Primary adrenal angiosarcoma: Insights into an exceptionally rare malignancy

Primary adrenal angiosarcoma is a high-grade vascular malignancy. This case describes a 78-year-old female who presented with left upper quadrant abdominal pain, early satiety, nausea, weight loss, and a feeling of being “cold all the time.” Computed tomography of the abdomen and pelvis demonstrated a 15 cm× 13 cm x 16 cm heterogeneous, enhancing mass with interspersed calcifications, originating from the left adrenal gland. Adrenal labs were collected and were not consistent with a functional adrenal lesion, and lymphoma was ruled out. The patient underwent an exploratory laparotomy with resection of the left retroperitoneal mass, left adrenalectomy, splenectomy, and left partial nephrectomy due to the tumor impeding surrounding structures. All surgical margins were negative, with no evidence of metastatic disease. Immunohistochemical staining showed the tumor to be strongly and diffusely positive for cytokeratin, CD31, and ERG, while negative for CD34. Additional staining for FLI1 was positive. Based on these findings, a final pathological diagnosis of primary epithelioid angiosarcoma of the adrenal gland was made. The patient received asplenia vaccines and was discharged on postoperative day three without complications and was seen two weeks postoperatively in clinic. Recommendations were made in agreement with surgical and medical oncology for surveillance imaging, including computed tomography of the chest, abdomen, and pelvis every three months for a minimum of five years.