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British Journal of Neurosurgery, December 2012; 26(6): 912–914 © 2012 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2012.697219

/ ISSN 1360-046X online DOI: 10.3109/02688697.2012.697219 SHORT REPORT Intracranial mesenchymal chondrosarcoma: case

SHORT REPORT

Intracranial mesenchymal chondrosarcoma: case report and literature review

Zhisheng Kan, Hao Li , Ji Zhang & Chao You

Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China

Abstract Background. Mesenchymal chondrosarcoma is a very rare malignant cartilaginous forming tumour in central nervous system (CNS), which is rarely encountered in clinical practice and generally occurs in young adults. This article describes a case of primary intracranial mesenchymal chondrosarcoma in a 31-year-old woman and reviews the literature on its manifestations and management. Case report. This patient had suffered from severe headache, intermittent nausea and vomiting for 1 week. Systemic examination was unremarkable. Magnetic resonance imaging (MRI) demonstrated a giant, heterogeneous, intensely enhancing mass of 6 5 4 cm, occupying the bilateral frontal and based on the anterior falx cerebri, which was initially thought to be a simply meningioma. The patient underwent a bicoronal craniotomy and gross total resection of the tumour. Pathologic examination revealed the mesenchymal chondrosarcoma. Conclusion. Intracranial mesenchymal chondrosarcoma is an extreme rare neoplasm, which should be considered in the differential diagnosis of intracranial mass like a meningioma. We emphasize the importance of surgical intervention and combination of microsurgical resection and radiotherapy, it should be the therapeutical choice of the future.

Keywords: falx cerebri; mesenchymal chondrosarcoma; radiotherapy

Introduction

Mesenchymal chondrosarcoma of the central nervous sys- tem is uncommon neoplasms, which account for < 0.16% of all intracranial tumours and constantly arise from the basal synchondroses and can also arise from the meninges along the falx cerebri, tentorium and convexity. 1 This chondro- genic lesion is a malignant small round cell neoplasm with focal cartilaginous differentiation. We present a patient with intracranial mesenchymal chondrosarcoma, which was ini- tially thought to be a simply meningioma. This rare tumour should be considered in the differential diagnosis of young adults with an aggressive-appearing intracranial lesion.

Case report

A 31-year-old female patient was admitted to our department with a history of progressive headache and dizziness asso- ciated with intermittent nausea and vomiting for 1 month. She was an alert, well-oriented patient with normal physical findings, no recent generalized seizure and normalities in the high grade neurological activity on her initial evaluation. Neurological examination found no hemiparesis and sen- sory disturbance. Her past medical history was unremark- able. Computed tomography (CT) scan showed a bilateral frontal lobes, high-density lesion along the falx cerebri, with severe mass effect, perifocal edema and calcification sur- rounded by tumour. The collapse of the left lateral ventricle was also noted, suggesting a simply meningioma. Magnetic resonance imaging (MRI) demonstrated a well-demarcated parafalx mass with a 7.2 4.8 6.7 cm in size, occupying the bilateral frontal lobes. The lesion has a signal intensity higher than grey matter on T1-weighted images and slightly higher than grey matter on T2-weighted images. When gadolinium-diethylene triamine pentaacetic acid contrast was injected, obvious enhancement in non-calcified portions were detected (Fig. 1). Her preoperative symptom quickly disappeared through infusing mannitol after admission. Bicoronal craniotomy was performed with total resection of the tumour arising from falx cerebri and having no exten- sion into sagittal sinus. The mass which was reddish-brown and rubbery soft was enucleated piece by piece from the left side to the right through opening falx cerebri. The postopera- tive course of the patient was eventful without neurosurgi- cal deficit. The patient was discharged 5 days after surgery without postoperative adjuvant radiotherapy and then went back to her job as a lawyer. Microscopic examination of the tumour specimen revealed undifferentiated round or spindle-shaped cells and islets of cartilaginous tissue (hematoxylin & eosin, 200) (Fig. 2). Immunohistochemical studies demonstrated positivity for S-100 (Fig. 3), which is consistent with the diagnosis of mesenchymal chondrosar- coma. Postoperative serial MRI studies showed no evidence of recurrence 3 months.

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Br J Neurosurg Downloaded from informahealthcare.com Fig. 1. Preoperative MRI demonstrating a bifrontal mass with

Fig. 1. Preoperative MRI demonstrating a bifrontal mass with strongly intense enhancement, obvious mass effect, perifocal edema, the collapse of the lateral ventricle.

Discussion

Since the first report of extraskeletal intracranial chond- rosarcoma was reported in 1962, only a limited number of intracranial chondrosarcomas arising away from the base of skull have been reported in the literature. 2 Mesenchymal chondrosarcoma is a high-grade malignant tumour with a very high local recurrence and distant metastasis. Mesen- chymal chondrosarcomas of the falx cerebri, which account for approximately 1% of all chondrosarcomas, are character- ized as mesenchymal, classic and myxoid variants, and are extremely rare. Chondrosarcomas which arise at the skull base from the cartilaginous synchondroses, are usually located in the para- sellar region, the cerebellopontine and petrous bone region,

sellar region, the cerebellopontine and petrous bone region, Fig. 2. Histological sections of mesenchymal chondrosarcoma

Fig. 2. Histological sections of mesenchymal chondrosarcoma showing primitive undifferentiated cells possessed round or ovoid hyperchromatic nuclei and scanty, poorly outlined, eosinophilic cytoplasm (Hemotoxylin and eosin, 200).

Intracranial mesenchymal chondrosarcoma

913

eosin, 200). Intracranial mesenchymal chondrosarcoma 913 Fig. 3. Th e cartilaginous portion of the tumour was

Fig. 3. Th e cartilaginous portion of the tumour was positive for S-100 protein ( 200).

but occasionally from the meninges along the falx cerebri, tentorium and cerebral convexity. The peak age of incidence ranges between the second and third decades of life. Mes- enchymal, classic and myxoid chondrosarcomas differ from each other in terms of cytoarchitecture, natural history and prognosis. To date the origin of chondrosarcoma is still not clear. The embryonal cartilaginous rests in the cranial bones and dura, originate from the meningeal fibroblasts, or from the multipotent mesenchymal cells in the dura or arachnoid. Radiological examination including CT, MRI and angiography is essential for any intracranial lesion. CT scans usually show slightly hyperdense or isodense lesions with heterogeneous enhancement and frequent calcification in patients with chon- drosarcomas. Calcification in the centre of the tumour along cerebral falx is remarkable on CT in our patient. MRI which can define a possible neurovascular involvement and the extent of mass has an absolute significance on preoperative evaluation. Meningioma, oligodendroglioma and hemangiopericytoma should be involved in preoperative presumptive diagnosis. The treatment strategy is the radical removal of these les- sions, regardless of anatomic site, especially having a mar- gin with neurovascular structures. The majority of reported parafalx chondrosarcomas are mesenchymal type tumours, which have usually been described as highly vascular lesions, but the present case stood out from these as a poorly vascu- larized tumour. Total excision of the tumour was achieved without any neurological deficit after surgery. Postoperative adjuvant radiotherapy is a preferred treat- ment for the remnant of the lesion. When the invasive char- acter of chondrosarcoma was considered, some authors recommend adjuvant radiotherapy even after the success- ful radical resection. 3 Similar treatment of other sarcomas are available, postoperative adjuvant radiotherapy has been standard practice for mesenchymal chondrosarcoma. 4,5 The benefits of chemotherapy still remain to be not clear, because there is no current reliable evidence for its effec- tiveness. Generally, benefit from chemotherapy and/or radiation therapy is under investigation, but chemotherapy has been recommended by some authors for mesenchy- mal chondrosarcoma. 6,7 The factors of prognosis include the use of postoperative adjuvant radiation therapy,

mal chondrosarcoma. 6 , 7 The factors of prognosis include the use of postoperative adjuvant radiation

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914 Z. Kan et al.

pattern, previous treatment (surgery or radiation therapy) and extent of tumour removal. The overall 5 and 10-year survival for patients with mesenchymal chondrosarcoma, when considering all sites, is 55% and 27%, respectively. 8 Among the reported cases in parasagittal region, three patients died 3 days to 5 years after surgery. Two patients had recurrence after surgery. Our patient experienced no evidence of recurrence for at least 3 months after sur- gery and neurologic deficit. Her performance in job was the same as before. The mass in our patient was resected radically without radio-chemotherapy. Some authors rec- ommend that radiotherapy with or without concomitant chemotherapy should be considered for treatment of patients with unresectable mesenchymal chondrosarcoma and for those whom the surgical margins are inadequate. However, the potential benefits of radiotherapy and che- motherapy was under uncertainty because of the rarity of the tumour. Future development in the therapy will benefit from better knowledge of the biology of the tumour.

Conclusion

Intracranial mesenchymal chondrosarcoma of the falx is an extreme rare neoplasm, with a strong tendency to local aggression and distant metastasis. Differential diagnosis is very difficult because of its rarity and atypical imagine. The treatment strategy is radical resection with close postopera- tive follow-up, because it offers the greatest opportunity for a long-term, recurrence-free period for patients, although the

long-term outcomes may be disappointing. Radiotherapy and chemotherapy may be considered to reduce the risk of recurrence. We emphasize the importance of surgical intervention and combination of microsurgical resection and radiotherapy should be the therapeutical choice of the future.

Declaration of interest: Th e authors report no confl icts of interest. The authors alone are responsible for the content and writing of the paper.

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