Journal of Pediatric Oncology, 2014, 2, 3-9 3

E-ISSN: 2309-3021/14 © 2014 Pharma Publisher

Congenital Posterior Mediastinal Teratoma with Intraspinal Extension

Mohammed Basamh1, Fahad A. Alghamdi2, Osama Rayes3 and Saleh S. Baeesa*,1

Division of Neurosurgery1, Department of Pathology

2, and Division of Pediatric Surgery

3, Faculty of Medicine,

King Abdulaziz University, Jeddah, Saudi Arabia

Abstract: Congenital teratoma is the commonest tumors of the nervous system, which are predominantly midline located. However, their spinal location is extremely rare. We present a case of a female twin newborn with huge right

side mediastinal tumor presented afterbirth with a chest infection. She underwent complete surgical resection via thoracotomy. Histopathological examination revealed immature teratoma. She had complete respiratory recovery but presented three months later with progressive paraparesis due to intrapinal tumor. She underwent thoracic laminectomy

and complete excision for what turned out histopathologically to be mature teratoma. She recovered well from surgery and received adjuvant chemotherapy over one year. Her 5-year follow up revealed a healthy child with no recurrence of the mediastinal or intraspinal teratoma on regular imaging surveillance.

We report herein a rare congenital posterior mediastinal teratoma with intraspinal extension and discuss the clinical

features, imaging studies, histopathological examination, and management.

Keywords: Congenital teratoma, Mediastinal teratoma, Spinal cord tumor, Spinal teratoma.

INTRODUCTION

Teratoma is a multipotential cell tumor consisting of

all three primitive germ cell components that further

classified, based on the degree of differentiation, into

mature, immature or malignant types based on the

degree of cellular differentiation [1]. They may originate

anywhere along the midline; however, teratoma has

predominance to sacrococcygeal, gonadal, medias-

tinal, retroperitoneal, cervicomandibular and intracra-

nial locations.

Mediastinal teratomas are commonly seen in

children and accounting 8% to 13% of all mediastinal

tumors [2] and Up to 75% of are mature type [3].

Intraspinal teratomas are extremely rare occurrence

accounting of 0.15% of adults and 4-10% of pediatric

spinal tumors (Table 1), and occur either as an

extradural extension from adjacent paraspinal origin

(Table 2) [4-7], or as an isolated intradural origin [8-14].

We aim to report a newborn presented with

congenital mediastinal immature teratoma with

extradural spinal extension of mature cystic teratoma

and discuss the management.

CASE REPORT

A newborn, monozygotic twin, girl presented since

birth with respiratory distress while feeding. She and a

healthy twin sister were a product of an uneventful

*Address correspondence to this author at the Division of Neurosurgery, Faculty of Medicine, King Abdulaziz University, P.O. Box 80215, Jeddah 21589, Kingdom of Saudi Arabia; Fax: +966-12-6408469; E-mail: sbaeesa@kau.edu.sa

pregnancy that was ended with cesarean section at the

37th

week. She was brought to a polyclinic 2 weeks

later with low-grade fever and cough. Her temperature

was 37.5oC and her physical exam revealed no

cyanosis, but with right lung basal crepitation and

decreased air entry. Her pulse Oxymetry revealed 90%

oxygen saturation and complete blood count (CBC)

was normal. Chest radiography demonstrated right

lower lung opacity, initially reported as pneumonia, and

she was discharged on oral antibiotics.

Table 1: Summary of Intraspinal Tumors Series Reported in the Literature

Author Number of

Cases Size of series

Percentage of cases

Sloof et al. 2 1,322 0.15%

Black and German

1 142 0.7%

Oi and Raimondi

2 64 3.1%

Matson 13 134 9.7%

Hendrick 3 80 3.75%

Kordas et al. 2 80 2.5%

DeSousa et al.

31 81 3.7%

Average 3.4%

Abbreviations: *Series of children,

1Authors reported 6 intraspinal teratoma

cases of 81 intraspinal tumors; 3 of 6 were originating from the sacral area that was not included.

She improved for a week but presented to our

emergency department with fever and progressive

respiratory distress. General physical examination

revealed a temperature of 38.5oC, tachypnea (34

4 Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 Basamh et al.

B/min) and tachycardia (96 B/min), with no central

cyanosis, and she had marked decreased air entry in

the right lung. The neurological exam was within

normal.

She was admitted to pediatric intensive care unit,

intubated, and started on IV antibiotics and bronchio-

lytic medications. Routine laboratory investigations

including; CBC, coagulation, renal, hepatic profiles

Table 2: Reported Cases in the Literature of Posterior Mediastinal Teratoma with Spinal Extension

Year Author Age Sex Symptoms Level Pathology Treatment Result

1961 Howard [8] 4y M Paraplegia with normal

sensations Thoracic Teratocarcinoma

Palliative decompression

Died 49 d

1981 Kolandaivelu

[9] 3.5y M

Paraplegia sensory level at T9

Thoracic, epidural

Benign teratoma GTR GR

1998 Kondo [10] 42y M Left Brown-Séquard

syndrome Thoracic, epidural

Adenocarcinoma in teratoma

GTR MR

2004 Schneider [11] NA NA NA Thoracic

Teratoma with malignant

transformation, recurred as epidural

benign teratoma

NEC + GTR, then 2

nd

resection NED

Abbreviations: NA=not available, d=days, GR= good (neurological) recovery, GTR= gross total resection, M= male, MR= minimal (neurological) recovery, NEC= neo-adjuvant chemotherapy, NED= no evidence of disease, y=years.

Figure 1: Coronal (A), Sagittal (B), and axial (C) CT scans demonstrating large tumor occupying the right hemi-thorax with

marked mass effect and shift of the right lung and mediastinal contents.

Congenital Posterior Mediastinal Teratoma with Intraspinal Extension Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 5

were within normal. Computed tomography (CT) scan

revealed a large right hemi-thorax heterogeneous mass

occupying the posterior mediastinum that measures 6.5

x 5.5 cm with areas of cystic and solid components

(Figure 1).

Serum tumor markers including serum alpha-

fetoprotein (AFP) and beta-human chorionic gonado-

tropin (B-HCG) levels were unremarkable.

The patient’s general and respiratory condition was

optimized, and then she was taken to the operating

room 48 hours later where surgery was performed

through right-side thoracotomy by pediatric surgery

team. A large posterior mediastinal capsulated reddish

brown tumor was encountered, which was adherent,

but not infiltrated, to pericardium and aorta; there was

no spinal extension. The patient tolerated the surgery

well, extubated after two days and had a smooth

complete recovery of her respiratory dysfunction and

discharged home after two weeks with a postoperative

CT scan showing no residual tumor. Histopathological

examination of the mediastinal specimen revealed

immature teratoma composed of neuroblastic tissue

with mature elements of mesodermal and ectodermal

origin (Figure 2).

Figure 2: Histopathology of the mediastinal specimen

demonstrating immature teratoma contain an immuture

neublastic tissue (A), and mature elements like respiratory

ciliated columnar epithelium and colonic glands (B).

The patient was followed up in pediatric surgery and

oncology clinics where adjuvant chemotherapy was

recommended.

During this time, reached her 4-month of age, she

was brought to the emergency department with rapidly

progressing decreased movement of her lower

extremities for 2 weeks. Her neurological examination

revealed normal mental function for age and cranial

nerves. She moved her upper extremities normally but

had flaccid paraparesis of motor grade 2 out of 5 and

absent reflexes. Urgent magnetic resonance imaging

(MRI) scan of the spine demonstrated significant cord

compression by a heterogeneous thoracic intraspinal

cystic mass extending from fourth to seventh thoracic

vertebral level (T4 to T7) that measured 3.2 x 1.4 cm,

and associated with paraspinal and ribs extension

through more than one foramina (Figure 3). MRI scan

of the brain and CT scan of the chest and abdomen

was within normal.

The patient was started on IV steroids and taken to

the operating room where thoracic laminectomy, from

T4 to T8, was performed. The tumor was located in the

epidural space extending to 2 intervertebral foraminae

but without extraforaminal extension. It was relatively

vascular encapsulated reddish-brown tumor, which was

easily dissected from the dura and completely removed

using microsurgical ultrasonic aspiration.

Histopathological examination of the spinal

specimen revealed a mature cystic teratoma composed

of three germ lines. Variable cysts were lined by

ciliated columnar epithelium, mature cartilage, vessels,

fat, stratified squamous epithelium and glial tissue

(Figure 4).

The patient had smooth postoperative recovery and

discharged in a good condition and showed

progressive neurological improvement and normal

recovery achieved after one month.

She received six cycles adjuvant chemotherapy

using modified JEB protocol (Carboplatin, Etoposide,

and Bleomycin), which were well tolerated and followed

up regularly in the clinic.

At her fifth anniversary, she remained with normal

neurological examination and development, and her

MRI scan of the spine (Figure 5) and CT scan of the

chest (Figure 6) revealed no recurrence. Worth to

mention here is that her twin has been evaluated with

head to spine MRI scans that revealed no similar or

other tumors.

6 Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 Basamh et al.

Figure 3: Sagittal (A) and axial (B) T2-WI MRI of the spine demonstrating intraspinal heterogeneous mass with marked spinal

cord compression.

Figure 4: Histopathology of the intraspinal specimen demonstrating mature teratoma contains respiratory ciliated columnar

epithelium (A), mature cartilage (B), stratified squamous epithelium (C), and adipose tissue (D).

Congenital Posterior Mediastinal Teratoma with Intraspinal Extension Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 7

Figure 5: 5-year follow up sagittal (A) and axial (B) T1-WI MRI scan demonstrating no recurrent tumor.

Figure 6: 5-year follow up axial CT (A) and axial MRI (B) scans demonstrating normal restoration of lung and mediastinal

structures and no recurrent tumor.

DISCUSSION

Teratoma is most common germ cell tumors in the

pediatrics age group [15]. The anterior mediastinum is

the commonest extragonadal site for these tumors

while it occurs in the posterior mediastinum only in 3-

8% of all mediastinal teratomas [16,17]. The posterior

mediastinal tumor may bulge unilaterally or bilaterally

from the usual midline contours to involve the spine [5].

They are also common in the sacrococcygeal spinal

region but otherwise very rare in the rest of spine [18].

Spinal teratomas are rarely occurring tumors, excluding

the relatively common sacrococcygeal form, constitute

less than 10% of all pediatrics intraspinal tumors.

Within the spinal canal, teratomas are most commonly

intradural [19] and,

particularly in children, are

associated with spina bifida [20].

Kondo et al., presented a case of 42-year-old man

who had a thoracic extradural immature teratoma [6]. It

developed over a period of more than 10 years after an

operation for a posterior mediastinal immature

teratoma. Another reported case by Schneider et al, of

an adult patient presented with epidural mature

teratoma occurring after chemotherapy and excision of

8 Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 Basamh et al.

posterior mediastinal teratoma with malignant

transformation [7]. These two cases have in common

the fact of late occurrence of the epidural tumors

following the resection of posterior mediastinal

teratomas. Furthermore, the latency for the epidural

tumor to appear clinically after their excision of the

mediastinal mass was 27 weeks and 10 years. Our

patient presented after 12 weeks of resection of the

posterior mediastinal teratoma.

CT scan is the modality of choice to detect

mediastinal teratomas in general [16], as it reveals the

extent of the heterogeneous mass containing a mixture

of soft tissues, fat, calcifications and/or teeth. However,

in cases of intraspinal teratomas, as in other spinal

tumors, magnetic resonance imaging (MRI) scan plays

a more important role because it delineate better the

tumor and surrounding neural anatomy. The

morphological appearances of intraspinal teratomas in

MRI scans are commonly oval or lobulated

heterogeneous masses, whereas extradural teratomas

were more commonly found to be dumbbell-shaped

and are commonly accompanied with vertebral body

malformation.

Tumor markers, including serum -human chorionic

gonadotropin ( -hCG) and -fetoprotien (AFP), were

applied for the diagnosis and prognosis of recurrences

of teratomas in general; however, this application was

limited in the mature teratomas as the recurrence may

have originated from non-secreting parts of the

previous lesion [7,21]. However, the diagnostic efficacy

of AFP and its role in predicting survival or altering

outcomes remains in question as we yet lacking

adequate data in this regard. Moreover, AFP since birth

is normally secreted, and its level declines gradually

and eventually plateaus between the 9th

-12th

months

[21].

An extensive sectioning and thorough histopatho-

logical examination is mandatory to avoid missing

immature or malignant tissues and preventing

misdiagnosis. This is because these immature and

malignant tissues are found in close relation to the

mature components as demonstrated in our case

[18,22].

The primary treatment for teratomas is complete

surgical excision, which may also be applied to mature

teratomas. The management of immature teratoma

remains an issue of some controversy due to its rarity,

potential missed malignant component within larger

lesions and the fact that adult patients are included in

some series in spite of their different disease course

[23]. Surgical resection of immature teratoma with

close follow-up and reserving platinum-based

chemotherapy for recurrence resulted in 98.6% 3-year

tumor free survival in a small study and seems,

therefore, a safe approach. The current standard

therapy for extragonadal nonseminatious germ cell

malignancies includes platinum-based chemotherapy

followed by resection of residual tumor [7]. The JEB

protocol was found to be more effective and less toxic

than other protocols [24]. Immature mediastinal

teratomas were observed to behave more aggressively

in adolescents and younger adults, while they are of a

benign course during infancy [25]. The combined

review of the Pediatric Oncology Group (POG) and

Children’s Cancer Study Group (CCG) reported the

grade of immaturity as the only valid risk factor for

recurrence in pediatrics immature teratoma regardless

of the site or age group [26]. This was due the strong

correlation between higher degrees of immaturity and

microscopic foci of malignant endodermal sinus

tumors.

CONCLUSION

Spinal teratomas, excluding the sacrococcygeal

location, are relatively rare. The epidural location is

even less common, with the posterior mediastinum

being an extremely rare origin. We recommend to role

out intraspinal involvement in any case with posterior

mediastinal tumor with MRI scans. Complete resection

and thorough histopathological examination are key

elements in the management of these patients. The

role of chemotherapy in immature spinal teratoma

should be further investigated.

REFERENCES

[1] Willis RA (ed): Teratomas. In: Atlas of Tumour Pathology. 1st edition. Armed Forces Institute of Pathology, Washington, District of Columbia, pp. 9-58, 1951.

[2] de Castro MA Jr, Rosemberg NP, de Castro MA, et al.

Mediastinal teratoma mimicking pleural effusion on chest X-rays. J Bras Pneumol 2007; 33:113-115.

[3] Nichols CR. Mediastinal germ cell tumors. Clinical features and biologic correlates. Chest 1991; 99:472-479. http://dx.doi.org/10.1378/chest.99.2.472

[4] Howard FH, Michael IK, Devere W. Teratocarcinoma of the

posterior mediastinum. JAMA 1961; 175: p. 240-242. http://dx.doi.org/10.1001/jama.1961.63040030010017b

[5] Kolandaivelu G, Govindan R. Posterior mediastinal teratoma with spinal compression. Indian J Chest Dis Allied Sci 1981; 32(1): p. 44-46.

[6] Kondo M, Hokezu Y, Yanai S, Nagamatsu K, Kida H. A case

of thoracic extradural spinal cord teratoma with neurological sequelae more than 10 years after surgery. Rinsho Shinkeigaku 38: 693-696, 1998 (In Japanese).

Congenital Posterior Mediastinal Teratoma with Intraspinal Extension Journal of Pediatric Oncology, 2014, Vol. 2, No. 1 9

[7] Schneider BP, Kesler KA, Brooks JA, Yiannoutsos C,

Einhorn LH. Outcome of patients with residual germ cell or non-germ cell malignancy after resection of primary mediastinal nonseminomatous germ cell cancer.J Clin Oncol

2004; 22 (7): 1195-1200. http://dx.doi.org/10.1200/JCO.2004.07.102

[8] Sloof JL, Kernohan JW, McCarty CS. Primary Intramedullary Tumors of the Spinal Cord and Filum Terminale. WB Saunders, Philadelphia, PA, pp134, 1964.

[9] Black SW, German WJ. Four congenital tumors found at

operation within the vertebral canal, wit observations on their incidence. J Neurosurg 1950; 7: p. 49-61. http://dx.doi.org/10.3171/jns.1950.7.1.0049

[10] Oi S, Raimondi AJ. Hydrocephalus associated with intraspinal neoplasms in childhood. Am J Dis Child 1987; 135: p. 1122–1124.

[11] Matson DD, Tachdjian MO. Intraspinal tumors in infants and children; review of 115 cases. Postgrad Med 1963; 34: 279-285.

[12] Hendrick EB. Spinal cord tumors in children, in Youman’s neurological surgery, Y. J, Editor. Saunders: Philadelphia. 1982; pp. 3215-3221.

[13] Kordás M, Paraiez E, Szénásy J. Spinal tumors in infancy and childhood.Zentralbl Neurochir 1977; 38(3): 331-7.

[14] DeSousa AL, Kalsbeck JE, Mealey J Jr, Campbell RL, Hockey A. Intraspinal tumors in children. A review of 81 cases. J Neurosurg 1979; 51: 437-445. http://dx.doi.org/10.3171/jns.1979.51.4.0437

[15] Lo Curto M, D'Angelo P, Cecchetto G, Klersy C, Dall'Igna P,

Federico A, Siracusa F, Alaggio R, Bernini G, Conte M, De Laurentis T, Di Cataldo A, Inserra A, Santoro N, Tamaro P, Indolfi P. Mature and immature teratomas: results of the first

paediatric Italian study. Pediatr Surg Int 2007; 23 (4): p. 315-322. http://dx.doi.org/10.1007/s00383-007-1890-1

[16] Moeller KH, Rosado-de-Christenson ML, Templeton PA.

Mediastinal mature teratoma: imaging features. AJR Am J Roentgenol 1997; 169(4): 985-990. http://dx.doi.org/10.2214/ajr.169.4.9308448

[17] Sinclair DS, Bolen MA, King MA. Mature teratoma within the posterior mediastinum.J Thorac Imaging 2003;18 (1):53-55. http://dx.doi.org/10.1097/00005382-200301000-00010

[18] Dillard BM, Mayer JH, McAlister WH, McGavrin M,

Strominger DB. Sacrococcygeal teratoma in children. J Pediatr Surg 1970; 5 (1): 53-59. http://dx.doi.org/10.1016/0022-3468(70)90520-8

[19] Li Y, Yang B, Song L, Yan D. Mature teratoma of the spinal cord in adults: An unusual case. Oncol Lett 2013; 6 (4): 942-946.

[20] Suri A, Ahmad FU, Mahapatra AK, Mehta VS, Sharma MC, Gupta V. Mediastinal extension of an intradural teratoma in a patient with split cord malformation: case report and review of

literature. Childs Nerv Syst 2006; 22 (4): 444-449. http://dx.doi.org/10.1007/s00381-005-1240-3

[21] Tsuchida Y, Endo Y, Saito S, Kaneko M, Shiraki K, Ohmi K. Evaluation of alpha-fetoprotein in early infancy. J Pediatr Surg 1978; 13 (2): 155-162. http://dx.doi.org/10.1016/S0022-3468(78)80010-4

[22] Massie RJ, Van Asperen PP, Mellis CM. A review of open biopsy for mediastinal masses. J Paediatr Child Health 1997; 33 (3): 230-233. http://dx.doi.org/10.1111/j.1440-1754.1997.tb01585.x

[23] Billmire DF. Malignant germ cell tumors in childhood. Semin

Pediatr Surg 2006; 15 (1): 30-36. http://dx.doi.org/10.1053/j.sempedsurg.2005.11.006

[24] Mann JR, Raafat F, Robinson K, Imeson J, Gornall P, Phillips M, Sokal M, Gray E, McKeever P, Oakhill A: UKCCSG's

germ cell tumour (GCT) studies: improving outcome for children with malignant extracranial non-gonadal tumours--carboplatin, etoposide, and bleomycin are effective and less toxic than previous regimens. United Kingdom Children's

Cancer Study Group. Med Pediatr Oncol 1998; 30 (4): p. 217-227. http://dx.doi.org/10.1002/(SICI)1096-911X(199804)30:4<217::AID-MPO3>3.0.CO;2-J

[25] Carter D, Bibro MC, Touloukian RJ. Benign clinical behavior of immature mediastinal teratoma in infancy and childhood: report of two cases and review of the literature. Cancer. 1982; 15; 49 (2): 398-402.

[26] Heifetz SA, Cushing B, Giller R, Shuster JJ, Stolar CJ,

Vinocur CD, Hawkins EP. Immature teratomas in children: pathologic considerations: a report from the combined Pediatric Oncology Group/Children's Cancer Group. Am J

Surg Pathol 1998; 22 (9): 1115-1124. http://dx.doi.org/10.1097/00000478-199809000-00011

Received on 22-02-2014 Accepted on 24-03-2014 Published on 30-06-2014

DOI: http://dx.doi.org/10.14205/2309-3021.2014.02.01.1

© 2014 Basamh et al.; Licensee Pharma Publisher. This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.