47 Year Old Female with a Recurrent Abdominal Perivascular Epithelioid Cell Tumor: Case Report and Literature Review

Abstract

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by histologically and immunohistochemically distinctive epithelioid or spindle cells. These tumors express both smooth muscle markers and melanocytic markers. They can arise in various anatomical locations throughout the body, including the gastrointestinal tract, genitourinary system, retroperitoneum, and soft tissues. The exact cell of origin for these tumors remains unidentified. Clinical outcomes vary widely, spanning benign to malignant behavior, with the potential for local recurrence and metastasis. The management of PEComas typically involves surgical resection, though use of adjuvant chemotherapy and immunotherapy has been reported. The high recurrence rate of PEComas highlights the necessity for long-term surveillance and a multidisciplinary approach to treatment. We present the case of a 47-year-old female with a previous abdominal PEComa, who presented to our clinic with an enlarged recurrent abdominal tumor. Confirmation of recurrence was obtained through ultrasound and PET-CT imaging. Additionally, post-operative pathology findings supported the diagnosis.

Keywords

• Perivascular epithelioid cell neoplasms
• Recurrence
• Mesenchymal neoplasms

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