Clinical and Pathologic Features of Primary Angiosarcoma of the Kidney

Abstract

Approximately 64 cases have been reported in the literature, and most cases occur in the 6th-7th decade with a strong male predominance. The aetiology is unknown. Patients present with flank pain, haematuria, abdominal mass and weight loss. A considerable number of patients develop metastatic disease at diagnosis or shortly afterwards. Grossly, the tumour comprises ill-defined haemorrhagic spongy masses often with necrosis. Microscopically, the tumour is composed of anastomosing capillary-sized vessels which are lined by malignant endothelial cells. The mainstay of treatment is surgery followed by radiation therapy with or without chemotherapy. Renal angiosarcomas are highly aggressive tumours with dismal outcome, and they must be distinguished from morphologically similar lesions of the kidney.

Primary renal angiosarcoma: a diagnostic and therapeutic challenge

“…Primary renal angiosarcoma is an extremely rare subgroup accounting for 1% of all the cases. According to a comprehensive review by Omiyale and colleagues, approximately 64 cases of primary angiosarcoma of the kidney have been reported to date 4. In previously reported cases, these lesions carry a clear gender predominance with a male to female ratio of 3:1 6.…”

Angiosarcoma of kidney with calcification: A rare case report

“…Primary renal angiosarcoma is very rare, with fewer than 70 cases reported in the literature, and has a poor outcome due to rapid local spread and metastasis [1–5]. We share a rare case of renal angiosarcoma with calcification.…”

“…According to the literature analysis, surgery is the most effective treatment for renal angiosarcoma. Radical nephrectomy must be performed along with radiotherapy and/or chemotherapy [1–5].…”

“…The diagnosis is made on a morphologic basis, proven by the immunohistochemical study. Renal angiosarcoma is highly malignant and has poor prognosis [1–5]. According to the literature analysis, surgery is the most effective treatment for renal angiosarcoma.…”

Clinical Features of Disseminated Intravascular Coagulation With Spontaneous Ruptured Primary Renal Angiosarcoma

“…Angiosarcoma is a rare malignant neoplasm consisting of vascular spaces lined by endothelial cells, and it accounts for ＜2% of all soft tissue sarcomas. 1,2 Approximately onethird of angiosarcomas arise in the skin, one-third in the soft tissue, and one-third in other anatomical sites such as the liver, breast, and bone. 3 Angiosarcoma of the kidney is usually found in metastasis from the skin of visceral primary lesions, while primary angiosarcoma arising in the kidney is extremely rare.…”