Primary leiomyosarcoma of the adrenal; a case report

INTRODUCTION AND IMPORTANCE: Primary adrenal leiomyosarcoma (PAL) is an extremely rare neoplasm that usually arises from the smooth muscle cells of the adrenal or adjacent vascular structures. The tumor is asymptomatic until it grows up and develops a mass effect in the retroperitoneal region. Although there are about 50 reported valid cases, surgical intervention is mandatory in the majority of patients. CASE PRESENTATION: Herein, we report the case of a 32-year-old healthy woman with a chief complaint of vague abdominal pain. After initial clinical and radiological examinations, we found a large retroperitoneal mass located around the right adrenal gland. Due to the patient's pain, a laparotomy was performed, and a large mass was resected with free margins. Immunohistochemical examination was positive for vimentin, smooth muscle actin (SMA), and desmin. Therefore, the diagnosis of PAL was confirmed. CONCLUSION: Although PAL is an uncommon malignancy, its diagnostic and therapeutic approaches are almost straightforward. A computed tomography scan can show the characteristics of the tumor and direct the management. Surgical resection is the mainstay of treatment, and the effects of adjuvant therapies have not been apparent yet.