Malignant Gastric PEComa: A Rare Malignancy

Introduction: Perivascular epithelioid cell tumor (PEComa) is characterized by its perivascular location and spindle appearance of tumor cells with clear to lightly granular eosinophilic cytoplasm and a round-to-oval centrally located nucleus. Immunohistochemically, nearly all PEComas show reactivity for melanocytic (HMB-45 and/or melan-A) and smooth muscle (actin and/or desmin) markers. Malignant gastric PEComa is extremely rare and only 3 cases have been reported to best of our knowledge. We report a 4th case of malignant gastric PEComa.Case presentation: We are presenting a case of a 48 year-old Caucasian female who presented to the emergency department with complaint of abdominal pain for 3-4 weeks associated with intermittent nausea and vomiting. CT Abdomen/Pelvis with contrast showed a mass at the greater curvature of the distal stomach. Patient underwent resection of the mass and pathology result was consistent with malignant PEComa.Conclusion: PEComas are a family of rare mesenchymal tumors, composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These tumors co-express, the muscle and melanotic markers. Surgical resection is the best treatment option. Most commonly, they arise in the retroperitoneum and colon is most the common site followed by small intestine in the GI tract