International Clinical and Medical Case Reports Journal (ISSN: 2832-5788) | Volume 2, Issue 4 | Case Report | Open Access

Extraskeletal Ewing’s Sarcoma of the Infratemporal Fossa with Orbital and Intracranial Extension: A Rare Tumor in A Rare Location: A Case Report

Khadija LAASRI*

Pediatric Radiology Department, Rabat Children’s Hospital, Ibn SinaHospital, Mohamed V University, Rabat-Morocco

*Correspondence to: Khadija LAASRI 

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Abstract

Extraskeletal Ewing’s Sarcoma is a rare soft tissue tumour morphologically similar to the commoner Ewing’s Sarcoma arising from bone. Head and neck origin of this tumour is rarely observed with infratemporal fossa Ewing’s Sarcoma forms another uncommon subset. We report a case of Extraskeletal Ewing’s Sarcoma in the infratemporal fosssa in a 5-year-old girl; who presented with a rapidly growing mass in the left cheek. CT scan of neck and head showed soft tissue mass in left infratemporal fossa with intraorbital and intracranial extention. Histopathological examination of the biopsy specimen showed round cell tumour and immunohistochemistry was positive for CD99 and Vimentin. According to the clinical manifestation, CT findings, histological pattern and the results of the immunohistochemical studies, the final diagnosis was Extraskeletal Ewing’s Sarcoma. Our patient was initially treated with chemotherapy. The patient responded very well to chemotherapy. For the management suite, a radiotherapy is programmed. To our knowledge, this is the second case of EES in infratemporal fossa in paediatric age group. Early and certain diagnosis coupled with combined surgical excision and modern chemotherapy/radiotherapy seems to be the most effective treatment plan.

Keywords:

Extraskeletal Ewing’s Sarcoma; Infratemporal fossa; Child; CT scan; Immunohistochemistry

Citation:

Khadija LAASRI, Meriem ZHIM, Salma MARRAKCHI, Taha Yassine Aaboudech, Najat Lamalmi, Laila Hessissen, et al. Extraskeletal Ewing’s Sarcoma of the Infratemporal Fossa with Orbital and Intracranial Extension: A Rare Tumor in A Rare Location: A Case Report.Int Clinc Med Case Rep Jour. 2023;2(4):1-9.