Perivascular epithelioid cell neoplasms: a systematic review of prognostic factors

Abstract

Perivascular epithelioid cell tumors (PEComas) are rare, soft tissue tumors characterized by epithelioid cells with clear or eosinophilic cytoplasm and a perivascular disbursement. We compiled the treatment and follow-up results from an extensive collection of reported gynecologic PEComa cases and statistically analyzed their respective therapy modalities and corresponding patient outcomes. In the group of patients with PEComa who received surgical management alone, there was a tendency for them to exhibit a lower disease recurrence rate. Conversely, patients with PEComa who initially received surgery and chemotherapy or radiation therapy were associated with a higher disease recurrence rate (P =.024). Metastatic involvement was related to higher patient mortality rates (P =.0001), although this finding was unrelated to treatment type. Surgical management alone may suffice for nonaggressive lesions, but chemotherapy and radiotherapy appear necessary for patients who present with high-risk histologic condition or metastatic disease. Because PEComas exhibit varying biologic behavior and an ill-defined presentation, the treatment for these lesions necessitates further evaluation.