Academy of Medical Sciences of I.R. Iran Archives of Iranian Medicine 1029-2977 28 12 2025 12 01 Pancreatic PEComa: Case Report of an Extremely Rare Tumor 719 722 10.34172/aim.34740 EN Dmitry Zinovkin https://orcid.org/0000-0002-3808-8832 Denis A. Davydov Pavel G. Kisialeu Diana A. Kolbik Sergey L. Achinovich Anna S. Portyanko Md Zahidul Islam Pranjol https://orcid.org/0000-0002-6164-6281 Journal Article 10.34172/aim.34740 2024 07 12 2025 09 12 Pancreatic perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with only a few reported cases. Their non-specific clinical presentations and imaging features often lead to misdiagnosis. We report a case of a 63-year-old female with intermittent left upper quadrant pain. Imaging revealed a hypervascular mass in the pancreatic tail, initially suspected to be a neuroendocrine tumor. The patient underwent distal pancreatectomy with splenectomy. Histopathological examination showed that the tumor consisted of epithelioid and spindle cells with clear cytoplasm, a rich vascular network and low mitotic activity. Immunohistochemically, the tumor cells were positive for HMB-45, Melan-A, and smooth muscle actin, confirming the diagnosis of pancreatic PEComa. The postoperative course was uneventful. Given the uncertain malignant potential of PEComas, complete surgical excision is the preferred treatment option, with long-term follow-up recommended. This case highlights the diagnostic challenges of pancreatic PEComas and underscores the role of histopathology and immunohistochemistry in their accurate identification and management.