Cavernous Sinus Ewing's Sarcoma

Sarcoma de Ewing do Seio Cavernoso

Ewing's sarcomas (ES) are malignant tumors mostly found in children and young adults^1,2,3. There are only six cavernous sinus ES reported in the literature^1,2,3,4,5.

A 21-year-old male presented with diplopia and left VI nerve palsy. Imaging revealed a lesion in the left cavernous sinus (Figure 1). A pretemporal craniotomy was performed with partial resection and recovery.

Figure 1
A. post-contrast T1 MRI showing a peripheral contrast enhancing ovoid lesion measuring 2.1 x 1.6 x 1.6 cm in the left cavernous sinus. The center of the lesion is heterogeneously enhanced with hypointense areas that may denote necrosis. B. FLAIR sequence MRI showing a hyperintense lesion. C. and D. post-contrast coronal T1 MRI showing the tumoral relationship to the carotid artery, sphenoid sinus and middle cranial fossa. Differential diagnosis includes meningioma, lymphoma, pituitary tumor, carcinoma, neuroblastoma, rhabdomyosarcoma, small round cell sarcoma and hemangioma

The tumor consisted of small round cells on histopathological examination (Figure 2), positive for CD99, CD56, BCL-2 and Ki 67 positive in 10% of the cells and were negative for desmin, synaptophysin, GFAP, S-100 protein, CLA, cytokeratin 7 and 20, 34BE12, AE1/AE3, consistent with Ewing's sarcoma.

Figure 2
Hematoxylin-eosin magnification x 40 showing an undifferentiated neoplasm, consisting of almost all small cells with oval or rounded nuclei, and scarce cytoplasm with indefinite limits. The cells are compactly arranged, without forming rosette structures.

References

• ¹
  Srivastava G, Jallo GI, Miller NR. Primary Ewing sarcoma of the cavernous sinus. Childs Nerv Syst. 2015;31(9):1583-8. https://doi.org/10.1007/s00381-015-2743-1
  » https://doi.org/10.1007/s00381-015-2743-1
• ²
  Patibandla MR, G Uppin S, Thotakura AK, Panigrahi MK, Challa S. Primary Ewings sarcoma of cavernous sinus in an infant: a case report and review of literature. Turkish Neurosurgery. 2013;23(1)98-103. https://doi.org/10.5137/1019-5149.JTN.4131-11.1
  » https://doi.org/10.5137/1019-5149.JTN.4131-11.1
• ³
  Kushen BSM, Gulbahce HE, Lam CH. Ewing's sarcoma of the cavernous sinus: case report. Neurosurgery. 2005;56(6):E1375.
• ⁴
  Idrees M, Gandhi C, Betchen S, Strauchen J, King W, Wolfe D. Intracranial peripheral primitive neuroectodermal tumors of the cavernous sinus a diagnostic peculiarity. Arch Pathol Lab Med. 2005;129(1):e11-15. https://doi.org/10.1043/1543-2165(2005)129<e11:IPPNTO>2.0.CO;2
  » https://doi.org/10.1043/1543-2165(2005)129<e11:IPPNTO>2.0.CO;2
• ⁵
  Pekala JS, Gururangan S, Provenzale JM, Mukundan Jr S. Central nervous system extraosseous Ewing sarcoma: radiologic manifestations of this newly defined pathologic entity. Am J Neuroradiol. 2006;27(3):580-3.

Publication Dates

• Publication in this collection
  Nov 2018

History

• Received
  02 Apr 2018
• Reviewed
  27 May 2018
• Accepted
  31 May 2018