Uterine perivascular epithelioid cell tumour (PEComa): a case report and literature review

Abstract

Case report A 45-year-old female with a history of a renal angiomyolipoma and complex partial seizures, presented with menorrhagia. Following pelvic ultrasound detection of a uterine fundal vascular lesion, a laparoscopic hysterectomy and bilateral salpingectomy was performed. Multiple small ill-defined pale areas admixed with variably sized blood vessels located within the myometrium were identified on cut-up. Histology showed perivascular epithelioid cell tumours (PEComas), confirmed by the demonstration of immunopositivity for HMB45, Melan-A, smooth muscle actin (SMA) and desmin. Of note, the tumour cells showed scattered tumour multinucleated giant cells and coupled with the irregular and infiltrative margins; it was further classified as to have an uncertain malignant potential. Discussion PEComas are uncommon mesenchymal tumours but are being increasingly recognised, with the uterus being one of the more frequent sites. Studies have proposed histological criteria to assess the aggressive behaviour of such PEComas and stratify them according to clinical outcome. We reviewed the current literature and discussed the features of this tumour, emphasising specific histological features which suggest malignancy. A 45-year-old female with a history of a renal angiomyolipoma and complex partial seizures, presented with menorrhagia. Following pelvic ultrasound detection of a uterine fundal vascular lesion, a laparoscopic hysterectomy and bilateral salpingectomy was performed. Multiple small ill-defined pale areas admixed with variably sized blood vessels located within the myometrium were identified on cut-up. Histology showed perivascular epithelioid cell tumours (PEComas), confirmed by the demonstration of immunopositivity for HMB45, Melan-A, smooth muscle actin (SMA) and desmin. Of note, the tumour cells showed scattered tumour multinucleated giant cells and coupled with the irregular and infiltrative margins; it was further classified as to have an uncertain malignant potential. PEComas are uncommon mesenchymal tumours but are being increasingly recognised, with the uterus being one of the more frequent sites. Studies have proposed histological criteria to assess the aggressive behaviour of such PEComas and stratify them according to clinical outcome. We reviewed the current literature and discussed the features of this tumour, emphasising specific histological features which suggest malignancy.