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



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


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 (Name of Journal)

Journal of Cancer Prevention & Current Research