Brain Tumor Res Treat.  2021 Oct;9(2):75-80. 10.14791/btrt.2021.9.e10.

Intracranial Extraskeletal Myxoid Chondrosarcoma in Fourth Ventricle

• Hong YG
• Yoo J
• Kim SH
• Chang JH

• KMID: 2522223
• DOI: http://doi.org/10.14791/btrt.2021.9.e10

Abstract

We present an extremely rare case of intracranial extraskeletal myxoid chondrosarcoma. A 36-year-old male presented with dizziness persisting for 2 weeks. MRI of the patient showed well-enhanced mass of fourth ventricle. The tumor was totally removed under telovelar approach. Pathology results confirmed an intracranial extraskeletal myxoid chondrosarcoma. Adjuvant radiotherapy was initiated one month after the surgery, and MRI followed 3 months after initial operation and showed no evidence of tumor recurrence.

Keyword

Chondrosarcoma; Brain neoplasms; Extraskeletal; Myxoid; Fourth ventricle

Figure

• 

  Fig. 1 Preoperative MRI of the patient. A: T1-weighted enhanced axial image. B: T2-weighted axial image. C: T1-weighted enhanced sagittal image. D: T2-weighted sagittal image. The tumor shows strong homogenous contrast enhancement on T1-weighted enhanced images and moderate to high signal on T2-weighted images.

• 

  Fig. 2 Microscopic images of tumor. The tumor was removed via telovelar approach. A: Cerebellum is exposed after dura incision. B: Tela choroidea is exposed after retraction of right cerebelar tonsil and vermis. C: The tumor attached to the choroid plexus and the fourth ventricle floor is removed en bloc.

• 

  Fig. 3 Pathologic findings. A: Low power view (H&E stain, ×12.5) shows a somewhat well demarcated mass. B: In the mass, myxoid stroma (oval circle) is noted (H&E stain, ×200). C: High power view (H&E stain, ×400) shows haphazardly arranged spindle cells with myxoid stroma. D: The GFAP immunohistochemistry shows negative reaction (GFAP stain, ×400). These findings are compatible with extraskeletal myxoid chondrosarcoma. H&E, hematoxylin and eosin; GFAP, glial fibrillary acidic protein.

• 

  Fig. 4 Postoperative MRI at 3 months after surgery. T1-weighted enhanced axial (A) and sagittal (B) images show no residual tumor.

Reference

1. Scott RM, Dickersin R, Wolpert SM, Twitchell T. Myxochondrosarcoma of the fourth ventricle. Case report. J Neurosurg. 1976; 44:386–389. PMID: 1249620.

2. Smith TW, Davidson RI. Primary meningeal myxochondrosarcoma presenting as a cerebellar mass: case report. Neurosurgery. 1981; 8:577–581. PMID: 7266799.

3. Salcman M, Scholtz H, Kristt D, Numaguchi Y. Extraskeletal myxoid chondrosarcoma of the falx. Neurosurgery. 1992; 31:344–348. PMID: 1513440.

4. Sato K, Kubota T, Yoshida K, Murata H. Intracranial extraskeletal myxoid chondrosarcoma with special reference to lamellar inclusions in the rough endoplasmic reticulum. Acta Neuropathol. 1993; 86:525–528. PMID: 8310804.

5. Sala F, Talacchi A, Beltramello A, Iuzzolino P, Bricolo A. Intracranial myxoid chondrosarcoma with early intradural growth. J Neurosurg Sci. 1998; 42:159–163. PMID: 10192057.

6. Chaskis C, Michotte A, Goossens A, Stadnik T, Koerts G, D'Haens J. Primary intracerebral myxoid chondrosarcoma. Case illustration. J Neurosurg. 2002; 97:228. PMID: 12134922.

7. González-Lois C, Cuevas C, Abdullah O, Ricoy JR. Intracranial extraskeletal myxoid chondrosarcoma: case report and review of the literature. Acta Neurochir (Wien). 2002; 144:735–740. PMID: 12181708.

8. Im SH, Kim DG, Park IA, Chi JG. Primary intracranial myxoid chondrosarcoma: report of a case and review of the literature. J Korean Med Sci. 2003; 18:301–307. PMID: 12692436.

9. O'Brien J, Thornton J, Cawley D, et al. Extraskeletal myxoid chondrosarcoma of the cerebellopontine angle presenting during pregnancy. Br J Neurosurg. 2008; 22:429–432. PMID: 18568733.

10. Sorimachi T, Sasaki O, Nakazato S, Koike T, Shibuya H. Myxoid chondrosarcoma in the pineal region. J Neurosurg. 2008; 109:904–907. PMID: 18976082.

11. Dulou R, Chargari C, Dagain A, et al. Primary intracranial extraskeletal myxoid chondrosarcoma. Neurol Neurochir Pol. 2012; 46:76–81. PMID: 22426765.

12. Park JH, Kim MJ, Kim CJ, Kim JH. Intracranial extraskeletal myxoid chondrosarcoma: case report and literature review. J Korean Neurosurg Soc. 2012; 52:246–249. PMID: 23115670.

13. Qin Y, Zhang HB, Ke CS, et al. Primary extraskeletal myxoid chondrosarcoma in cerebellum: a case report with literature review. Medicine (Baltimore). 2017; 96:e8684. PMID: 29381948.

14. Velz J, Agaimy A, Frontzek K, et al. Molecular and clinicopathologic heterogeneity of intracranial tumors mimicking extraskeletal myxoid chondrosarcoma. J Neuropathol Exp Neurol. 2018; 77:727–735. PMID: 29924341.

15. Jroundi L, Kacemi L, Bounhir B, Chami I, Boujida N, Bacadi D. Intracerebral chondrosarcoma in a 12 year old child. J Radiol. 2005; 86:173–175. PMID: 15798629.

16. Korten AG, ter Berg HJ, Spincemaille GH, van der Laan RT, Van de Wel AM. Intracranial chondrosarcoma: review of the literature and report of 15 cases. J Neurol Neurosurg Psychiatry. 1998; 65:88–92. PMID: 9667567.

17. Sangoi AR, Dulai MS, Beck AH, Brat DJ, Vogel H. Distinguishing chordoid meningiomas from their histologic mimics: an immunohistochemical evaluation. Am J Surg Pathol. 2009; 33:669–681. PMID: 19194275.

18. Oliveira AM, Sebo TJ, McGrory JE, Gaffey TA, Rock MG, Nascimento AG. Extraskeletal myxoid chondrosarcoma: a clinicopathologic, immunohistochemical, and ploidy analysis of 23 cases. Mod Pathol. 2000; 13:900–908. PMID: 10955458.

19. Wernicke M, Piñeiro LC, Caramutti D, et al. Breast cancer stromal myxoid changes are associated with tumor invasion and metastasis: a central role for hyaluronan. Mod Pathol. 2003; 16:99–107. PMID: 12591961.

20. Harrison BT, Dillon DA. An update of mucinous lesions of the breast. Surg Pathol Clin. 2018; 11:61–90. PMID: 29413660.

21. Drilon AD, Popat S, Bhuchar G, et al. Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Cancer. 2008; 113:3364–3371. PMID: 18951519.

22. Stacchiotti S, Ferrari S, Redondo A, et al. Pazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: a multicentre, single-arm, phase 2 trial. Lancet Oncol. 2019; 20:1252–1262. PMID: 31331701.