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| CASE REPORTS |
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| Year : 2021 | Volume
: 1
| Issue : 1 | Page : 39-42 |
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Intracranial extraskeletal mesenchymal chondrosarcoma: A case report
Megha P Paramban, Vishnu Asokan, Apoorva Vijayan, Prasanth C Chandran
Department of Radiation Oncology, Government Medical College, Kozhikode, Kerala, India
| Date of Submission | 06-Sep-2021 |
| Date of Acceptance | 01-Nov-2021 |
| Date of Web Publication | 06-Jan-2022 |
Correspondence Address:
Dr. Vishnu Asokan
New PG Hostel, Department of Radiation Oncology, Government Medical College, Kozhikode 673 008, Kerala.
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/bjoc.bjoc_19_21
Background: Mesenchymal chondrosarcoma (MCS) is a rare and aggressive malignant tumor, a subtype of chondrosarcoma assumed to arise from remnants of the embryonic cartilage or metaplasia of meningeal fibroblasts. Intracranial extraskeletal MCSs are rare tumors, accounting for <0.16% of intracranial tumors. They usually occur in the age group of 20–30 years and are commonly found in the frontoparietal region arising from the falx and surrounding dura. Case Description: A 17-year-old girl presented with weakness of the right lower limb. Magnetic resonance imaging showed a large lobulated extra-axial mass lesion in the left parasagittal frontal region. She underwent a left frontoparietal craniotomy and excision of the tumor. She received adjuvant radiotherapy 60Gy/30 fractions. Conclusion: Intracranial MCSs are rare malignant tumors with a poor prognosis. The prognosis depends upon surgical resection and postoperative radiotherapy. Keywords: Chondrosarcoma, mesenchymal tumor, radiotherapy
How to cite this article:
Paramban MP, Asokan V, Vijayan A, Chandran PC. Intracranial extraskeletal mesenchymal chondrosarcoma: A case report. Bengal J Cancer 2021;1:39-42 |
| Introduction | |  |
Mesenchymal chondrosarcoma (MCS) is a rare and aggressive malignant tumor, a subtype of chondrosarcoma assumed to arise from remnants of the embryonic cartilage or metaplasia of meningeal fibroblasts.[1] Intracranial extraskeletal MCSs are rare tumors, accounting for <0.16% of intracranial tumors.[2] They are usually described as occurring in the age group of 20–30 years and are commonly found in the frontoparietal region arising from the falx and surrounding dura. It has a slight predominance in females.[3]
| Case History | | |
History and presentation
We present a history of a 17-year-old girl who presented with chief complaints of right-sided lower limb weakness of one-month duration with difficulty in walking. No history of headache, vomiting or seizure is reported. She had an uneventful past medical and surgical history. On examination, right lower limb power was grade 4. Muscle tone, bulk, reflexes and gait were normal. Bilateral cranial nerve examination was normal. Other system examinations were clinically normal. Complete laboratory investigations and cardiac evaluations were normal.
Radiological findings
Magnetic resonance imaging (MRI) brain findings revealed a large lobulated extra-axial mass lesion measuring ~6·8 × 7.8 × 5.2 cm involving the left parasagittal frontal region [Figure 1]. The lesion is hypointense on T1 with areas of blooming within. The lesion shows significant postcontrast enhancement. There is a mass effect and a midline shift toward the right side. The lesion is closely adherent to the superior sagittal sinus. | Figure 1: Preoperative magnetic resonance imaging scan of axial/coronal/sagital images
Click here to view |
Surgical treatment
She underwent left frontoparietal craniotomy and excision. The craniotomy revealed a grayish white lesion in the left parafalcine region broad-based to falx. The postoperative period was uneventful, and she recovered with no neurological deficit.
Histopathology[4]
Gross description: Lobulated, solid, firm, gray tan, fleshy mass with scattered gritty white calcification.
Microscopic description: Solid sheets of undifferentiated small blue cells [Figure 2] with a hemangiopericytoma like vascular pattern mixed with islands of mature appearing, well-differentiated hyaline cartilage [Figure 3].  | Figure 3: Islands of cartilage in the midst of undifferentiated small blue cells
Click here to view |
The final histopathology report was MCS. Imunohistochemistry profile is as follows: positive for S100 [Figure 4] and negative for epithelial membrane antigen and CD34 [Figure 5].
Radiotherapy treatment
A postoperative MRI showed a residual lesion measuring 2 × 2.8 cm in the frontal lobe broad-based to falx with intraparenchymal infiltration. She received adjuvant radiotherapy 60Gy in 30 fractions [Figure 6], one fraction per day, 5 days a week, started 1 month after surgery, by using a three-dimensional conformal radiotherapy technique. Target volumes were: tumor bed plus 1.5 cm margin as clinical target volume and clinical target volume plus 0.5 cm for planning target volume.
Follow-up
She was under regular follow-up and remained free of disease 2 years after treatment.
| Discussion | | |
MCS was first described by Lichtenstein and Bernstein. MC is a rare disease with a poor prognosis. It is a subtype of chondrosarcoma. It mostly occurs in young children and adults in the age group of 15–30 years. Four types of chondrosarcoma have been cited in the literature: grade I, grade II, mesenchymal, and myxoid. Mesenchymal variety has a higher proportion of extraskeletal tumors than conventional chondrosarcoma. It can arise from the soft tissues, especially the lower extremities, meninges, and orbit.[5] Intracranial extraskeletal MCSs are rare tumors.
The clinical presentation of MC depends on the location and size of the tumor. Diagnostic imaging is the first step in the workup of MC. MRI shows a soft-tissue mass that may be hypo to isointense on T1W imaging, iso or hyperintense in T2W MRI and intense heterogeneous enhancement after administration of gadolinium.[6],[7],[8] Computed tomography scan can also be taken. MRI is superior to computed tomography scan in lesion demarcation for surgical management and radiation planning. In this case, radiologically, the tumor resembled a meningioma.
Histologically, this tumor exhibits a biomorphic appearance of undifferentiated mesenchymal cells with islands of mature hyaline cartilage.[9]
Overall survival analysis of SEER database of MCS at 5 and 10 years was 51% and 43%. The presence of metastatic disease and increased tumor size are the main predictors of a poor survival outcome.[10]
Chen et al.[11] reported a similar case in a 13-year-old girl. There is no evidence of recurrence for at least 30 months after surgery. Diplopia and muscle weakness subsided considerably. No major neurologic deficit persisted after surgery. Among the reported cases, 20 patients died 3 days to 9 years after surgery (mean: 40 months). Fourteen patients had recurrence after surgery, whereas nine patients did not have recurrence 5 months to 4.5 years (mean: 23 months) after surgical resection.
| Conclusion | | |
MCSs are highly aggressive neoplasm and are difficult to differentiate on the basis of radiology. Histopathological examination and imunohistochemistry are keys for diagnosing such lesions. Radical excision is the optimal treatment of choice. Postoperatively, radiation can be given to patients to reduce recurrence. As these tumors are highly aggressive and invasive with the potential for recurrences and metastasis, the patient should be kept under regular follow-up.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 2. | Schneiderman BA, Kliethermes SA, Nystrom LM Survival in mesenchymal chondrosarcoma varies based on age and tumor location: A survival analysis of the SEER database. Clin Orthop Relat Res 2017;475:799-805. |
| 3. | Lin H-S, Tsai C-C, Chang C-K, Chen S-J Giant intracranial mesenchymal chondrosarcoma with uncal herniation. Formos J Surg 2012;45:93-6. |
| 4. | Shakked RJ, Geller DS, Gorlick R, Dorfman HD Mesenchymal chondrosarcoma: Clinicopathologic study of 20 cases. Arch Pathol Lab Med 2012;136:61-75. |
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| 6. | Kubota T, Hayashi M, Yamamoto S Primary intracranial mesenchymal chondrosarcoma: Case report with review of the literature. Neurosurgery 1982;10:105-10. |
| 7. | Heros RC, Martinez AJ, Ahn HS Intracranial mesenchymal chondrosarcoma. Surg Neurol 1980;14:311-7. |
| 8. | Yang BT, Wang YZ, Wang XY, Wang ZC Mesenchymal chondrosarcoma of the orbit: CT and MRI findings. Clin Radiol 2012;67:346-51. |
| 9. | Arora K, Riddle ND Extraskeletal mesenchymal chondrosarcoma. Arch Pathol Lab Med 2018;142:1421-4. |
| 10. | Bhardwaj S, Sharma A, Sharma A Mesenchymal chondrosarcoma of the brain with metastasis: A case report with literature review. J Neurosci Rural Pract 2020;11:344-8. |
| 11. | Chen JY, Hsu SS, Ho JT Extraskeletal intracranial mesenchymal chondrosarcoma: Case report and literature review. Kaohsiung J Med Sci 2004;20:240-6. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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