Adrenal PEComa Treated by Surgical Resection and Postoperative Radiotherapy: A Case Report.

Abstract

BACKGROUND Perivascular epitheloid cell neoplasm (PEComa) is a rare mesenchymal tumor that is evaluated in the same tumor family as angiomyolipoma, sugar cell tumor of the lung, and lymphangioleiomyomatosis. Immunohistochemically, the disease can express melanocytic and myogenic markers, such as HMB45, HMSA1, MelanA/Mart1, and actin. The disease can be seen in almost every organ, especially the uterus and retroperitoneum. Adrenal gland-derived PEComa is extremely rare and leads to difficulties in diagnosis. Here, we present a case of a adrenal gland PEComa in which we applied postoperative radiation therapy. CASE REPORT A 24-year-old female patient visited the doctor in April 2022 due to abdominal pain that had been increasing steadily for the previous few months. Abdominopelvic computed tomography shows a solid mass of 10 cm on the right adrenal gland. Right adrenal gland mass excision surgery was performed in May 2022. The pathology was reported as malign PEComa. The patient was admitted for postoperative radiotherapy because of uncertainty about the surgical boundaries. Systemic treatment was not required. A fraction dose of 4680 cGy/26 was applied by 8-field IMRT to the tumor bed area. In December 2022, the patient's radiotherapy was completed. No acute adverse effects from the radiotherapy were observed. The patient's follow-up after treatment continued, without disease and long-term adverse effects. CONCLUSIONS Surgical resection is the primary treatment approach in the treatment of localized disease. Although the literature is far from making a clear recommendation on adjuvant therapy, pathologic risk factors should be considered when deciding on adjuvant therapy.