Title

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

Document Type

Report

Department

Internal Medicine (East Africa); Pathology (East Africa); Imaging and Diagnostic Radiology (East Africa)

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.

Publication

Journal of Cancer Prevention & Current Research

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