Adrenal angiosarcoma

Abstract

Adrenal angiosarcoma is an uncommon neoplasm that derives from the vascular endothelium; due to its biological behavior, it should be distinguished from other adrenal tumors. We herein report a case of a 57-year-old woman with diagnosis of an adrenal tumor that was suspected to be malignant. The specimen was histopathologically proved to be an angiosarcoma. The patient was suffering from right upper quadrant pain; after laboratory and radiological workup, a non-functioning right adrenal mass, 14 cm in size, was recognized. A right subcostal incision was made, and adrenalectomy was performed successfully with tumor-free surgical margins. Two months after the operation, a positron emission tomography-computed tomography scan was ordered for follow-up. No tumor tissue or any other metastatic foci remained. The patient had been referred to our medical oncology department and underwent retroperitoneal radiotherapy. However, unfortunately, the patient died due to cardiac insufficiency during the follow-up period.Keywords: Adrenal angiosarcoma, adrenal gland, malignant vascular tumors, adrenal incidentaloma INTRODUCTIONAdrenal angiosarcomas are infrequently encountered neoplasms in daily practice; therefore, they are not well documented in the literature. The etiological factors of this neoplasm are still not clearly understood; however, in a case report, exposure to some carcinogens was believed to be the cause of an adrenal angiosarcoma. There is no evidence supporting the relationship between primary adrenal angiosarcoma and multiple neuroendocrine tumors. Also, administration of radiation therapy in the past or previous abdominal traumas has no correlation with adrenal angiosarcoma (1). In most cases, adrenal angiosarcomas are diagnosed coincidentally following routine workup for patient complaints, which are generally non-specific abdominal discomfort or pain (2, 3). In this report, we present a new case of adrenal gland angiosarcoma of unusual size, which will enrich the collected scientific data on this very rare clinical entity. CASE PRESENTATIONA 57-year-old female patient with right upper abdominal pain was admitted to our hospital. Her past medical history included hypertension and hypercholesterolemia, which were under control. On physical examination, slight tenderness was found in the right upper quadrant, without any rebound or Murphy's sign. Cholecystitis was one of our differential diagnoses; therefore, the patient underwent abdominal ultrasound (US). Multiple gallstones were detected; however, significantly, a 14-cm abdominal lesion was also recognized beneath the liver. Therefore, computed tomography (CT) scans of the abdomen (Figure 1) and chest were ordered. A heterogeneous mass 14×12 cm in diameter, originating from the right adrenal gland, was revealed without contrast dying. There were no additional metastatic disseminations. Blood and urinary analyses documented no hormonal activity of the tumor. Preoperative LDH measurement was 422 U/L, VMA was 1.93 mg/day, normetanephrine was 255.5 µg/24 h, m...

Imaging findings of primary epithelioid sarcoma of adrenal gland: a case report and literature review

“…The necrosis area of angiosarcoma is greater, compared to that of ES. Meanwhile, it has specific imaging features on enhanced CT or MRI: demonstrating peripheral enhancement in arterial phase and expansion to the center in venous and delayed phase, which is similar to typical findings of hemangioma (10,11). A large mass with intra-tumor necrosis and adjacent structure invasion are common imaging findings of leiomyosarcoma, but bleeding and calcification are rare.…”