Intracranial mesenchymal chondrosarcoma

Aeta neuropath, (Berl.) 24, 86--89 (1973) 9 by.Springer-Verlag 1973 Kuvze Originalmitteilungen 9 Short Original Communications Communications originales br~ves

Intracranial Mesenchymal Chondrosarcoma H. N. Seth a n d M a h e n d r a Singh Neurosurgical Centre Army Hospital, Delhi Cantt Received September 25, 1972

Summary. A case of mesenchymal chondro-sarcoma originating from the skull base and presenting as an intraeranial space occupying lesion with multiple cranial nerve paralysis is reported. This is the 52rid case of this tumour in the literature. Key words: 3~esenchymal Chondrosarcoma -- Intraeranial Space Occupying Lesion. M e s e n c h y m a l e h o n d r o s a r c o m a s are r a r e b u t h i g h l y characteristic t u m o u r s . T h e r e are o n l y 51 eases in t h e literature. L i c h t c n s t e i n a n d Bernstein (1959) described t h e first two examples. D a h l i n a n d H e n d e r s o n (1962) r e p o r t e d t e n cases f r o m M a y o Clinic. S a l v a d o r et al. (1971) p r e s e n t e d d e t a i l e d o b s e r v a t i o n s on 30 new eases from t h e same i n s t i t u t i o n a n d r e v i e w e d t h e existing literature. The remaining nine cases a r e single case r e p o r t s b y B e n e d e t t i (1961), Chaves (1965), G o l d m a n (1967), Nezelof et al. (1965), R a s k i n d a n d G r a n t (1966), R e e h (1966), W i r t h a n d S h i m k i n (1943), W u a n d L a p i (I970) a n d D o M i n g (1964) whose r e p o r t on t w o eases i n c l u d e d one from series of D a h l i n a n d H e n d e r s o n (1962). W e r e p o r t a n o t h e r case of this t u m o n r , which p r e s e n t e d as a n i n t r a c r a n i a l t u m o u r w i t h m u l t i p l e cranial nerve i n v o l v e m e n t .

Case Report Male, aged 32 years, developed pain along the distribution of the right trigeminal nerve in March 1968. By March 1971 he had developed VIth cranial nerve palsy with squint and diplopia, followed by paralysis of I I I r d cranial nerve on right side in Aug 1971. X-Ray Chest and skull, pneumoencephalogram and cerebrospinal fluid were normal. On right carotid angiography there was a suspicion of a median temporal mass. He left hospital against medical advice. In March 1972, he lost vision in both eyes, first of the right, all in a week's time. He reported to Neurosurgical Centre in April 1972. Examination: Alert and oriented though completely blind. There was a diffuse painless swelling over right subtemporal region. Right eye showed a non-pulsating proptosis with no visual acuity and complete corneal sensory loss. The right III, IV and VI cranial nerves were paralysed. Except for loss of vision, left eye was normal. No papilloedema. There was nasal blockage right side due to a mass filling the right maxillary antrum. Investigations: X-Ray skull showed erosion of the floor of right middle cranial fossa. Right carotid angiogram showed a large vascular tumor at the floor of middle cranial fossa extending into the right maxillary antrum. The unilateral non-pulsatile irreducible exophthalmos, complete ophthalmoplegia, blindness both eyes and the angiographie findings suggested possibility of a rather extensively spreading sphenoidal ridge meningioma. Biopsy from the maxillary growth was diagnosed as undifferentiated malignant growth on frozen section. Possibility of meningioma was ruled out. Exploratory eraniotomy was deferred pending final histologic diagnosis which was mesenchyreal ehondrosarcoma.

Intracranial Chondrosarcoma

Fig. 1

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Fig. 2

Fig. 1. Undifferentiated cellular tumour enclosing easily identifiable chonch'oid area. H.E. • 200 Fig.2. Undifferentiated cellular tumour with haemangiopericytoid features. In many sections no cartilage differentiation was seen. H.E. • 200

Operation: In May 1972 through a right subtemporal craniotomy an extensive tumour extending medially along the floor of middle cranial fossa, anteriorly to superior orbital fissure, downwards into the maxillary antrum through pterygomaxillary fossa was excised piecemeal. The tumour was extradural. Post Operative: A course of radiation therapy was given. No change in vision and ophthalmoplegia. Proptosis showed improvement. Pathological Examination: Multiple fleshy soft pieces of tissue 4 • 5 cm. No haemorrhage or necrosis. Histology from multiple areas sampled showed structure of a mesenchymal chondrosarcoma with highly cellular areas enclosing foci of abrupt chondroid transformation (Fig. i). Perivascular clusters with haemangiopericytoid feature were also seen (Fig.2). A hitherto undescribed finding was isolated single chondroid cells in the loose oedematous myxoid stroma of the tumor (Fig. 3). Spotty calcification was present.

Comments Salvador et al. (1971) have reviewed the c u r r e n t s t a t u s of these t u m o u r s . T h e y c o m m o n l y occur in the second or t h i r d decade with a female p r e p o n d e r a n c e (57~ Of the 51 t u m o u r s 34 were skeletal i n origin a n d 17 (330/0) arose i n soft tissues. The p a t t e r n of neurological i n v o l v e m e n t a n d operative findings suggest origin of this t u m o u r from the base of the skull i n the region of f o r a m e n ovale.

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Fig.3. Single isolated chondroid cell in the ioosr myxoid fibrillary stroma of the tumour. No chondroid differentiation is seen. H.E. • 450

H i s t o l o g i c a l l y these t u m o u r s should be differentiated from m o r e cellular varieties of e h o n d r o s a r c o m a where c h o n d r o i d differentiation is m o r e g r a d u a l a n d n o t so a b r u p t as in these t u m o u r s . The cartilage cells in c h o n d r o s a r c o m a s are larger a n d show g r e a t e r p l e o m o r p h i s m . T h e y h a v e a p e a k incidence in t h e sixth decade, a n d a m u c h lower p e r c e n t a g e arise in soft tissues. H a e m a n g i o p e r i c y t o m a a n d o t h e r sarcomas also m a y sometimes be confusing. H a l l m a r k of m e s e n c h y m a l chondros a r c o m a s are h i g h l y u n d i f f e r e n t i a t e d small or r o u n d cells a l t e r n a t i n g w i t h zones of easily identifiable cartilage (Salvador et al., 1971). Clinical course is variable. S u r v i v a l h a s r a n g e d from 5 to 23 y e a r s (Dahlin a n d Henderson, 1962). T h e t u m o u r is c h a r a c t e r i s e d b y r e p e a t e d local recurrence a n d wide s p r e a d metastases. R a d i c a l surgical e x t i r p a t i o n p r o m p t l y u n d e r t a k e n is t h e t r e a t m e n t of choice (Dowling, 1964). R a d i a t i o n is o n l y palliative. References Benedetti, G. B. : Tumore condroblastico della mandibola a caratteri istologici peculiari. Chir. Organi Mov. 50, 135--144 (1961). Chaves, E. : Condrosarcoma mesenquimal. Hospital (Rio de J.) 67, 1217--1225 (1965). Dahlin, D. C., Henderson, E. D. : Mesenchymal chondrosarcoma: further observations on a new entity. Cancer 15, 410--417 (1962). Dowling, E. A. : Mesenchymal chondrosarcoma. J. Bone J t Surg. A 46, 747--754 (1964). Goldman, R. L. : "~escnchymal" chondrosarcoma, a rare malignant chondroid tumor usually primary in bone: report of a case arising in extraskeletal soft tissue. Cancer 20, 1494--1498 (1967). Lichtenstein, L., Bernstein, D. : Unusual benig and malignant chondroid tumors of bone: A survey of some mesenchymal cartilage tumors and malignant chondroblastic tumours, including a few multicentric ones, as well as many atypical benign chondroblastomas and chondromyxoid fibromas. Cancer 12, 1142--1157 (1959). l~ezelof, C., Mazabraud, A., Meary, R. : Le chondrosareoma mesenchymateux: Apropos d'un cas de loealisation para-rachidienne. Arch. Anat. loath. ]8, 26--28 (1965).

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l~askind, I~., Grant, S. : Primary mesenchymal chondrosarcoma of the cerebrum: report of a case. J. Neurosurg. 24, 676--678 (1966). l~eeh, M. J.: Hemangiopericytoma with cartilaginous differentiation involving orbit. Arch. 0phthal. 75, 82--83 (1966). Salvador, A. H., Beabout, J. W., Dahlin, D. C. : Mesenchymal chondrosarcomas--observations on 30 new cases. Cancer 28, 605--615 (1971). Wirth, J. E., Shimkin, M. B.: Chondrosarcoma of the nasopharynx simultating juvenile angiofibroma. Arch. Path. 86, 83--88 (1943). Wu, W. Q., Lapi, A. : Primary non-skeletal intracranial cartilaginous neoplasma: report of a ehondroma and a mesenehymal chondrosareom~. J. Neurol. Neurosurg. Psychiat. 88, 469--475 (1970). Lt Col It. H. Seth Army Hospital Delhi Cantt-10, India