Abstract

Perivascular epithelioid cell tumors (PEComas) are a group of mesenchymal neoplasms with different biological presentations from benign to malignant types. Hence, we report the first description of PEComa presenting with an acute surgical abdomen. Her definite diagnoses were made based on the histomorphology results at Imam Khomeini Hospital, Tehran University of Medical Sciences, in 2020. Considering different clinical presentations, unknown characteristics of imaging, and rarity of PEComa, preoperative diagnosis of it seems impossible. However, IHC can play an important role in its diagnosis. © 2022.