Uterine Perivascular Epithelioid Cell Tumor (PEComa) in A 56-year-Old Woman

Perivascular epithelioid cell tumors (PEComas) are infrequent mesenchymal neoplasms. Primary uterine PEComas are extremely uncommon. To the best of knowledge, around 110 cases of uterine PEComas have been documented in the English-language literature thus far. Herein, we present the case of primary uterine PEComa in a 56-year-old Saudi woman who presented to clinical attention with a six-month history of left-sided abdominal pain. Gynecological examination showed a 5-cm solid mass involving the left adnexa. Tumor markers were normal. Computed tomography scan demonstrated a 4.2 x 4.4 x 3.4 cm superior left fundal exophytic mass. Patient underwent total abdominal hysterectomy plus bilateral salpingo-oophorectomy. Final histopathological examination demonstrated benign/uncertain malignant potential PEComa. No further adjuvant therapy was administered. At six-month follow up, the patient was asymptomatic without recurrence. In conclusion, uterine PEComas are rare. Histopathological assessment establishes the definitive diagnosis. Surgery remains the gold standard in the treatment of uterine PEComas and adjuvant therapy should be guided based on clinical and histopathological risk factors. Keywords: Uterine perivascular epithelioid cell tumor; PEComa; Uterine sarcoma; hysterectomy.