Primary leiomyosarcoma of the adrenal; a case report

Abstract

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.

Primary adrenal leiomyosarcoma

“…Primary adrenal leiomyosarcoma is an extremely rare mesenchymal tumor that arises from smooth muscle cells located in the wall of the central adrenal vein or its branches [1] . It was first reported in 1981 [3] .…”

“…It was first reported in 1981 [3] . Since then, less than 50 cases have appeared in the medical literature [1] . Unfortunately, the tumor is usually not detected until an advanced stage because it is not hormonally active and there are no specific tumor markers [4] .…”

“…Surgical resection is the standard of care [ 2 , 6 ]. Chemotherapy and radiation therapy are of uncertain benefit [1] .…”

“…Primary adrenal leiomyosarcoma is an extremely rare mesenchymal tumor that arises from smooth muscle cells that reside in the wall of the central adrenal vein or its branches [1] . A recent review of case reports found less than 50 cases have been reported in the English medical literature [2] .…”

Challenges in the diagnosis of the enigmatic primary adrenal leiomyosarcoma: two case reports and review of the literature

“…[ 20 ] 2023 74 F Pain (abdominal) L 3.4 None Operative pathology SMA, desmin and h-caldesmon Adrenalectomy Alive ND Oshidari B. [ 21 ] 2022 32 F Pain (abdominal) R 10.6 IVC Operative pathology SMA, desmin and vimentin Adrenalectomy Alive 2 Waack A. [ 22 ] 2022 58 F Pain (abdominal) L 5.5 None Operative pathology SMA, desmin, vimentin, and h-caldesmon Adrenalectomy Alive 30 Wang Y.…”