Primary Ewing Sarcoma of Sphenoid Bone with Intracranial Extension: A Common Tumour at an Uncommon Location

• Main Authors: Guddi Rani Singh, Vijayanand Choudhary, Rawi Agrawal
• Format: Article
• Language: English
• Published: JCDR Research and Publications Private Limited 2017-02-01
• Series: Journal of Clinical and Diagnostic Research
• Subjects: cd99 (mic2); cranial tumour; primitive neuroectodermal tumor; small round cell tumour
• Online Access: https://jcdr.net/articles/PDF/9275/23541_CE[Ra1]_F(RK)_PF1(PI_RK)_PFA(AK)_PF2(AG_RK)_PF3(AG_OM).pdf

Primary Ewing Sarcoma of the cranial bone is rare, contributing to only 1% of all Ewing Sarcomas. Primary cranial Ewing Sarcoma occurs most commonly in temporal bone followed by parietal and occipital bones. Sphenoid bone is less commonly involved. We report a case of Ewing Sarcoma of the sphenoid bone with intra-cranial extension in a 20-month-old boy. On CT scan a provisional diagnosis of rhabdomyosarcoma was made. Fine Needle Aspiration Cytology (FNAC) and histopathological examination of core needle biopsy showed small round cell tumour. On Immunohistochemistry (IHC), CD99 (MIC2) and FLI 1 were strongly positive and final diagnosis of Ewing Sarcoma was made. Considering the rarity of this unusual site, we report a case of primary Ewing Sarcoma arising in the sphenoid bone with erosion of adjacent bones and intra-cranial extension.