Primary renal angiosarcoma: A case report and review of the literature

Abstract

Primary renal angiosarcoma is very rare. To our knowledge, only 15 cases have been reported to date. A 77-year-old Japanese man with a unilateral kidney presented with massive hematuria followed by renal failure. A renal tumor was suspected and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma of the kidney. A hemorrhagic tumor measuring 10 x 5 cm and clotted blood was found in the medullary area. The atypical tumor cells had a sinusoidal and solid appearance, and showed immunohistochemically positive reactions for some of the endothelial markers. The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver and retroperitoneum. One of the differential diagnoses of the case was angiomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area. Fifteen case reports with titles that included the term 'hemangiosarcoma/angiosarcoma', 'hemangioendothelioma/endothelioma' or 'vascular sarcoma' of the kidney were reviewed and compared to the present case.

Renal primary angiosarcoma

“…For the most part, chemotherapy regimes have been used that are similar to the ones used in other types of sarcoma, such as cisplatin with iphosphamide or doxo/ epirubicine and iphosphamide with response rates of 44% and 71%, respectively [16], however, this cannot be extrapolated given the paucity of cases. Partial responses have been described, as has one total response lasting for several months with these treatment regimes in angiosarcomas located elsewhere [17].…”

Primary Renal Neoplasia of Dogs

“…A similar phenomenon has been observed in 81% of humans presenting with angiosarcoma. 24 Arising from mesenchymal structures, thus avoiding intraluminal urinary obstruction or bleeding, these tumors may cause fewer clinical signs than carcinomas or nephroblastomas before growing large enough to cause pain.…”

Primary renal angiosarcoma

“…Angiosarcomas are localized or multicentric tumors, with various grades of differentiation and are originated in the endothelium of the blood and lymphatic vessels (1,2). They are high degree malignant neoplasms of primary occurrence, extremely rare in the kidneys, with 24 cases described (3).…”

“…They are high degree malignant neoplasms of primary occurrence, extremely rare in the kidneys, with 24 cases described (3). They are frequently hemorrhagic tumors, being able to simulate a retroperitoneal hematoma or cause massive hematuria (1)(2)(3).…”