A rare case of metastatic proximal-type epithelioid sarcoma of the ischioanal fossa: case report and literature review

Epithelioid sarcoma (ES) is a rare high-grade sarcoma subtype that constitutes less than 1% of soft tissue sarcomas (STS).There are two types: distal-type epithelioid sarcoma and proximal-type epithelioid sarcoma, based on anatomic location and the histopathological features. The clinical presentation of ES is varied and can lead to a delay in diagnosis. histopathology examination followed by immunohistochemistry will help to establish the diagnosis. The treatment of choice of Localized ES is a radical excision with microscopically radical margins and perioperative radiotherapy. systemic therapies are used in cases of locally advanced or metastatic ES. We describe a case of reoccurring proximal-type epithelioid sarcoma of the ischioanal fossa. A 56-year-old man operated two years ago for a epithelioid sarcoma of the ischioanal fossa. The patient presented with reoccurring mass at the same location, Magnetic resonance imaging (MRI) of the pelvis showed a mass of the ischioanal fossa. computed tomography (CT) of the chest, abdomen, and pelvis showed multiple pulmonary and liver metastasis. Histopathological features and immunohistochemistry were those of proximal type epithelioid sarcoma. He received intravenous doxorubicin with a partial response after 3 cycles of treatment.