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|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 2 | Page : 214-216
A giant falcine chondrosarcoma: Case report and literature review
Yan-Wu Yang, Shuang Liu, Xiang Wang, Qing Mao
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China
|Date of Submission||22-Sep-2013|
|Date of Decision||17-Dec-2013|
|Date of Acceptance||31-Mar-2014|
|Date of Web Publication||14-May-2014|
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Yang YW, Liu S, Wang X, Mao Q. A giant falcine chondrosarcoma: Case report and literature review. Neurol India 2014;62:214-6
Parafalcine chondrosarcoma is extremely rare and may be difficult to differentiate preoperatively from falx meningioma. We report yet another such rare case.
A 26-year-old male presented with a 3-week history of progressive headache and right hand numbness. Neurological examination revealed decrease in proprioception in the right hand. Cranial magnetic resonance imaging (MRI) revealed a well-marginated parafalx mass (9.4 × 6.4 × 6.0 cm) occupying bilateral frontal lobes and along the falx cerebri. It was hypointense on T1-weighted and hyperintense on T2-weighted images [Figure 1]a with no perilesional edema. Contrast-enhanced MRI scan showed a lobulated heterogeneously enhanced honeycomb lesion [Figure 1]b. Preoperative diagnosis was parafalcine meningioma and MR venography (MRV) showed oppression of superior sagittal sinus by the tumor [Figure 1]c. Total resection of the tumor was performed by bicoronal craniotomy. The lesion was hard, lobulated, gray-white, with a very firm cartilaginous consistency. The tumor and the dural attachment were totally removed. Regular follow-up MRI scans at 18 months showed no recurrence [Figure 1]d. She has recovered well and is able to run again. Microscopic examination revealed lobulated appearance with variable low to moderate cellularity composed primarily of individual cells separated by a basophilic matrix. Neoplastic chondrocytes had plump and hyperchromatic nuclei with few mitotic figures [Figure 2]. Immunohistochemical studies demonstrated positivity for S-100, which is consistent with the diagnosis of a cartilaginous tumor [Figure 3].
|Figure 1: Imaging appearances of the intracranial chondrosarcoma. (a) Axial T2-weighted image reveals a bilateral frontal mass and has high signal intensity relative to cortex, with the low signal areas representing probable calcification (b) Axial contrast-enhanced T1-weighted image shows a lobulated heterogeneously enhanced tumor (c) MRV shows a part of the superior sagittal sinus that was oppressed by the tumor (d) There was no sign of recurrence at 18-month follow-up MRV = Magnetic resonance venography|
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|Figure 2: High-power view of the surgical specimen shows neoplastic chondrocytes with moderate cellular atypia with identifiable nuclei|
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|Figure 3: The cartilaginous portion of the tumor was positive for S-100 protein|
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Chondrosarcomas are malignant tumors of cartilage and may arise from bone or soft tissues.  They account for 6% of skull = base neoplasms and 0.15% of all intracranial tumors.  Approximately 75% of all cranial chondrosarcomas occurs at the base of the skull and mostly in the middle cranial fossa. , The rare locations include the following: Choroid plexus, dura mater, and intraparenchymal. Only 61 cases of dural origin have been reported to date and of them only nine were classic low-grade type. ,,, Skull-based chondrosarcomas are most commonly classic type,  but about 62% dural-based chondrosarcomas are mesenchymal variant.  The variants of parafalcine chondrosarcomas have a strong female preponderance, clinical symptoms at onset were similar to falx meningiomas and the preoperative diagnosis was frequently meningioma.
Chondrosarcomas are classified into the classic, mesenchymal, and myxoid subtypes based on the histologic architecture; these variants significantly differ in clinical presentation and prognosis. Classic chondrosarcoma is easily distinguished from the mesenchymal variant by its well-differentiated structure with few or no mesenchymal components and from the myxoid variant, by the absence of myxoid stroma. The mesenchymal subtypes are the most malignant of the three, illustrated by a strong tendency for intradural and cerebral growth, and occasionally distant metastases. 
On computed tomography (CT) scans, these lesions are usually isodense to hyperdense, with variable degrees of heterogeneous enhancement. These tumors have varying amounts of histologic and radiologic calcifications, but falcine chondrosarcomas show lesser calcification compared with skull-base variants. MRI offers better demarcation of the lesion and tumor characterization. These tumors are frequently hypointense on T1-weighted images and extremely hyperintense on T2-weighted images. They show some mild or moderate degree of contrast enhancement and may have a honeycomb appearance.  The absence of a dural tail, minimal vasogenic edema, and relative avascularity on angiography could be the characteristics for differential diagnosis.
The most important differentials include falx or parasagittal meningioma, hemangiopericytoma, metastasis, and vascular malformations. Higher signal intensity on the T2-weighted MRI, islands of low signal intensity despite lack of gross calcification on all the sequences due to well-differentiated cartilage, "honeycomb" appearance on contrast enhancement, and lack of perfusion may differentiate chondrosarcomas from meningiomas.  The relative absence of edema can also be used to distinguish it from tumors such as glioblastomas and metastases. Lack of vascularity and absence of flow voids differentiate it from a vascular malformation or the mesenchymal variety. To specially mention, none of the MRI or CT features appear to be useful for differentiating chordomas from chondrosarcomas preoperatively. 
Radical surgical resection is the preferred treatment modality. The widest possible resection seems to be a significant prognostic factor. Chondrosarcomas at the skull base have a strong tendency for local recurrence due to the difficulty of complete resection. In a report by Korten et al.,  53% of patients had recurrence attributed to partial resection due to the proximity of the lesion to critical neuronal and vascular structures. However, no recurrences have been reported to date in dural-based classical chondrosarcomas. ,,, Mesenchymal and myxoid subtype indicates worse clinical behavior and poorer prognosis than conventional histology. About 50% of the mesenchymal and nearly all the myxoid variants showed recurrence.  Some authors recommend adjuvant radiation therapy to prevent recurrence, but it is difficult to tell if this mode of adjuvant therapy is effective because the few cases prohibit drawing any conclusions about such treatment.
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[Figure 1], [Figure 2], [Figure 3]