Malignant Pervascular Epitheloid Cell Tumor (PEComa), Presenting as a Large Intra-Abdominal Mass in a Young Woman: A Case Report and Review of Literature

Abstract

Introduction: Perivascular epithelioid cell tumors (PEComa) are rare mesenchymal tumors of variable biologic behavior. Information on epidemiology, risks, treatment and prognosis is extremely limited. Case presentation:We are presenting case of a 20 years old girl, who presented with huge retroperitoneal PEComa, deemed un-resectable due to encasement of the major vessels and extreme friability. Treatment response was poor to Adriamycin based chemotherapy. She however responded to mTOR signaling pathway blocking therapy for a short time before gross progression and massive pleural effusion. Conclusion:Malignant PEComas are very rare mesenchymal tumor; there is a need of better understanding of pathophysiological and molecular pathways of this rare but potentially fatal malignancy in order to define the best possible treatments.