Clinicopathological features and outcomes of primary genitourinary and adrenal angiosarcomas

Primary angiosarcomas of the genitourinary tract and adrenal gland are rare, aggressive tumors for which clinicopathological and outcome data remain limited. This study characterized their clinicopathological features, treatment patterns, and outcomes at a single center. We retrospectively reviewed 12 patients with histologically confirmed primary genitourinary or adrenal angiosarcoma treated at Shanxi Provincial People’s Hospital between January 2014 and December 2024. Clinical, imaging, pathological, treatment, and follow-up data were analyzed descriptively. The series included seven men and five women aged 30–72 years. Primary sites were the kidney ( n = 4), adrenal gland ( n = 4), bladder ( n = 2), vagina ( n = 1), and ovary ( n = 1). Lumbar or flank pain and hematuria were the most common manifestations. CD31 was positive in 12/12 cases, CD34 and FLI-1 in 9/12 each, ERG in 5/12, and D2-40 in 1/11. All 12 patients underwent R0 resection; 10 developed recurrence or metastasis during follow-up. At the last follow-up, two patients were alive with no evidence of disease, two were alive with disease, and eight had died. Primary genitourinary and adrenal angiosarcomas have nonspecific clinical and imaging features and an aggressive clinical course. Diagnosis requires integrated histopathological and immunohistochemical evaluation. Frequent recurrence or metastasis despite R0 resection highlights the need for close follow-up. Given the small, anatomically heterogeneous series, treatment observations remain descriptive and do not permit efficacy comparisons. Larger multicenter studies are needed to clarify treatment efficacy and prognostic factors.