Early diagnosis and straight forward surgery of an asymptomatic primary angiosarcoma of the kidney led to long‐term survival

Abstract

We report the first case of a diagnosis of an asymptomatic primary renal angiosarcoma. The patient underwent laparoscopic nephrectomy and is alive after long-term follow up. We provide the preoperative imaging studies and the histologic features of this exceedingly rare tumor.

Primary renal angiosarcoma

“…Clinical and radiological assessment can only suggest a malignant renal mass and diagnosis is possible only after histopathological examination of the nephrectomy specimen and confirmation with positive immunohistochemical staining for CD-31, CD-34 and factor VIII. [3]…”

Primary renal angiosarcoma: a diagnostic and therapeutic challenge

“…In these patients, pathological analysis show malignant endothelial cells forming vascular structures. CD34 and CD31 are endothelial markers used to identify the vascular nature of the disease 2. FLI-1 protein is a member of ETS family of DNA-binding transcription factors.…”

“…It is generally associated with metastasis resulting in poor prognosis 2. This disease was first reported by Prince in 1942 3.…”

Primary renal angiosarcoma with progressive clinical course despite surgical and adjuvant treatment: A case report

“…Metastases are often present at the time of diagnosis or develop due to hematogenous spread within a couple of months or weeks after surgery. The common metastatic sites are the lungs, liver and bones (8,11,12). Overall, an extremely small number of patients survive more than one year from the time of diagnosis.…”

“…Tumors measuring <5 cm in diameter confer an improved prognosis compared with those >5 cm (11,12). The five-year survival rates for tumors >5 and <5 cm are 13 and 32%, respectively (9).…”