Acta Neurochirurgica 78, 123-132 (1985) : A c t a . - 7 7 - - . Ndurochlrurglca �9 by Springer-Veflag 1985

Primary Cranial and Intracranial Chondrosarcoma A S u r v e y

M. Hassounah l, O. A1-Mefty 2, M. Akhtar 3, J. R. Jinkins 4, and J. L. Fox 2

1Division of Neurosurgery, The University of Alberta, Edmonton, Alberta, Canada, Departments of 2Neurosciences, 3 Pathology, and 4 Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

Summary

Fifty cases of intracranial and cranial chondrosarcoma were reviewed in the world literature including two of our own. These were analyzed relevant to their histological subgroup, site of origin, age and sex incidence, calcification and vascularity, recurrence, and metastases. The analysis was done in order to clarify points in the diagnosis, management, and prognosis of this rare tumour. The mesenchymal subtype is a more malignant form with a higher tendency for recurrence, metastasis, and increased vascularity.

Keywords: Myxochondrosarcoma; mesenchymal chondrosar- coma; cranial tumour; infratemporal approach.

Introduction

O n l y 0 .16% o f all c ran ia l a n d i n t r a c r a n i a l les ions are

c a r t i l a g i n o u s t u m o u r s . C h o n d r o s a r c o m a cons t i t u t e s

a b o u t 14% o f these c h o n d r o m a t o u s t u m o u r s 6. M o r t 31

in 1899 r e p o r t e d one ear ly case o f i n t r a c r a n i a l m y x o -

c h o n d r o s a r c o m a . N o su rge ry was d o n e a t t ha t t ime,

a n d the p a t i e n t d ied s o m e years a f t e r the onse t o f

s y m p t o m s . L i c h t e n s t e i n a n d Be rns t e in 28 in 1959 in t ro -

d u c e d the t e r m m e s e n c h y m a l c h o n d r o s a r c o m a , dif-

f e r en t i a t i ng it h i s to log ica l ly f r o m o t h e r c h o n d r o m a t o u s

t u m o u r s .

W e su rveyed 48 cases o f p r i m a r y i n t r a c r a n i a l and

c ran ia l c h o n d r o s a r c o m a in the w o r l d l i t e r a tu re 2 - 3, 5, 6, 8, 9,11,12,15-18, 20- 22, 24, 25, 27 - 47 a n d desc r ibe two m o r e cases

o f p r i m a r y c ran i a l c h o n d r o s a r c o m a . C h o n d r o s a r c o m a

o r i g i n a t i n g f r o m the o rb i t a n d the p a r a n a s a l sinuses,

s e c o n d a r y m e t a s t a t i c c h o n d r o s a r c o m a , a n d c h o n d r o s -

a r c o m a s a s soc i a t ed wi th Ol l i e r ' s d isease o r M a f f u z z i ' s

s y n d r o m e are exc luded in these stat is t ics .

Illustrative Cases

Case 1. A 33-year-old female presented on February t6, 1983 with a 20 months' history of headache, pain behind the right ear, nausea

and vomiting. She had blurring of vision for four months. There was no history of head trauma. On physical examination she was awake, alert, and orientated. There was bilateral papilloedema. Hearing was normal in both ears. The rest of the cranial nerves were intact. There was some difficulty in doing tandem walking but no other cerebellar signs or nystagmus were present.

Skull X-rays showed a large calcified lesion in the right posterior fossa. Computed tomography (CT) of the head revealed a calcified posterior fossa mass on the right with invasion from the right temporal and occipital bones (Fig. 1). The mass was avascular on cerebral angiography. With a microsurgical technique gross total removal was obtained through combined posterior fossa and in- fratemporal approaches. The tumour, located extradurally, was attached to the dura.

The tumour weighed 28 grams; measured approximately 5 x 4 x 3.5 cm; and consisted of soft, gelatinous, bluish and white tissue with occasional fragments of bone. Microscopically the tumour was composed of irregular and stellate-shaped cells with abundant myxoid stroma. The cytoplasm contained fine vacuolations and varied from scarcity to abundance. Nuclei, which were moderately hyperchromatic, were round to slightly irregular in shape and contained occasional mitotic figures. The neoplasm had a prominent lobular pattern (Fig. 2). Bony trabeculi were scattered within the tumour. The histological diagnosis was myxochondrosarcoma.

Postoperatively, a bone scan and metastatic workup failed to show extracranial involvement. The patient received 5,400 rads of high voltage X-radiation to the tumour bed. No further surgery or adjuvant chemotherapy was recommended. After three years the patient has shown no evidence of recurrence, clinically or radiologi- cally, and is quite well.

Case 2. A 65-year-old male, who had had a leftsided hearing loss and pain in the left ear for 5 years, underwent a left exploratory tympanotomy in another institution six weeks prior to his admission on June 13, 1984 at our hospital. The procedure was terminated because of profuse bleeding from the meatal incision. A tentative diagnosis of glomus jugulare tumour was made. There was no subsequent bleeding or otorrhea from the left ear, no pulsatile tinnitus, and no history of head trauma.

On examination there was a mass behind the left tympanic membrane. Audiometry revealed complete hearing loss in the left ear. The rest of the cranial nerves and neurological examinations were normal. Skull X-rays were normal. CT scan of the head demonstrated

124 M. Hassounah et al.: Primary Cranial and Intracraniai Chondrosarcoma

a large, enhancing lesion involving the left petrous pyramid and part of the mastoid bone and jugular foramen (Fig. 3A). Cerebral angiogram demonstrated a highly vascular tumour on the left at the base of the skull (Fig. 3B).

With microsurgical methods the tumour was totally removed through an infratemporal approach. The surgical specimen consisted of multiple fragments of bone, together measuring approximately 3 x 1 x 1 cm. Microscopic examination revealed bone extensively replaced by a malignant neoplasm containing a biphasic morpholog- ical pattern. Part of the tumour was composed of undifferentiated spindle-shaped cells with variable vascularity while other parts contained islands of moderately well-differentiated cartilaginous tissue (Fig. 4). The histological diagnosis was mesenchymal chondrosarcoma.

A metastatic workup done postoperatively failed to show any evidence of metastasis. Chemotherapy or radiation therapy was not recommended due to the low mitotic activity of the tnmour and because the tumonr was totally removed. Except for a partial facial paresis, the patient remains well 10 months after operation.

Discussion

Pr imary crania l and in t rac ran ia l c h o n d r o s a r c o m a is

a rare ma l ignan t neoplasm. The cl inical and pa tho log-

ical features o f 50 cases are summar ized in Tab. 1. The

exact site o f or igin in m a n y o f the cases is deba tab le

or unclear . I t appea r s tha t a b o u t ha l f o f the

c r an i a l / i n t r ac r an i a l c h o n d r o s a r c o m a s arise a long the

base o f the skull where the c h o n d r o c r a n i u m was fo rmed

f rom fusion o f a n u m b e r o f separa te cart i lages. Ossifi-

ca t ion occur red in la ter deve lopment . Bone des t ruc t ion

of ten seen by roen tgenog rams in this a rea also suggests

or igin f rom the chond roc ran ium.

Origin f rom a m e m b r a n o u s bone ( squamous por -

t ion o f the t e m p o r a l bone) was r epor t ed by Vil lani 44.

W h e n the tu rnou t has a t t a chmen t or invaded the falx,

t en tor ium, or dura l sinuses, the du ra is t hough t to be

the site o f origin. The meningea l f ibroblas ts which line

the undersur face o f the du ra and a r achno id were

t hough t by Alpers 1 to give rise to the car t i lag inous

tumours . In a rare case o f c h o n d r o s a r c o m a o f the

four th ventr icle descr ibed by Scot t 39, the tu rnou t was

pos tu l a t ed to have ar isen f rom the choro id plexus. This

ex t r a - ske le t a l or igin is best expla ined by the bel ief tha t

mesenchymal c h o n d r o s a r c o m a s arise f rom pr imi t ive

mul t i -po ten t i a l mesenchymal cells 14, 2s, 3s Bernstein

e t a l . 7 r epor ted a case o f c h o n d r o s a r c o m a o f the

Fig. 1. A) Lateral skull showing the irregular, ill-defined calcifica- tions overlying the posterior fossa. B) Axial section through the posterior fossa demonstrating the hypodense mass with internal areas of classic "popcorn" calcifications. C) Section at higher level again showing the typical calcifications within the hypodense tumour stroma

M. Hassounah et al.: Primary Cranial and Intracranial Chondrosarcoina 125

Fig. 2. Photomicrograph of neoplasm showing a lobulated pattern. The tumour cells are stellate-shaped and are surrounded by abundant mucoid intercellular material (Case 1). Hematoxylin and eosin stain x 150

Fig. 3. A) Post-IV contrast cranial CT demonstrating the lyric destructive process in the lateral petrous bone extending into the mastoid air cells with internal areas of either residual bone, or new bone formation. B) Lateral view of arterial DSA showing the marked tumour hypervascularity associated with this lesion

126 M. Hassounah e t al.: Primary Cranial and Intracranial Chondrosarcoma

Fig. 4. Photomicrograph of chondrosarcoma showing a biphasic pattern. Part of the tumour is composed of undifferentiated spindle-shaped cells while the other part reveals moderately well-differentiated cartilage (Case 2). Hematoxylin and eosin stain x 150

cerebellum 16 years after radiation treatment of a cerebellar astrocytoma.

In this review of 50 cases, 23 were mesenchymal, 6 were myxoid, and 21 were chondrosarcomas. Thus, mesenchymal chondrosarcoma appears to be the most common subtype among the cranial chondrosarcomas. Mesenchymal chondrosarcoma was first described by Lichtenstein and Bernstein in 1959. It is characterized histologically by a bimorphic pattern consisting of sheets of undifferentiated round or spindle-shaped cells and small or large islands of relatively well- differentiated cartilage. It is a relatively uncommon tumour. In a series of 6,221 benign and malignant bone tumours seen at the Mayo Clinic, 15 were mesenchymal chondrosarcomas, only one of which involved the cranial bone 10

Myxochondrosarcoma is the name suggested by Enzinger and Weiss 13 for a group of soft-tissue chondrosarcomas with a predominantly myxomatous pattern. Similar tumours have been reported in bone under the designation of choroid sarcoma, but it is now felt that myxochondrosarcoma is a more precise and generally preferred term. The number of reported cases of these tumours within the bone is still too small to allow a meaningful evaluation of their natural history and prognosis. However, in the extraskeletal location,

myxochondrosarcomas appear to have a better prog- nosis as compared to other types of chondrosarcoma.

In addition to the obvious histological differences between myxochondrosarcoma and mesenchymal chondrosarcoma, ultrastructural studies have revealed additional points of distinction 14. In myxochondrosar- coma the tumour cells contain abundant intra- cytoplasmic glycogen, a feature which is lacking in mesenchymal chondrosarcoma. The stroma in mes- enchymal chondrosarcoma is rich in collagen fibrils, while that in myxochondrosarcoma is devoid of these fibres 14.

In the 50 reported cases 10 had recurrences. Of these, eight were mesenchymal chondrosarcoma. Three of six cases with systemic metastases were mesenchymal chondrosarcomas. This supports the view that the mesenchymal subtype is a more malignant form with a poorer prognosis 19, 23, 38

Of the 50 cases included in this review, there were 23 males and 27 females. We concluded that there is no significant sex predilection except that 15 out of 23 mesenchymal ehondrosarcoma cases were female. The youngest patient was a 7-year-old male 38 and the oldest was a 65-year-old male (our case 2). Occurrence is more common during the second and third decades of life (11 and 19 patients in the second and third decades,

Tab

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M. Hassounah et al.: Primary Cranial and Intracranial Chondrosarcoma 131

respectively). The dura t ion o f symptoms is usually long

and insiduous, the longest being 20 years 5.

Nineteen out o f 34 (56%) patients with reported

radiological studies showed some form of calcification

o f the tumour on skull X-ray or CT scan of the head.

C h o n d r o s a r c o m a ranges in vascularity f rom avascular

which is the more common, to a highly vascular

neoplasm (10 out o f 34 cases, or 29%). Eight out o f the

10 vascular neoplasms were mesenchymal types. Heros

etal . 2~ suggested preoperat ive radiat ion therapy or

embolizat ion in lesions so vascular they resemble

arter iovenous malformat ions .

There is a general agreement that radical resection is

the t reatment o f choice for chondrosa rcoma of the

cranium. When the tumour is localized in the temporal

bone, the infra temporal approach described by K u m a r and Fisch 26 is quite useful. Adjunct radiotherapy and

chemotherapy could be added. The beneficial effect of

radiat ion therapy has been occasionally documented by

compar ing CT scans before and after treatment: a 26%

reduct ion in the volume o f the tumour and an increased

density (approaching that o f bone) o f the entire t umour has been reported 22. Minagi and Newton 3~ also de-

scribed extensive, coarse calcification following radia- t ion therapy. Suit e ta l . 42 achieved local control with

p ro ton beam therapy in some cases. Harsh and Wilson 19 concluded that i rradiat ion and chemotherapy

should be considered even when patients have a long disease-free period after radical excision. Huvos et al. 23

noted there appeared to be a histological subtype o f

mesenchymal chondrosa rcoma which is characterized

by undifferentiated, small cells and which responds to a

combina t ion o f chemotherapy and irradiation.

Twenty percent o f the cases have shown evidence o f recurrence 2 months to 13 years after the first opera-

tion. One patient survived two recurrences in 18 years, this being the longest reported survival o f a patient

with chondrosa rcoma 24. Extracranial metastasis o f

chondrosa rcoma occurred in 12% of the 50 cases reviewed. The blood stream is the main route. A m o n g

the six cases whose neoplasms metastasized four

tumours were vascular and two tumours were of u n k n o w n vascularity. There was no relationship be-

tween metastasis and dural sinus a t tachment o f the

pr imary tumour . There is some correlat ion between the frequency of mitotic figures and the likelihood of recurrence and metastasis 38. Finally, according to

Arlen e ta l . 4, chondrosa rcomas of the head and neck

occur in younger patients and their growth pat tern is less aggressive in compar i son with those o f the peri-

pheral chondrosarcomas .

Acknowledgement

The authors would like to acknowledge Ms. Virginia D. Lacson for her secretarial assistance in the preparation of this manuscript.

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Authors' address: Dr. O. A1-Mefty, Department of Neuro- sciences, King Faisal Specialist Hospital and Research Centre, P.O. Box 3354, Riyadh 11211, Saudi Arabia.