and minimal midline shift to the right side (large arrowheads). It appears homogenously hyperdense.

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A B

C D

177


most common types of sarcomatous differentia-tion are fibrosarcomatous and malignant fibrous histiocytoma-like types 1,7. The chondrosarcoma and osteosarcoma patterns have also been re-ported in association with postradiation GSa 21,22. The smooth and striated muscles differentiation of a sarcomatous component is also known 23.

No definite risk factors are known to be as-sociated with this apparent differentiation or metaplasia, although GSa was diagnosed at the time of second craniotomy for a previously resected and irradiated GBM. Perry et al 7 re-viewed 32 cases of pathology-proven GSa. He described 25 cases of de novo GSa (primary GSa) and seven cases arising after irradiation of previously resected glioblastoma (secondary GSa). The clinical presentation and location for the post-irradiation GSa were not different from the primary GSa patients. Whether therapeutic irradiation given for GBM induces or influences sarcomatous transformation is speculative.

GSa is mostly seen in the supratentorial re-gion with rare cases reported in the cerebel-lum 24. In data from SEER, predilection for the temporal and frontal lobes comes in accordance with the literature 2,7, where both locations ac-counted for more than 70% of all cases. It is of note, that in six of our cases, the tumor in-volved the parietal lobe, a well described but uncommon site of involvement 2,3,7,8,12,24-26.

Clinico-radiologic correlation is very impor-tant in making the diagnosis, although no spe-cific radiological features are unique to GSa. Most cases appear as intraaxial, variably en-hancing necrotic tumor 7, but some GSa show a superficial location and dural invasion 24, re-sembling meningiomas 27. The presence of as-sociated parenchymal involvement, infiltrative outline, severe edema, and irregular enhance-ment should assist with radiologic diagnosis of GSa over meningioma. Intratumoral calcifica-tion may occasionally appear and corresponds to chondroid or osteoid metaplasia. Cystic com-ponents are reported to be relatively common

7 although we noticed this in only one of our cases. MR imaging characteristics of GSa in cerebral hemispheres have been reported to be variable 21,22. Although they are generally reported to be isointense with gray matter in T2-weighted sequences, a heterogeneous ap-pearance in T1- and T2-weighted sequences can be observed because of necrosis and hem-orrhage 25,26. On the enhanced T1-weighted MR images they demonstrate intense tumor en-hancement, often with a ring-like appearance. The isointense component on the T2-weighted

cases and was either strongly or diffusely posi-tive (three cases), or completely negative (one case) in both components. Discrepant stain-ing for P53 between the glial and sarcomatous component was not noted in any of the cases.

Discussion

GSa is an uncommon primary central nerv-ous system tumor composed of intermingled malignant glial and sarcomatous elements. It was first described in 1895 by Stroebe 12 and gained general acceptance after it was rede-scribed by Feigin in 1955 13 and Robinstein e year later 14. GSa has an estimated incidence of 1.8-8.0% of all malignant astrocytic neoplasms

2,8. This study is unique in combining the radio-logical findings and the clinical course in eight cases of GSa seen at the primary oncology refer-ral center in Jordan over a period of eight years making this report the largest in the literature to date. In doing so, we have tried to overcome some of the limitations associated with study-ing rare tumors, such as the long periods re-quired to assimilate a large number of cases.

Interestingly, three out of our eight patients (37%) were rather young (<40 years) as opposed to the patients registered in the SEER data-base 15, who were mostly elderly, with <10% of patients younger than 40 years of age. Males predominated in the SEER database.

Histogenesis of GSa was initially deemed con-troversial 7, with capillary endothelium 9, astro-cytic elements 16, multipotential stem fibrohisti-ocytic cell 9,17, and vascular smooth muscles 9 all suggested as possible origins. However, the dem-onstration of identical mutations in both histo-logical components 18,19 suggested a monoclonal origin 3 probably pluripotential stem cells 20. The

← Figure 2 A 48-year-old male (case 7) known to have epi-lepsy for the last 30 years which is not well-controlled. A) CT scan done in April 1989 shows a heavily calcified intra-axial Rt temporal lobe lesion (black arrow) (first demonstrated on a poor quality CT dated 1980) which was documented to re-main stable till 1998. There was no pathological diagnosis. In January 2010 brain CT and MRI documented tumor progres-sion. B) The tumor is located at the right temporal lobe and is complex with large enhancing cystic component (white arrow) and a partially enhancing solid component that reaches the brain surface with associated leptomeningeal enhancement (small arrowheads). Signal void is noted within the solid part of the tumor on T1 and T2 corresponding to the known calci-fication (asterisk). The wall of the cystic component involves the ependymal surface of the right lateral ventricular trigone (large arrowhead) (C). On the non-enhanced axial T1 weighted images a focal hyperintense area (curved arrow) that did not appear on an MRI done a month earlier, is considered a small hematoma (D).

178


Figure 3 A 64-year-old woman (case 3). Brain MRI in September 2007 showed a superficially located left parasagittal parietal mass. It appears hypointense on T1 (A) hyperintense on T2 with mild surrounding edema (black arrow) (B). After IV contrast administra-tion the mass shows vivid but slightly heterogenous enhancement with infiltrative outline (C). It appears to be infiltrating the dura (falx cerebri) with an associated enhancing dural tail (small arrow heads) and shows a small non enhancing mostly necrotic area (not shown here). Five months earlier the patient had a brain MRI at the onset of symptoms which showed a focal area of enhanc-ing leptomeningeal thickening (large arrow head) at the same site as the brain tumor (D). No intervention was done at that time.

A B

C D

179


Conclusion

In summary, we described eight cases of pathologically proven GSa. This is a high-grade tumor associated with a dismal prognosis. Ra-diologically, two main forms of the tumor were encountered in our cases. One was a deeply seated tumor related to the ependymal sur-face of the lateral ventricle that could resem-ble high grade glioma. The other form we en-countered was superficial location with a dural attachment resembling meningioma or malig-nant meningioma when associated with brain parenchymal infiltration, marked surrounding white matter edema, as well as rapid growth. There was a high tendency for parietal lobe in-volvement with preference for the female sex. Radiologically, the tumor tends to have an in-filtrative outline with either ependymal or me-ningeal involvement and associated surround-ing edema.

We recommend considering the diagnosis of GSa in the differential diagnosis of brain tu-mors that resemble the radiological appearance of meningioma especially when it demonstrates

images corresponded to this area of intense enhancement. Reports exist of a more homo-geneous pattern of enhancement encountered in primary GSa of the posterior fossa 24. Thus, the radiological differential diagnosis should include GSa when features of aggressive clini-cal behavior and highly malignant radiologic features coexist. While two of our cases were suspected initially to be meningiomas due to the presence of a dural tail, these cases shared atypical features such as parenchymal invasion and surrounding edema which should alert for the possibility of a more aggressive diagnosis.

GSa is associated with a dismal prognosis. In the SEER database the median survival was seven months. Surgery and radiotherapy are the two mainstays of treatment, but survival is not markedly improved with either modality. It is uncertain if sarcomatous features adversely affect survival. Examples of systemic metas-tases have been documented in the literature with sites of spread including lung, bone, and lymph nodes 26,28, few of which described metas-tasis from GSa containing only mesenchymal elements 29,30.

Figure 4 Contrast-enhanced brain MRI studies for two women aged 66 (A) and 54 (B) years (cases 5 and 8 respectively) showing enhancing dural based well-defined small masses that are considered to be incidental findings of meningiomas (arrowheads). In (A) the occipital mass shows peripheral ring enhancement with shaggy inner margin (arrow).

A B

180


tricular ependymal surface. It is worth men-tioning that in our cases we noted a tendency for presentation at a young age compared to the data from the SEER.

high grade features, rapid progression and a tendency to recur. It is also recommended to consider this diagnosis in deeply seated white matter brain tumors with relation to the ven-

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Dr Asem MansourDiagnostic Radiology DepartmentKing Hussein Cancer CenterQueen Rania Al Abdullah StJubeiha, Amman, Amman, JordanTel.: 0096265300460Fax: 0096265342567E-mail: [email protected]

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SUMMARY – It has been suggested that a difference in signal intensity (SI) between the resec-tion cavity and normal cerebrospinal fluid (CSF) on fluid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI) in partially resected gliomas indicates subsequent or coinci-dent tumor progression. We considered that this would hold true for resected meningiomas as well. Hence, we aimed to assess whether or not such a difference in the SI during the follow-up evalua-tion helps predict residual or recurrent tumor in resected meningiomas. We evaluated 63 patients with resected meningiomas. The SI within the resection cavity observed on FLAIR images was qualitatively and quantitatively assessed during follow-up. Qualitative analysis comprised visual comparison of the SI in the resection cavity with that of normal CSF by neuroradiologists. The SI in the resection cavity was quantitatively assessed by region of interest (ROI) analysis and normal-ized against the background noise and CSF SI. Normalized SI recorded during follow-up was com-pared with that recorded immediately after resection. Tumor progression was defined as a 20% or greater increase in the diameter of the longest residual or recurrent meningioma (Response Evalu-ation Criteria in Solid Tumors). The sensitivity and specificity of the elevated SI in the resection cavity for indicating residual or recurrent tumor were calculated. Qualitative analysis by FLAIR MRI showed that patients with remnant tumor following surgery had a prolonged SI increase in the resection cavity. Further, SI increase could not always be observed before recurrence, and both SI increase and regrowth remnant/recurrence could be detected in the same MRI examination. In resected meningiomas, leakage of tumor elements into the resection cavity, presumably tumor cells, manifests as an SI increase on FLAIR images and indicates residual or recurrent tumor. However, unlike the previous reports on partially resected gliomas, we concluded that the SI change does not always precede tumor progression or recurrence.

DiagnosticAbilityofFluid-AttenuatedInversionRecoveryMRImagingtoDetectRemnantorRecurrentMeningiomasafterResection

H.MORI1,A.KUNIMATSU1,O.ABE3,H.SASAKI1,H.TAKAO1,T.NOJO1,K.KAWAI2,N.SAITO2,K.OHTOMO1

1 Department of Radiology, 2 Department of Neurosurgery, Graduate School and Faculty of Medicine, The University of Tokyo; Tokyo, Japan3 Department of Radiology, Nihon University School of Medicine; Tokyo, Japan

Key words: MR advanced techniques, meningioma, recurrence, remnant

Introduction

Recently, Winterstein et al. hypothesized that in case of partially resected gliomas, a dif-ference in the signal intensity (SI) between the resection cavity and the normal cerebrospinal fluid (CSF) as determined by fluid-attenuated inversion recovery (FLAIR) magnetic reso-

nance imaging (MRI) indicates subsequent or coincident tumor progression 1. We extended this hypothesis to resected meningiomas. Hence, the current study aimed to assess whether or not a difference in SI between the resection cavity and CSF helps predict resid-ual or recurrent tumor progression in resected meningiomas.

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Diagnostic Ability of Fluid-Attenuated Inversion Recovery MR Imaging to Detect Remnant or Recurrent Meningiomas after... H. Mori

Systems) by using the standard circular polar-ized head coil. The imaging protocol involved transverse T1-weighted spine-echo (SE), T2-weighted fast SE (repetition time (ms)/echo time (ms), 4000/108), and coronal FLAIR (ex-cept one patient with transverse FLAIR) se-quences with identical slice parameters. The T1-weighted sequences were repeated in the transverse and coronal planes after application of contrast media. For T1-weighted SE (540/9) sequences, the following slice parameters were used: 23 slices with a matrix size of 224 × 256, a square field of view of 210 × 210 mm2, a slice thickness of 5 mm, and a slice gap of 1.5 mm. For the FLAIR sequence, the parameters were a repetition time (ms)/echo time (ms) of 8000/144 and an inversion time of 2000 ms, 23 slices with a matrix size of 192 × 256, a square field of view of 210 × 210 mm2, and a slice thick-ness of 5 mm, using interleaved acquisitions. The T1-weighted SE sequences (transverse and coronal) were obtained (560/9) after application of contrast medium at a slice thickness of 5 mm.

Image Analysis

Image analysis was performed by two inde-pendent analysts (HM and AK) using our picture archiving and communication system. Both an-alysts were blinded to the clinical data and each other’s results. During the follow-up analysis, we qualitatively and quantitatively evaluated SI within the resection cavity using FLAIR MRI. Only the FLAIR and T1-weighted sequences were considered for the measurement and eval-uation of SI. This is because the SI within the resection cavity on T2-weighted images was found to be very high on average; and hence, an increase in this parameter would hardly be de-tected. In addition, all available sequences were carefully studied to estimate tumor progression and detect intra- or peritumoral hemorrhage.

Qualitative Analysis

Both the analysts visually compared the SI within the resection cavity with that of nor-mal CSF. For this purpose, all the FLAIR and T1-weighted images were scrutinized during follow-up in order to evaluate whether the SI within the resection cavity was isointense or hyperintense as compared to the CSF and background noise (Figure 1). The assessment continued until progressive disease occurred or until the last MRI examination included in this analysis was conducted. All unenhanced T1- and

Materials and Methods

Patients

We retrospectively identified and reviewed reports from our institution’s clinical database from November 2006 to April 2010 using the following keywords: meningioma, operation, re-section, and resected. We found records for 63 patients. A radiologist (HM) reviewed the clini-cal charts and conventional MR images on our picture archiving and communication system (Centricity PACS; GE Healthcare Integrated IT Solutions, Barrington, IL, USA) to identify patients with intracranial meningiomas that had been histologically verified.

Of these 63 patients, ten (16%) were excluded because information from only a single MRI ex-amination was available. Among these, three patients had undergone resection twice and one patient had undergone resection five times. Thus, the study involved 53 patients with a to-tal of 60 resected meningiomas (33 women and 20 men; mean age at the time of imaging, 60.1 years [standard deviation = 2.0]) who under-went follow-up MRI between November 2006 and April 2010. The location of the tumors was as follows: 12 convexity; ten parasagittal; six cerebellopontine angle; six petroclival; five sphe-noid ridge; three falx; three multiple; three ten-torial; two cavernous sinus; two posterior fossa; two skull base; one clinoidal; one clival; one intraventricular; one middle cranial fossa; one olfactory groove; and one petrous meningiomas.

In terms of distribution, 30 of the 60 meni-giomas were meningothelial, ten were fibrous, nine were transitional, five were atypical, five were anaplastic, and one was lymphoplasma-cyte-rich. In addition, gamma-knife sterotactic radiosurgery was conducted for four lesions during the observation period.

Definition of Tumor Progression

On the basis of the Response Evaluation Cri-teria in Solid Tumors 2,3, tumor progression was defined as a progressive disease with a 20% or greater increase in the diameter of the longest tumor, as observed on FLAIR images.

MRI Protocol

MR images were obtained at four 1.5-T units (Signa EXCITE HD/HDx, GE Medical Systems; MAGNETOM Avanto, Siemens Medical Solu-tions; EXCELART Vantage, Toshiba Medical

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1). The ROI values obtained from the resec-tion cavity were normalized according to those obtained from ROIs placed on background noise (ROI 2) and CSF (ROI 3; Figure 2). The observed SI increase was defined as three or more times the normalized SI ratio relative to CSF ([ROI 1 – ROI 2]/[ROI 3 – ROI 2]). Dur-ing follow-up, these normalized SI values were compared with the SI values obtained from the MRI examination performed immediately (within a week) after resection or during the first examination of the study period.

Results

Follow-up

In 13 of the 60 lesions (22%), progressive dis-ease was detected (nine regrowth remnants, and four recurrent tumors). The median follow-up time was 1.7 years (minimum, 0.1 year; max-imum, 6.7 years) in the group of patients with progressive tumors and 3.5 years (minimum, 0.1 year; maximum, 36.4 years) in the group

T2-weighted images were assessed for signs of hemorrhage. In addition, the analysts assessed whether contrast enhancement in the tumor tissue could be identified, and if it was detected, whether the enhancement was close (<1.5 cm) to the resection cavity. In this study, all MRI ex-aminations were jointly randomized, and iden-tifying variables such as the patients’ names, were omitted. The analysts were also blinded to the dates of the MRI examinations and the date when progressive disease was identified.

Quantitative Analysis

Four weeks after the qualitative analysis, the first reader (HM) was asked to quantita-tively assess the images. The analyst was again blinded to the same details as those blinded to in the quantitative analysis. Only the FLAIR images were quantitatively analyzed in this case because the qualitative analysis of these images revealed that the increase in the SI was easier to detect on them. The quantita-tive analysis entailed a region of interest (ROI) analysis of SI within the resection cavity (ROI

A B

Figure 1 Progressive disease with increased signal intensity and recurrence of tumor size at the same time. Follow-up MR im-ages in 73-year-old woman with histopathologically proven anaplastic parasagittal meningioma. A) FLAIR MR image illustrate qualitative analysis of the resection cavity. Qualitative analysis was performed by visual comparison of signal intensity within the resection cavity (arrow) with that of CSF and background noise. B) Contrast-enhanced T1-weighted image shows a recurrent tumor (arrow) at the periphery of the resection cavity.

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Figure 2 Nonprogressive disease with increased signal intensity. Follow-up MR images in 61-year-old man with histopathologi-cally proven meningothelial convexity meningioma. A) FLAIR MR image illustrate quantitative analysis of signal intensity using ROI analysis. ROI 1, resection cavity; ROI 2, background noise; ROI 3, CSF. B) Contrast-enhanced T1-weighted image shows a residual tumor (arrow) without progression during the observation period at the periphery of the resection cavity. The resection cavity (arrowheads) represent slight hyperintensity also on T1-weighted image.

Figure 3 Progressive disease with increased signal intensity and progression of tumor size at the same time. Follow-up MR im-ages in 78-year-old woman with histopathologically proven meningothelial petroclival meningioma. A) At initial study within the observation period, FLAIR shows iso-intensity of the resection cavity (arrow) relative to CSF. B) At 11 months later, the increase in signal intensity (arrow) occurred at the same time as the progression of tumor size.

A B

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months before the increase in tumor size (Fig-ure 4). In one of the three patients with recur-rence, the FLAIR images showed an increase in SI. Of the 25 patients with coexisting men-ingiomas, 16 showed no increase in SI. In one patient with residual meningioma, the FLAIR images revealed an increase in SI after gamma-knife stereotactic radiosurgery (Figure 5). One of 16 patients without remnant regrowth or re-currence showed an SI increase.

The results of the group in which operation was not performed (–) during the observation period (Table 1) indicated that two progressing meningiomas (one remnant regrowth and one recurrence) showed an increase in SI and tumor size progression at the same time point (Figure 1), whereas six of 11 tumors (ten remnants re-growth and one recurrence, Figure 2) showed no increase in SI. In one patient with a large tumor remnant, the remote CSF space also had high SI. None of the eight patients without remnant or recurrent meningiomas showed SI increase.

Overall, the SI increase on FLAIR images had 39% (95% confidence interval [CI]: 31%, 41%) sensitivity, 96% (95% CI: 83%, 99%) specificity, 93% (95% CI: 73%, 99%) positive predictive value, 51% (95% CI: 45%, 53%) negative predic-tive value, and 62% (95% CI: 52%, 64%) accuracy for remnant or recurrent meningiomas (Table 2).

with nonprogressive tumors. The follow-up time was significantly different between these two (P = 0.001, two-sided Mann-Whitney U-test).

Qualitative analysis

Interrater agreement. A total of 360 images were qualitatively analyzed by two independ-ent raters. Kappa coefficients were 1.0 for both FLAIR and T1-weighted images, indicating per-fect agreement. Because no discordant assess-ments between raters were observed, the vari-ance of the kappa coefficient was zero, and hence, confidence intervals could not be calculated.

Quantitative analysis

The results of the group in which operation was performed (+) during the observation pe-riod (Table 1) showed that in the quantitative analysis performed within a week after resec-tion, a prolonged increase in SI within the re-section cavity on the FLAIR images was ob-served in 28 of the 41 (68%) patients. In five of the eight patients with regrowth remnants, the FLAIR images revealed an increase in SI and the tumor size at the same time point (Figure 3). In one patient with disease progression, the SI within the resection cavity increased four

Operation during observation period Remnant Regrowth/recurrence No. of SI increase (%)

+ 41

+ 22+ 8 5 (62.5%)

– 14 3 (21.4%)

– 19+ 3 1 (33.3%)

– 16 1 (6.3%)

– 19

+ 10+ 1 1 (100%)

– 9 3 (33.3%)

– 9+ 1 1 (100%)

– 8 0 (0%)

Table 1 Analysis sheet.

Remnant/recurrence

+ –

SI increase+ 14 1 15 PPV 93%

– 22 23 45 NPV 51%

36 24 60 accuracy 62%

sensitivity 39% specificity 96%

PPV = positive predictive valueNPV = negative predictive value

Table 2 Two-by-two contingency table.

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Figure 4 Progressive disease with increased signal intensity before progression of tumor size. Follow-up MR images in 75-year-old woman with histopathologically proven anaplastic parasagittal meningioma. A) The increase in signal intensity is already visible within the resection cavity (arrow) at the time of two months after surgery. B) Contrast-enhanced T1-weighted image shows only a tiny residual tumor (arrow) at the same time. C,D) Four months later, FLAIR and contrast-enhanced T1-weighted images dem-onstrate rapid tumor progression (arrow).

A B

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Figure 5 Regressive disease with increased signal intensity during regression of tumor size after gamma-knife stereotactic ra-diosurgery. Follow-up MR images in 36-year-old woman with histopathologically proven meningothelial cerebellopontine angle meningioma. A,B) No increase in signal intensity within the resection cavity was observed on FLAIR image despite the massive residual tumor. C) The increase in signal intensity appears on FLAIR image following radiosurgery. D) The residual tumor be-comes decreased in size.

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there was a sustained increase in the SI on FLAIR images within a week after operation (28/41patients = 68.3%). Gamma-knife stere-otactic radiosurgery in such conditions would increase the level of tumor necrosis and lead to an increased protein concentration in the cavity 10,11. When the residual tumor volume is large, the resection cavity, and even the remote CSF spaces may contain tumor products, thus showing hyperintensity on the FLAIR images. However, in open spaces with high CSF flow, such as the paracavernous region and cerebel-lopontine angle, FLAIR was unable to indicate an SI increase, despite the presence of tumor remnants or recurrence 12.

The limitations of our study were its retro-spective design and the relatively small number of patients. In our patient population, MRI ex-amination was performed with four different kinds of MR units. Hence we cannot guarantee that this imaging sign is consistently applica-ble. However, considering that the reason for the increase is SI is concurrent meningioma breakdown, we believe that the reported im-aging sign will be useful for resected meningi-omas in the absence of contrast media as long as tumor breakdown has occurred.

Conclusion

In conclusion, high concentrations of certain proteins or blood elements in the resection cav-ity of resected meningiomas, presumably due to tumor breakdown, results in an increase in the SI within the resection cavity on FLAIR images. This finding is a highly specific sign of coinci-dent tumor. Although the sensitivity of the in-creased SI is not very high, it can easily be used in follow-up MRI examinations. However, unlike previous reports on partially resected gliomas, we concluded that a change in the SI does not always precede tumor regrowth or recurrence.

Discussion

This study analyzed the SI within the resec-tion cavity of resected meningiomas on FLAIR MR images. Both quantitative and qualitative analyses revealed that an increase in SI can help detect remnant or recurrent tumors. We showed that this simple morphologic sign had a high specificity and positive predictive value for coexisting meningioma in our study popula-tion. However, the sensitivity of this imaging indicator, i.e., 39%, was too low. Furthermore, in only two of 13 cases, could the increase in SI be detected several months before the progres-sion of tumor size or recurrence. However, the achieved sensitivity and specificity of 39% and 96%, respectively, are quite acceptable, espe-cially considering that no additional time was required for analyses. Moreover, the qualita-tive measurement obtained by comparing SI of the resection cavity with that of the CSF and background noise, which is used to determine whether SI is hyperintense or isointense, does not require much experience or time.

FLAIR imaging has been useful for demon-strating dural abnormality associated with meningiomas, without the initial need for con-trast medium 4,5. Our study showed that FLAIR imaging is useful for the diagnosis of residual or recurrent meningiomas even during follow up, using the parameter of SI increase in re-sected cavities. This approach is advantageous in situations where contrast medium cannot be used in patients with conditions such as renal dysfunction or drug hypersensitivity.

We postulate that the reason for the increase in SI in patients with progressive disease may be the leakage of proteins 6,7 or blood elements 8,9 from the tumor with fragile neovascularity into the resection cavity. Because of hypervascular-ity of meningiomas, the concentration of blood elements within the cavity of resected men-ingiomas is probably high because of which

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10 Fujimoto A, Matsumura A, Maruno T, et al. Normal pressure hydrocephalus after gamma knife radiosur-gery for cerebellopontine angle meningioma. J Clin Neurosci. 2004; 11: 785-787.

11 Kajiwara M, Yamashita K, Ueba T, et al. Normal pres-sure hydrocephalus after radiosurgery for sphenoid ridge meningioma. J Clin Neurosci. 2009; 16: 162-164.

12 Wu HM, Yousem DM, Chung HW, et al. Influence of imaging parameters on high-intensity cerebrospinal fluid artifacts in fast-FLAIR MR imaging. Am J Neu-roradiol. 2002; 23: 393-399.

References

1 Winterstein M, Münter MW, Burkholder I, et al. Par-tially resected gliomas: diagnostic performance of fluid-attenuated inversion recovery MR imaging for detec-tion of progression. Radiology. 2010; 254: 907-916.

2 Therasse P, Arbuck SG, Eisenhauer EA, et al. New guidelines to evaluate the response to treatment in solid tumors. European Organization for Research and Treatment of Cancer, National Cancer Institute of the United States, National Cancer Institute of Canada. J Natl Cancer Inst. 2000; 92: 205-216.

3 Eisenhauer EA, Therasse P, Bogaerts J, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer. 2009; 45: 228-247.

4 Takeguchi T, Miki H, Shimizu T, et al. The dural tail of intracranial meningiomas on fluid-attenuated inver-sion-recovery images. Neuroradiology. 2004; 46: 130-135.

5 Takeguchi T, Miki H, Shimizu T, et al. Evaluation of the tumor-brain interface of intracranial meningiomas on MR imaging including FLAIR images. Magn Reson Med Sci. 2003; 2: 165-169.

6 Melhem ER, Jara H, Eustace S. Fluid-attenuated in-version recovery MR imaging: identification of protein concentration thresholds for CSF hyperintensity. Am J Roentgenol. 1997; 169 (3): 859-862.

7 Maeda M, Yagishita A, Yamamoto T, et al. Abnormal hyperintensity within the subarachnoid space evalu-ated by fluid-attenuated inversion-recovery MR imag-ing: a spectrum of central nervous system diseases. Eur Radiol. 2003; 13 Suppl 4: L192-201.

8 Noguchi K, Ogawa T, Inugami A, et al. Acute sub-arachnoid hemorrhage: MR imaging with fluid-atten-uated inversion recovery pulse sequences. Radiology. 1995; 196: 773-777.

9 Ripoll MA, Raininko R. Experimental intracerebral and subarachnoid/intraventricular haemorrhages. Acta Radiol. 2002; 43: 464-473.

Harushi Mori, MDDepartment of RadiologyGraduate School and Faculty of MedicineThe University of Tokyo7-3-1 Hongo, Bunkyo-ku, Tokyo113-0033, JapanE-mail: [email protected]

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SUMMARY – Xanthogranuloma of the sellar region is a rare tumor. We describe a 41-year-old man complaining of several years of headache and passing out. Physical examination revealed absence of pubic and axillary hair while laboratory analysis showed panhypopituitarism. Magnetic resonance imaging showed a partially calcified slightly enhancing intrasellar mass with suprasellar extension which was slightly hyperintense on T1 images and hypointense on T2 images. Complete resection of the tumor mass using trans-sphenoidal approach was performed. On histopathologic analysis, there was a combination of fibrous scar tissue with chronic inflammation, highlighted by CD45 immunostaining, and extensively calcified necrotic debris, including cholesterol crystals consistent with a diagnosis of xanthogranuloma.

XanthogranulomaoftheSellarRegionACaseReport

A.AGARWAL,K.AGARWAL,H.K.LEEDepartment of Neuroradiology, Detroit Medical Center; Detroit, USA

Key words: xanthogranuloma, sella, pituitary, craniopharyngioma

Introduction

Xanthogranuloma of the sellar-suprasel-lar region is very rare. These tumors usually present with hypopituitarism or visual dysfunc-tion due to compression of optic chiasm. The first described series of 37 cases was reported by Paulus et al. in 1999 and xanthogranuloma of the sellar region was proposed as a distinct tumor, separate from craniopharyngioma 1. It was mentioned as a separate entity in the up-dated World Health Organisation classification of brain tumors from 2000. Usually, these tu-mors have a good prognosis with no reported case of relapse after complete resection.

We describe a case of xanthogranuloma of the sellar region manifesting as panhypopitui-tarism.

Case Report

A 41-year-old man presented with a history of several years of headache and passing out. The patient had a medical history of hypothy-roidism, hypercholesterolemia and gastro-es-ophageal reflux. There were no neurological deficits on clinical examination. Physical ex-amination revealed a complete absence of axil-

lary and pubic hair. Visual fields were intact by formal perimetry. Laboratory examination showed a very low level of pituitary hormones, growth hormone (GH) 0.1 ng/ml, follicle stimu-lating hormone (FSH) 0.1 m IU/ml, luteinizing hormone (LH) <0.1 m IU/ml, prolactin 1.6 ng/ml, plasma cortisol 4.2 ug/dl. GH releasing fac-tor, LH releasing hormone, TSH releasing hor-mone and ACTH releasing hormone tolerance tests showed poor responses. These findings suggested panhypopituitarism.

Non contrast-enhanced computed tomogra-phy scan of the head revealed a partially cal-cified mass in the sellar-suprasellar region (Figure 1). Magnetic resonance imaging dem-onstrated a sellar mass with suprasellar exten-sion which was slightly hyperintense on T1-weighted images, hypointense on T2-weighted images with slight enhancement post gadolin-ium (Figure 2). The mass was reaching almost up to the optic chiasm. There was no hydro-cephalus. There was no radiological evidence of cavernous sinus invasion. The normal flow voids of the cavernous carotid arteries were maintained. The pituitary gland was not seen separate from the mass. The preoperative diag-nosis was hemorrhagic pituitary adenoma.

The tumor was completely resected using a transsphenoidal approach. When the capsule

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A. Agarwal Xanthogranuloma of the Sellar Region

The histogenesis of xanthogranuloma re-mains unclear. It has been postulated that xanthogranulomas may reflect earlier patholo-gies of the sellar-suprasellar region like crani-opharyngioma, Rathke’s cleft cyst or pituitary adenoma which have undergone granuloma-tous degeneration secondary to hemorrhage, infarction or inflammation 3-5. Severe hypopi-tuitarism in these patients is most likely due to damage of the hypothlamo-pituitary axis by the granulomatous reaction 4,6.

There are no specific radiological features pathognomonic of xanthogranuloma. The com-mon differentials will include craniopharyngi-oma, Rathke’s cleft cyst and hemorrhagic pi-tuitary adenoma. Primarily intrasellar mass in an adolescent or young adult with severe hypopituitarism favours xanthogranuloma. One of the distinctive MR imaging features of xanthogranuloma reported in the literature is marked hypointensity of the lesion on T2-weighted images likely representing hemosi-derin deposits. In fact, micro or macrohemor-rhages may be essential in the formation of

was opened, yellowish fluid with crystal like material flowed out. On histopathologic analy-sis, no definite tumor was seen, though occa-sional poorly preserved cells were immunoreac-tive for keratin. The combination of fibrous scar tissue with chronic inflammation, highlighted by CD45 immunostaining, and extensively cal-cified necrotic debris, including cholesterol crys-tals are consistent with a xanthogranuloma.

The postoperative course was uneventful. His endocrinological dysfunction recovered partially. Follow-up MRI six weeks after the surgery showed no evidence of residual tumor.

Discussion

Xanthogranuloma of the sellar region was de-scribed for the first time in the literature in 1988

2. In a study published in 1999, 37 cases with a predominant xanthogranulomatous component were found among 110 cases of craniopharyngi-oma 1. Most of these tumors did not manifest the histological features of craniopharyngiomas.

Figure 1 A) Axial CT image in soft tissue window a densely calcified mass in the sella (arrow). B) Sagittal CT image in bone shows an enlarged sella with a calcified mass (open arrow) extending into suprasellar cistern.

A B

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Xanthogranuloma of the Sellar Region A. Agarwal

A B

C D

Figure 2 A) Axial T1 MR image shows a hyperintense mass in the sella (open arrows). B) Coronal T1 MR image redemonstrates the hyperintense mass in the sella (open arrow) with suprasellar extension and compression of the optic chiasm (white arrow). C) Axial T2-FLAIR image shows that the mass is predominantly hypointense on T2 (open arrow). D) Coronal T1 post contrast image shows that there is heterogenous enhancement of the mass, more so peripherally (open arrow). Also note the suprasellar component of the mass which is also showing peripheral enhancement (white arrow).

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Conclusion

Xanthogranuloma of the sellar-suprasellar region is rare but should be included in the differential diagnosis. Preoperative diagnosis, either radiological or surgical, is very difficult but marked T2 hypointensity of the mass with severe hypopituitarism in a young adult may suggest xanthogranuloma as a possible diag-nosis. A good clinical course may be obtained after complete resection of the tumor.

such tumors acting as a trigger for granulo-matous reaction 4. Calcification is rare in xan-thogranulomas, unlike craniopharyngioma. However, in our case, there was extensive cal-cification. Xanthogranulomas may show no, rim or heterogenous enhancement post gado-linium.

Surgical removal is necessary to obtain the correct diagnosis. All reported cases of xan-thogranuloma had favourable outcome with no recurrence after complete resection.

References

1 Paulus W, Honegger J, Keyvani K, et al. Xanthogranu-loma of the sellar region: a clinicopathological entity different from adamantinomatous craniopharyngioma. Acta Neuropathol. 1999; 97: 377-382.

2 Shirata K, Okada S, Matsumoto S. Histopathological study of the “cholesterol granuloma reaction” in the sellar and juxta-sellar tumors. No To Shinkei. 1988; 40: 133-139.

3 Sulentić P, Cupić H, Cerina V, et al. Xanthogranuloma of the sellar region in a patient with sarcoidosis. Acta Clin Croat. 2010; 49: 61-65.

4 Arai A, Nishihara M, Sasayama T, et al. Xanthogranu-loma of the sellar region–case report. Neurol Med Chir (Tokyo). 2010; 50: 488-491.

5 Sugata S, Hirano H, Yatsushiro K, et al. Xanthogranu-loma in the suprasellar region. Neurol Med Chir (To-kyo). 2009; 49: 124-127.

6 Murao K, Imachi H, Ishida T, et al. Panhypopituital-ism induced by xanthogranuloma of the sellar region. Nihon Naika Gakkai Zasshi. 2005; 94: 2597-2599.

Ajay Agarwal, MDDepartment of NeuroradiologyDetroit Medical Center3990 John RDetroit, 48084, USAE-mail: [email protected]

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SUMMARY – Chordomas are rare and aggressive tumors derived from notochordal remnants, usu-ally arising in the axial skeleton. The most frequently reported anatomic distribution of chordoma is 50% sacral, 35% spheno-occipital and 15% spinal. We describe the case of an elderly lady present-ing with progressive dysphagia, headache and neck pain. We found an expansile mass extending from C1 to C3. While running the diagnostic plan we considered a variety of lesions possibly involv-ing the cervical spine. Biopsy revealed the mass was a chordoma.

CervicalChordomaACaseReportandDifferentialDiagnosis

R.CONFORTI,G.TAGLIALATELA,F.RINALDI,E.QUARANTA,M.CIRILLO,G.PAOLISSO,S.CIRILLODepartment of Neuroscience, Neuroradiology, Second University of Naples; Naples, Italy

Key words: cervical chordoma, chordomas, cervical spine lesions, progressive dysphagia

Introduction

Chordomas are relatively rare low-grade ma-lignant tumors arising from embryonic rem-nants of the primitive notochord 1-3. They are considered locally invasive and aggressive 4 and rarely metastasize 2.

Chordomas account for 4% of all primary bone tumors 1,2. Approximately 15% occur in the vertebral column 1,3,4. They can affect patients of all ages, with a predilection for men. Median age is around 60 years, whereas skull-base lo-cations usually manifest earlier 3. The median survival rate is slightly lower in spinal chordo-mas (5.9 years) than in cranial and sacral le-sions (6.9-6.5 years) 1,3.

We present the case of an elderly woman with progressive dysphagia, hemoptysis and dizziness, caused by a cervical chordoma.

Case Report

An 82-year-old woman came to our observa-tion (09/11/07) complaining of progressive dys-phagia, haemoptysis on awakening and dizzi-ness. She referred three syncope episodes in the last six months resulting in head trauma with an extragaleal, left frontal hematoma and subsequent intense headache and neck pain.

On examination we found pain in the trape-zius muscles at acupressure, more evident on the right side and antalgic posture of the head that was leaned to the right and turned on the left. Neurologic examination was negative. The patient had a history of chronic atrial fibril-lation, hypertriglyceridemia, hypercholestero-lemia, c-reactive protein positivity.

Materials and Methods

Pharyngoscopy revealed a normal aspect of the pharyngeal mucosa. An MRI study of the neck, performed with and without i.v. gadolinium re-vealed an expansile mass involving the anterior part of the vertebrae from C1 to C3, anteriorly invading the pharyngeal space (Figure 1). Bone scintigram was negative. A cranio-vertebral junction CT scan showed that the expansile mass observed in MRI corresponded to a lytic lesion of the vertebral elements involved (Figure 2). A total body CT scan with iodinated i.v. contrast agent was negative. We proceeded to a biopsy.

Results

Histological diagnosis revealed the lesion was a chordoma of the cervical spine. At gross examination chordoma appears as a gelatinous

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R. Conforti Cervical Chordoma

calcifications thought to be bone sequestra. Solitary or multiple low-attenuation areas were sometimes seen within the bulk and represent myxoid and gelatinous material (Figure 3). After i.v. administration of iodinated contrast agent there was moderate to marked enhancement.

MRI is lacking in the evaluation of calcifica-tions and cortical bone, but it clearly depicted the soft mass and its relations with the adjacent tissues and structures. On T1 SE the chordoma

multilobulated mass, histologically character-ized by a pale matrix of mucopolysaccaride with a physaliphorous aspect. It can contain necrotic end hemorragic areas, in chondroid variant; the stroma resembles hyaline carti-lage with neoplastic cells in lacunae 2.

At CT chordoma appeared like a well-cir-cumscribed expansile soft tissue mass arising from the bone, hyperattenuating because of extensive bone destruction, with intratumoral

Figure 1 MRI of the neck shows an expansile, enhancing mass involving the anterior part of vertebrae from C1 to C3 (asterisk), mostly on the right, compressing the hypopharynx which is stenotic (arrowhead).

Figure 2 Cranio-vertebral junction CT shows lytic lesion of the involved vertebral elements, i.e. epistropheum erosion, whose tooth is fractured, and atlas erosion.

Figure 3 Histopathological examination discloses the lobular architecture: fibrous bands divide it into lobules containing cords of cohesive epithelioid cells with abundant eosinophilic cytoplasm and round nuclei within a myxoid matrix.

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Cervical Chordoma R. Conforti

tions like degenerative spondylosis, infection, trauma, metastatic disease, congenital abnor-malities, rheumatic conditions and primary spinal tumors or tumor-like lesions 5.

Mostly chordomas can be difficult to dis-tinguish from chondrosarcomas that are rare malignant tumors of the bone with a chon-droid aspect 9. As for the particular location we considered a cervical neuroma but it does not originate from the bone and does not involve the vertebral soma. In addition, we also had to consider rheumatic synovitis in our patient be-cause of c-reactive protein positivity but it does not directly originate from the bone 3,6. Addition-ally there is the relatively recently discovered intraosseous benign lesion, usually referred to as benign notochordal cell tumor, that in-cluded giant notochordal rests and notochordal hamartomas which can be a potential precur-sor of chordoma 6-8, having the same locations of chordomas but a mostly sclerotic aspect.

Our patient was sent for radiotherapeutic treatment, which she refused, so we opted for antalgic therapy and orthopedic neck brace.

had an intermediate to low signal, small foci of hyperintensity could be seen, representing hemorrhage or proteinaceous material. On T2 SE the chordoma showed high signal intensity, with low-signal-intensity septation, because of its vacuolated structure. After i.v. administra-tion of gadolinium the chordoma demonstrated moderate to marked enhancement 2.

Discussion and Conclusion

Chordomas most frequently arise from the axial skeleton, but extra-axial localizations have been reported. Treatment outcome and survival rate are influenced by the site and the size of the lesion 3. Chordomas destroy the bone and can encase and compress vascular and nervous structures 4. Symptoms are usu-ally insidious, neurological deficits depend on the location; pain is the most common, followed by weariness, especially in spinal lesions.

Cervical spinal canal compromise may be caused by common acquired pathologic condi-

References

1 Chugh R, Tawbi H, Lucas DL, et al. Chordoma: the nonsarcoma primary bone tumor. Oncologist. 2007; 12: 1344-1350.

2 Erdem E, Angtuaco EC, Van Hemert R, et al. Compre-hensive review of intracranial chordoma. Radiograph-ics. 2003; 23: 995-1009.

3 Jiang L, Liu ZJ, Liu XG, et al. Upper cervical spine chordoma of C2-C3. Eur Spine J. 2009; 18: 293-300.

4 Wippold FJ, Koeller KK, Smirniotopoulos JG. Clini-cal and imaging features of cervical chordoma. Am J Roentgenol. 1999; 172: 1423-1426.

5 Munday TL, Johnson MH, Hayes CW, et al. Muscu-loskeletal causes of spinal axis compromise: beyond the usual suspects. Radiographics. 1994; 14: 1225-1245.

6 Yamaguchi T, Iwata J, Sugihara S, et al. Distinguish benign notochordal cell tumors from vertebral chor-doma. Skeletal Radiol. 2008; 37: 291-299.

7 Yamaguchi T, Yamato M, Saotome K. First histologi-cally confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential di-agnosis of benign and malignant notochordal lesions. Skeletal Radiol. 2002; 31: 413-418.

8 Yamaguchi T, Watanabe-Ishiiwa H, Suzuki S, et al. In-cipient chordoma: a report of two cases of early-stage chordoma. Mod Pathol. 2005; 18: 1005-1010.

9 Dominguez C.J, Martin-Ferrer S, Rimbau J, et al. Chondrosarcoma cervical alto. Neurocirugia. 2005; 16: 261-265.

Gemma Taglialatela, MDDepartment RadiodiagnosticCasa Di Cura Clinica Villa Dei FioriCorso Italia80011 Acerra, Italy E-mail: [email protected]

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SUMMARY – Osteosarcomas are typically long bone tumors and rarely affect the skull, with most articles reporting single cases. As elsewhere in the body, these lesions may be classified as primary or secondary, chiefly post-Paget and post-radiation therapy. We describe two cases of primary oste-osarcoma of skull one presenting with cerebellar symptoms and another with giant skull swelling. Complete evaluation with 64 slice CT and histopathological correlation was carried out.

PrimarySkullOsteosarcoma:MDCTEvaluationandHistopathologicalCorrelationinTwoCases

K.GANGADHAR1,D.SANTHOSH2

1 Department of Radiodiagnosis and Imaging, 2 Department of Pathology, Institute of Medical Sciences, Banaras Hindu University; Varanasi, India

Key words: osteosarcoma, skull, chondroblastic

Introduction

Osteosarcomas arising from the skull are rare. In a survey of the literature 98 cases of primary osteosarcoma of the skull were found. Three large series have been reported. Skull osteosarcomas constituted 1.6-2% of osteosa-rcomas. Unlike osteosarcomas of the extremi-ties, those of the skull showed a tendency to develop in mature bone 1.

They occurred mainly in the third and fourth decades. Both no sex predominance and a fe-male predominance have been noted. The mean duration of complaints was less than a year. Skull osteosarcomas were more frequent in the vault than in the base. A lump with or without associated pain was the most common form of presentation.

In our cases one case involved the vault and the other the occipital region with skull base extension. Both patients were women in the third decade and both were histopathologically chondroblastic.

Case Reports

Case 1

A 28-year-old woman presented with a his-tory of left skull swelling, chronic head ache and right-sided limb weakness. On examination the patient had a left parietal giant bony hard swelling with adjacent scalp prominent vessels. Neurological examination revealed an alert, ori-ented patient with right-sided limb paresis with hypotonia. Her vitals were stable with normal respiratory, abdominal and cardiac examination.

Computed tomographic (CT) evaluation of the skull was carried out on a 64 slice GE CT scanner revealing a giant geographic sclerotic/lytic lesion involving the parietal bone on the left side with dense matrix mineralization (sunburst appearance) (Figures 1-4) showing mild contrast enhancement with gross mass ef-fect over the left cerebral hemisphere (parietal lobe) and a large outer extracranial scalp por-tion with associate prominent scalp arteries.

Histopathological examination (Figure 5) revealed chondroblastic grade 3 osteosarcoma showing lobules of malignant-appearing carti-lage with bone formation in the centre of the lobules.

List of Abbreviations:

MDCT: multi detector computed tomography;NCCT: non contrast computed tomography;HPE: histopathological examination

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Primary Skull Osteosarcoma: MDCT Evaluation and Histopathological Correlation in Two Cases K. Gangadhar

Figure 1 Coronal NCCT of cranium showing a giant geograph-ic sclerotic/lytic lesion involving parietal bone on left side with dense matrix mineralization (sunburst appearance) showing patchy central hypodense necrotic foci with gross mass effect over left cerebral hemisphere (parietal lobe) and large outer extracranial scalp portion.

Figure 2 Axial CECT of cranium showing giant geographic sclerotic/lytic lesion involving parietal bone on left side with dense matrix mineralization (sunburst appearance) showing mild contrast enhancement foci and patchy non-enhancing necrotic foci with gross mass effect over left cerebral hemi-sphere (parietal lobe) with displaced dural vessels and large outer extracranial scalp portion with associate prominent scalp arteries.

Figure 3 Axial CECT of cranium with bone window settings showing giant geographic sclerotic/lytic lesion involving pari-etal bone on left side with dense matrix mineralization (sun-burst appearance).

Figure 4 CECT volume rendered image showing dense matrix mineralization (sunburst appearance) and large outer extrac-ranial scalp portion with associated prominent scalp arteries evidently seen and displaced dural vessels as well.

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osteosarcoma arising out of Paget’s disease. Other bone abnormalities, such as fibrous dys-plasia, multiple osteochondromatosis, chronic osteomyelitis, myositis ossificans, and trauma, have also been proposed as risk factors 2.

The conventional type is a high-grade sar-coma. The usual appearance is that of a high-grade spindle cell neoplasm in which the tumor cells are associated with osteoid deposition. The osteoid is laid down in a lace-like pattern, very much like the inside of an osteoma. In fact, a well-differentiated osteosarcoma may be difficult to differentiate from an osteoblastoma. The difference is in the associated population of spindled or atypical cells and in the infiltrative periphery (best appreciated on x-ray). Osteosa-rcomas may have a wide range of morpholo-gies, including chondroblastic, fibroblastic, and small cell. The unifying and defining feature is the production of osteoid by tumor cells, but os-teoid may be sparse and focal. Some variants of osteosarcoma are more indolent. The parosteal osteosarcoma occurs on the surface of the bone, usually behind the knee of a young adult. This tumor is a low-grade sarcoma and is therefore not very cellular or atypical. It may resemble an osteochondroma, with well-formed cartilage and bone, but it is not in continuity with the marrow cavity. The similarly named but quite different periosteal osteosarcoma is also a sur-face lesion but is primarily chondroblastic and consists of a low-grade spindle cell population with cartilage formation. In our cases, both were chondroblastic type 3.

Radiologically osteosarcomas commonly have osteolytic and osteosclerotic features, a cloudy pattern of mineralization either diffuse or in clusters, and ill-defined borders 4. Radiating

Case 2

A 21-year-old woman presented with a his-tory of imbalance, nystagmus and right occipi-tal swelling. On examination the patient had ataxia and right occipital bony hard swelling with adjacent scalp boggy swelling. Her vitals were stable with normal respiratory, abdomi-nal and cardiac examination.

Computed tomographic (CT) evaluation of the skull was carried out with 64 slice GE CT scanner revealing a large geographic sclerotic/lytic lesion involving the occipital bone on the right side with dense matrix mineralization (sunburst appearance) (Figures 6 and 7) show-ing moderate contrast enhancement with gross mass effect over right cerebellar hemisphere (Figure 6) with mild skull base extension.

Histopathological examination (Figure 8) re-vealed high grade hyaline cartilage, which is intimately associated, and randomly mixed, with osteiod.

Discussion

In a survey of the literature around 100 cases of primary osteosarcoma of the skull were found, including our cases. The occur-rence of osteosarcoma in the craniofacial bones peaks in the third decade, whereas that in the skeleton peaks in the second decade. The etiol-ogy of osteosarcoma is unknown, but the major risk factors for its development in craniofacial bones may be similar to those of the long skel-etal bones, consisting of exposure to radiation, retinoblastoma, Li-Fraumeni syndrome, and Paget’s disease. The skull is a favored site for

Figure 5 HPE of chondroblastic osteosarcoma showing lobules of malignant-appearing cartilage with bone formation (arrow) in the centre of the lobules.

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Primary Skull Osteosarcoma: MDCT Evaluation and Histopathological Correlation in Two Cases K. Gangadhar

and MR scanning provide further details re-garding tumor structure and extent, namely to adjacent soft tissue, bone itself and neurovas-cular structures. A chest CT scan and a bone scintigraphy can be used to evaluate the exist-ence of lung and skeletal metastases respec-tively.

The differential diagnosis of cranial osteosa-rcoma to be considered includes chondrosar-coma and osteochondroma. The CT findings of new bone formation in the soft tissue mass and the characteristic matrix strongly suggest oste-

striations (sunburst appearance) and Codman’s triangle are signs of periosteal reaction due to periosteal elevation. These can be seen in pe-riosteal osteosarcomas and intramedullary os-teosarcomas with cortical extension, but not in paraosteal osteosarcomas. High grade surface osteosarcomas tend to have a more immature and basal pattern of mineralization. Para-os-teal osteosarcomas are usually dense sessile masses on the bone surface, with smooth or ir-regular margins. A thin radiolucent line may separate part of the tumor from the cortex. CT

Figure 8 HPE revealed high grade hyaline cartilage, which is in-timately associated, and randomly mixed, with osteoid (arrow).

↑ Figure 7 Axial CECT bone window settings showing a large geographic sclerotic/lytic lesion involving occipital bone on right side with dense matrix mineralization (sunburst appear-ance) with gross mass effect over right cerebellar hemisphere.

← Figure 6 Axial CECT showing a large geographic sclerotic/lytic lesion involving occipital bone on right side with dense matrix mineralization (sunburst appearance) showing moder-ate contrast enhancement with gross mass effect over right cerebellar hemisphere.

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size, stage, and the results of various labora-tory tests have been used in an effort to predict prognosis. However, response to pre-operative therapy is currently the most sensitive indi-cator of survival. At the same time, it is rec-ognized that a single system does not apply to all cases. Unique biological aggressiveness, coupled with an inability to completely resect the tumour at certain sites (e.g., skull, spine) is one example. There are certain sites (e.g., jaw, pelvis) in which response to therapy does not appear to reflect prognosis despite the capacity for complete surgical tumor removal.

Conclusion

The rarity of osteosarcomas of the skull makes it difficult to arrive at a definitive diag-nosis and treatment plan. When dealing with skull lesions, even if the typical rapid painful growth pattern is lacking or the supposedly ‘‘benign surface lesion’’ entourage is found, the neurosurgeon should have in mind this uncom-mon entity as a possibility.

Acknowledgements

We thank Shwetha, Sriram and Tarun Reddy for their kind support and encouragement.

ogenic sarcoma, but differentiation from chon-drosarcoma and osteochondroma cannot be made definitely without histology. Computed tomography in cases of chondrosarcoma of the skull base shows similar changes, but once the calvarium undergoes membranous ossification, it becomes an unlikely site for cartilaginous tu-mours. Osteochondromas usually are sharply defined and homogenous indensity 9.

Neoadjuvant chemotherapy (i.e. chemother-apy started before surgery) can be considered if a biopsy of the lesion is performed. Possi-ble advantages of neoadjuvant chemotherapy include: decrease in tumor size and a better chance of total surgical removal; and evalua-tion of response to drugs and change if inad-equate 5. Surgery and chemotherapy have been recommended for the management of skull osteosarcoma 6. A meta-analysis found im-proved patient survival using this strategy 5. A recently proposed protocol includes: surgery aimed at obtaining negative surgical margins; neoadjuvant chemotherapy when this is not at-tainable and for high grade lesions; and adju-vant radiotherapy when only close or positive surgical margins are achieved 7.

The identification of prognostic factors has been an additive process in which factors have been investigated, identified and incor-porated into an overall therapeutic strategy

8. Traditionally, age, gender, location, tumor

References

1 Mascarenhas L, Peteiro A, Ribeiro CA, et al. Skull os-teosarcoma: illustrated review. Acta Neurochir (Wien). 2004; 146: 1235-1239.

2 Ha PK, Eisele DW, Frassica FJ, et al. Osteosarcoma of the head and neck: A review of the Johns Hopkins experience. Laryngoscope. 1999; 109: 964-969.

3 Molavi DW. The Practice of Surgical Pathology: A Beginner’s Guide to the Diagnostic Process. Vienna: Springer Verlag; 2008. Chapter 28: Soft Tissue and Bone. p. 303.

4 Ayala AG, Ro JY, Raymond AK. Bone tumors. In: Damjanov I, Linder J eds. Anderson’s pathology. 10th edn. St. Louis: Mosby; 1996. p. 2531-2573.

5 Dorfman HD, Czerniak B. Bone Tumors. St. Louis: Mosby; 1998. p. 128-252.

6 Okada K, Unni KK, Swee RG, et al. High grade surface osteosarcoma. A clinicopathologic study of 46 cases. Cancer. 1999; 85: 1044-1054.

7 Patel SG, Meyers P, Huvos AG, et al. Improved out-comes in patients with osteogenic sarcoma of the head and neck. Cancer. 2002; 95: 1495-1503.

8 Anon. Age and dose of chemotherapy as major prog-nostic factors in a trial of adjuvant therapy of osteosa-rcoma combining two alternating drug combinations and early prophylactic lung irradiation. French Bone Tumor Study Group. Cancer. 1988; 61: 1304-1311.

9 Haque F, Fazal ST, Ahmad SA, et al. Primary osteosa-rcoma of the skull. Australas Radiol. 2006; 50: 63-65.

Kiran Gangadhar, MDResident RadiodiagnosisRoom 82, New doctors HostelInstitute of Medical ScienceBanaras Hindu UniversityVaranasiUttar Pradesh, INDIATel.: +91 9935402158Fax: +91 5139 3281E-mail: [email protected]

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INTERVENTIONAL NEURORADIOLOGYJournal of Peritherapeutic Neuroradiology, Surgical Procedures and Related NeurosciencesOfficial Journal of the World Federation of Interventional and Therapeutic NeuroradiologyOfficial Journal of the Asian & Australasian Federation of Interventional & Therapeutic NeuroradiologyOfficial Journal of the South American Working Group in Interventional and Therapeutic NeuroradiologyJournal sponsored by JSNET - Japanese Society of Neuro Endovascular Therapy

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Disclosure statement: This is not intended to prevent authors with potential conflicts of interest from contributing to NRJ. Rather, the Journal will place on record any relationship that may exist with dis-closed, or competing, products or firms. Disclosed information will be held in confidence during the review process and the Editors will exam-ine any printed disclosure accompanying a published article. Authors are responsible for notifying the Journal of financial arrangements in-cluding, but not limited to, agreements for research support including provision of equipment or materials, membership of speaker bureaus, consulting fees, or ownership interests. It is important that disclosure statements be updated promptly to reflect any new relationships that arise after initial submission of the manuscript. If the study is sup-ported by a commercial sponsor, the authors must document the input of the sponsoring agency in study design, data collection, analysis of results, interpretation of data, and report writing. It is important to specify whether the sponsors could have suppressed or influenced pub-lication if the results were negative or detrimental to the product they produce. Authors should also state if the company was involved in the original study design, the collection and monitoring of data, analysis and/or interpretation, and/or the writing and approval of the report.

Statement of human and animal rights: Manuscripts involving ex-amination of volunteers and patients must include a statement that the study protocol has been approved by an institutional review board (IRB) and that the subjects gave informed consent, thus meeting the standards of the Declaration of Helsinki (revised version) developed by the World Medical Association as a statement of ethical principles for medical re-search involving human subjects, including research on identifiable hu-man material and data (http://www.wma.net). With animal subjects, re-search should be performed based on a National or Institutional Guide for the Care and Use of Laboratory Animals, and ethical treatment of all experimental animals is expected throughout all experiments.

Duplicate/Redundant publication: NRJ only accepts manuscripts describing original research. The editorial office of the NRJ does not accept duplicate submission or redundant publication. Redundant (or duplicate) publication is publication of a paper that overlaps substan-tially with a paper already published in print or electronic media as defined by the updated ICMJE guidelines that cover allegations of sci-entific misconduct. When submitting a paper, authors should make a full statement to the Editor on all submissions and previous reports that might be regarded as redundant publication of the same or similar work. If authors believe that their manuscript may be considered re-dundant, they should address this issue in a letter to the Editor accom-panying the submission. The authors should also explain in the letter how their report overlaps already published material, or how it differs. Copies of such published material should be included with the submit-ted paper to help the Editor examine the possibility of redundant publi-cation. If redundant publication is attempted without such notification, authors should expect editorial action to be taken. At the very least, rejection of the manuscript may be expected.

Authorship: The Editors consider authorship to belong to those per-sons who accept intellectual and public responsibility for the statements

made and results reported. By submitting a manuscript for publication, each author acknowledges having made a substantial contribution to the concept and design of the study, the analysis and interpretation of the results, and the writing of the paper, in addition to having ap-proved the final submitted version. Authorship should not be attributed to Departmental Chairs not directly involved in the study, to physicians or technicians who provided routine services, or to technical advisors. A group study should carry the group name and reference contributing authors in the Acknowledgments.

Peer review processPapers are accepted on the understanding that they are subject to peer

review, editorial revision, and, in some cases, comment by the Editors. Manuscripts are examined by independent peer reviewers. Articles and other material published in the Journal represent the opinions of the au-thors and should not be construed to reflect the opinions of the publisher.

LanguageThe official language of NRJ is English. Most papers in NRJ have

been written by non-native English speakers, and they will also be read by many non-native speakers. For the purposes of clarity, it is strongly recommended that authors not fluent in English have their manuscript professionally edited for English usage prior to submission. A profes-sional editor will improve the English to ensure that author meaning is clear and to identify problems that require author review. Authors are invited to contact the NRJ language editor Anne Collins ([email protected]) for referral to professional English language editing services. Authors should make contact with and arrange payment to their chosen edit-ing service directly. Please note that the use of such a service is at the author’s own expense and risk and does not guarantee that the article will be accepted for publication. Centauro Publishers does not receive any commission or other benefit from editing services. Accordingly, Centauro Publishers neither endorses nor accepts any responsibility or liability for such editing services.

Copyright and purchase of Offprints All articles published in NRJ are protected by copyright, which em-

braces the exclusive right to reproduce and distribute the article (e.g., as offprints), as well as all translation rights. No material published in the Journal may be reproduced without written permission from the publisher. NRJ offers authors a complimentary hard copy and .pdf file of the issue in which their article is published, for personal use. To pur-chase offprints of articles published by NRJ please contact Centauro Srl for a quotation at [email protected].

Publication TypesOriginal Research Articles are full-length research papers, which are

favored by NRJ. Articles cover topics relevant to clinical studies, and may include both basic and experimental work.

Review Articles are comprehensive, state-of-the-art papers focusing on important clinical problems and should address a specific topic in a scholarly manner. Such articles may be invited by the Editor or may be unsolicited reviews.

Case Reports/Technical Notes should be unusually educational and medically important. Although NRJ preferentially encourages submission of full-length Original Research Articles, the Journal will consider publi-cation of a limited number of concise Case Reports and Technical Notes.

Editorials are usually invited by the Editor.Editorial Comments are specific comments on the articles published

in NRJ and are usually invited by the Editor.Letters to the Editor contain constructive comments or criticism of a

specific paper published by NRJ. Letters dealing with subjects of gen-eral interest within the field of NRJ, or personal opinions on a specific subject within the ambit of NRJ, may also be accepted.

Electronic submission of manuscriptsNRJ provides an electronic submission system (Editorial Manager)

and review process to promote expeditious peer review. Manuscripts should be submitted electronically to the following URL: http://www.editorialmanager.com/nrj/. Questions about manuscripts under consid-eration may be addressed to the editorial office.

The corresponding author listed on the manuscript must complete the forms in Editorial Manager, entitled Conflicts of Interest/Disclosures, Copyright Transfer Agreement, Financial Support, Exclusive Publica-tion Statement, and Author Contribution Form. Submissions not con-taining completed forms by the corresponding author will not progress to peer review. Prior to submitting any paper, please follow the instruc-tions given below. Please note that an author must have an e-mail ad-dress to use the online submission system.

Manuscript preparationIntroductions that Satisfy Reader Expectations– Overview: Once you have a revised draft, you need to ensure that

your Introduction frames it, so that your readers will understand where you are taking them. The Introduction should orient readers and moti-vate them to read the rest of the paper. The Introduction must also make

INSTRUCTIONS FOR AUTHORS First formulated in 1988 - Revised in February, 2011

a contract with the reader that a question will be answered. – Func-tions: – To awaken the reader’s interest. – To be informative enough to prepare readers to understand your paper. – Content and Organization: 1. Common ground ➜ Context ➜ Relevant background ➜ Orients the reader. 2. Disruption ➜ Problem ➜ Gap in Knowledge, Question ➜ Mo-tivates the reader. 3. Resolution ➜ Response ➜ Promise of an answer ➜ Makes a contract with the reader. 1. Common Ground: States the consensus, shared understanding, common ground in the field, what’s know and not known. Gives the reader context and provides relevant background information, not a literature review. 2. Disruption: States the problem/the question that the paper addresses. Conveys the signifi-cance, i.e. the cost of leaving problem unsolved, or the benefit of solving it. 3. Resolution: Implicitly promises that you will present your answer in the Results and Discussion. Try not to state the answer; that has the effect of closing off the paper rather than leading into it. When draft-ing: Although the Introduction unfolds in the above order, 1-2-3, when you’re drafting, write it in a 3-2-1 order. By writing the Introduction in this order, you are sure to work through and clearly set up your main point, the question that it answers, and to include only relevant back-ground information. When revising: “The key is to think like a reader -- readers expect and need a sense of structure. Since readers read each sentence in light of how they see it contributing to the whole, when you revise it makes sense to diagnose first the largest elements of the paper, then focus on the coherence of your paragraphs, the clarity of your sentences, and only last on matters of spelling and punctuation. Of course, in reality, no one revises so neatly; all of us revise as we go. But, it is useful to keep in mind that when you revise from the top down, from global structure to sections to paragraphs to sentences to words, you are more likely to discover useful revisions than if you start at the bottom with words and sentences and work up”. Booth WC, Colomb GG, Williams JM, et al. The Craft of Research. Chicago: University of Chicago Press; 2008.

Original research should be organized in the customary format, as described below. The text of the manuscript should be submitted as a single document with the following sections (in order):

Author Information Page (First page) Full title of the article, authors’ names, highest academic degree

earned by each author, authors’ affiliations, name and complete ad-dress for correspondence, address for reprints if different from address for correspondence, fax number, telephone number, and e-mail address.

Acknowledgments and Funding Page (Second page) The Acknowledgments section lists all funding sources for the re-

search of the study, and details substantive contributions of individuals. The authors must reveal all possible Conflicts of Interest/Disclosures.

Title Page (Third page) The full title, itemized list of the number of tables, the number and

types (color or black-and-white) of figures, and three-to-five key words for use as indexing terms should be included. Appropriate key words should be selected from the Medical Subject Heading. The word count of the text should be specified.

Summary A summary of up to 250 words should summarize the problems pre-

sented and describe the studies undertaken, results and conclusions. Since the abstract must be explicative, the abbreviations must be reduced to a minimum and explained. References should not be cited in the abstract.

Text Typical main headings include Introduction, Materials and Methods,

Results, Discussion, and Conclusions. Abbreviations must be defined at first mention in the text, tables and figures. The complete names and short addresses of manufacturers of any equipment used in Materials and Methods must be supplied. If animals are used in experiments, state the species, the strain, the number of animals used, and any other pertinent descriptive characteristics. If human subjects or patients are employed, provide a table with relevant characteristics. When describ-ing surgical or neurointerventional procedures on animals, identify the pre-anesthetic and anesthetic agents used, and state the amount or con-centration and the route and frequency of administration of each agent. Generic names of drugs must be given. Manuscripts that describe stud-ies on humans must indicate that the study was approved by an Insti-tutional Review Committee and that all subjects gave informed consent. Reports of studies on both animals and humans must indicate that all procedures followed were in accordance with institutional guidelines.

References Citations should be listed in order of appearance in the text, and between

square brackets [ ]. References must be listed at the end of the text in the order of citation. Journal titles should be abbreviated according to Index Medicus. For citation rules not specified here, authors should refer to the NLM Style Guide for Authors, Editors, and Publishers (http://www.nlm.nih.gov/citingmedicine). All references must be checked by the author(s).

Journal article:Laredo JD, Bard M. Thoracic spine: percutaneous trephine biopsy.

Radiology. 1986; 160: 485-489.

Journal article if the number of authors is more than six: list the first three authors followed by et al.

Geibprasert S, Krings T, Pereira V, et al. Clinical characteristics of dural arteriovenous shunts in 446 patients of three different ethnicities. Intervent Neuroradiol. 2009; 15: 395-400.

Entire book:Valavanis A. Medical radiology: interventional neuroradiology. Hei-

delberg: Springer Verlag; 1993. Part of book if number of authors is more than two: Bonneville JF, Clarisse J, et al. Radiologie interventionnelle. In:

Manelfe C ed. Imagerie du rachis et de la moelle. Paris: Vigot Editeur; 1989. p. 761-776.

Tables Each table must be typed on a separate sheet and double-spaced, if

possible. Tables should be numbered using the Arabic system, followed by a brief informative title. Use type of the same font and size as em-ployed in the text. Include footnotes at the bottom of each table. Tables must be numbered in the order cited in the text. Tables should not du-plicate data given in the text or figures.

Figure LegendsProvide figure legends on a separate sheet. Legends must be double-

spaced, and figures must be numbered in the order cited in the text. Figures Figures should preferably be submitted online in .tiff format to: http://

www.editorialmanager.com/nrj/. The time required to send files will vary depending on the number of figures, but image resolution must not be reduced to decrease transmission time. When labeling the figures, please ensure that the label corresponds to the figure number.

Digital images (originals or images acquired by a scanner) must meet the following criteria:

Black-and-white figures: Images must be acquired using the grey scale with a minimum resolution of 300 pixels per inch or 150 pixels per cm. Images must have a base of at least 8.1 cm for one item or a minimum base of 16.9 cm for several items.

Color figures: Images must be acquired using the full color CMYK method with a minimum resolution of 300 pixels per inch or 150 pixels per cm. Images must have a base of at least 8.1 cm for one item or a mini-mum base of 16.9 cm for several items. The RGB method is recommended for video reproductions only, as the quality of printed figures is poor.

Images must be saved in .tiff format. Images in .jpg format are not acceptable as details tend to be lost upon scanning, even at high resolu-tion. Image definition also depends on the enlargement factor. Thus, a large low-resolution image can be proportionally reduced (by 24%) for publication, thereby permitting optimal presentation in print. However, enlargement of a small high-resolution image will highlight all flaws, yielding a pixelated effect.

Do not submit figures already paged in Word, PowerPoint, or other documents, or images inserted in web pages. Such images are of low resolution and are unsuitable for printing.

Figures in .dcm (Dicom) format may be submitted as .dcm or .tiff files. The editorial office will process such images for printing.

Illustrations may be compressed using the StuffIt, Aladdin, or Zip programs.

Do not label an image with arrows, numbers, or letters, but rather in-dicate on a duplicate copy or a sketch where such indications are desired.

Authors are advised to refer to the Journal guidelines when format-ting their work. Otherwise the editorial office may return a submission to the authors for improvement before any Editor is assigned. If it is not possible to send figures via the Internet, images may be sent by express courier in one of the following formats:

Original x-ray films, slides, or glossy or opaque prints. Clearly indi-cate on the back of the image the first author’s name and the number corresponding to the figure caption and citation in the text.

Figures may be submitted on a CD-ROM or DVD, copied in ISO9006 format legible on both PC and MAC. Floppy disks are not recommended. Clearly indicate on one side of the figure both the top of the figure and the figure number. Do not label the actual image with arrows, numbers, or letters, but rather indicate the top and figure number on a duplicate copy or on a sketch. Do not cut or attach figures with adhesive tape or use paper clips. If images are sent offline, thumbnails of the images should be uploaded as a submission item online, together with the man-uscript, to allow the publishers to reserve space for the original high-resolution images. Authors are reminded that manuscripts must be sent online via Editorial Manager even when authors choose off-line figure submission. All off-line material should be sent to the editorial office:

NRJ Digital - The Neuroradiology Journal Centauro S.R.L.Via del Pratello, 8I-40122 Bologna – ItalyE-mail: [email protected]

Rivista di proprietà EDIZIONI DEL CENTAURO s.r.l. - Edita da CENTAURO s.r.l. - Sede legale: Via del Pratello, 8 – I-40122 BolognaC.F. e P. IVA IT 01896531207 – Cap. soc. Euro 15.600,00 i.v. – Reg. Impr. BO-R.E.A. BO n. 397358

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NRJ Digital - The Neuroradiology JournalCentauro s.r.l. - Via del Pratello, 8 - I-40122 Bologna - Italy - Fax: +39.051.220099 - E-mail: [email protected]

Manuscript No.: ________________________________________ Date: _______________

With respect to the article entitled: _________________________________________________________

The authors certify that:

(Conflicts of Interest/Disclosures) The corresponding author must disclose all relevant financial, personal, and/or professional relationships with other people or organizations related to the subject of this article. Is there any actual or potential conflict of interest in the subject of this article?

No (___). If yes, please give details in the space below.

_______________________________________________________________________________________________

(Copyright Transfer Agreement) All rights, title, and interest in the manuscript, including copy-right ownership, are transferred to Centauro Srl Publisher, Bologna. Authors reserve all proprietary rights (such as patent rights) other than copyright and the right to use parts of this article in their own subsequent work as soon as the article is accepted for publication. The article may not be published elsewhere without permission from the publisher.

(Financial Support) If the authors or their institution(s) is/are affiliated with any organization having a financial interest (including the provision of financial support) in the subject of this article, the authors should indicate (a) the name of the industry/institution providing support for the study, (b) the type of af-filiation of the organization (stockholder, consultant, donor of honorarium, granting body, source of other financial or material support, provider of equipment, or other support category), and (c) that the authors had complete control of the data and information submitted for publication. Any other non-financial con-flict of interest should also be disclosed to the Editor, with the understanding that the information may be published if deemed appropriate by the Editor.

No, I (We) do not have any conflict of interest related to this article (___).

If yes, (a) (_________) (b) (_________) (c) (_________).

(Exclusive Publication Statement) None of the material in this manuscript has been published pre-viously or submitted for publication elsewhere prior to appearance in NRJ.

(Author Contribution Form) The corresponding author must certify that all authors participated in and contributed sufficiently to the conception and design of the work, and analysis of the data, as well as the writing of the manuscript, and assume public responsibility for the content of the paper. The corresponding author also certifies that all authors have reviewed the final version of the manuscript and approved it for publication.

Name (Print) Signature Date

This signed statement must be received before the manuscript can be processed for publication.

Conflicts of Interest/Disclosures, Copyright Transfer Agreement,Financial Support, Exclusive Publication Statement, and Author Contribution Form

Via Nerviano, 3120020 Lainate (Mi)tel. +39 02 93305.1fax.+39 02 93305400www.abmedica.it

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