Clinical and Pathologic Features of Primary Angiosarcoma of the Kidney (original) (raw)

Primary renal angiosarcoma: Case report and literature review

Canadian Urological Association Journal, 2013

Angiosarcoma is a rare malignant tumour occurring in less than 2% of soft tissue sarcomas. Angiosarcoma involving the kidney usually represents metastasis from skin or visceral primary lesions, while angiosarcoma primarily occurring in the kidney is a very rare neoplasm. We report a case of angiosarcoma of the right kidney in a 59-year-old male. The computed tomography scan showed a solid tumour with a low increased density after administration of contrast medium. Histological examination of the piece of nephrectomy confirmed the diagnosis.

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

Anticancer Research, 2020

Primary angiosarcoma of the kidney is a rare tumor, hence little is known concerning its diagnostic features and therapeutic management. We conducted this survey to present a complete literature review with emphasis on clinicopathological features, diagnosis and therapy. A thorough search was conducted in MEDLINE/PubMed. All relevant studies concerning primary renal angiosarcomas in adults were thoroughly reviewed. Primary renal angiosarcoma is characterized by an overall poor prognosis, is of unknown etiology and occurs most commonly in males between 60 and 70 years old. Presence of distant metastasis at the time of diagnosis is prevalent. Histopathological examination and immunohistochemical studies are the most important diagnostic tools. Treatment options include surgery, chemotherapy, radiotherapy and immunotherapy. Conclusion: Primary renal angiosarcoma is a rare but aggressive malignancy with low response to available therapeutic regimens and dismal survival rates. Angiosarcoma is an extremely rare and aggressive malignant tumor occurring in fewer than 2% of all soft-tissue sarcomas (1). Primary angiosarcoma arises most commonly from the skin, superficial soft tissue, liver, spleen, bone, and breast (2, 3). An exceedingly infrequent location is the kidney, which accounts for 1% of the total of angiosarcomas diagnosed (4). Primary renal angiosarcoma (PRA) has an overall dismal prognosis, with an estimated average life expectancy of 6 months. It is also referred to as renal hemangiosarcoma and was first described in 1942, with few reported cases since then. Its etiology is still unknown and its predisposing factors remain ill-defined (5, 6). However, case reports show development of PRA in patients with pre-existing renal angiomyolipoma or multicystic kidney disease (7, 8). White males between 60-70 years old are most commonly affected (9). Presenting symptoms in the majority of patients include flank pain, hematuria, anemia and palpable mass (3). Initial diagnostic approach involves abdominal computed tomography (CT) and magnetic resonance imaging which may reveal a solitary renal mass. Further total body scan, which is indicated for TNM staging, may unveil distant metastatic lesions in the lungs, liver, bone, spleen, abdominal lymph nodes, peritoneum, or soft tissues (3). Histopathologically, it is defined by an angiomatous structure composed of atypical proliferation of endothelial cells, with epithelioid, spindle or histiocytoid characteristics, and anastomotic vascular channels (10). Biopsy and cytological characteristics combined with a positive immunohistochemical staining for CD31, CD34, factor VIII, vimentin, and friend leukemia integration 1 (FLI1) are the gold standard methods for the definitive diagnosis of PRA (6, 10).

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

Urologic Oncology-seminars and Original Investigations, 2006

Angiosarcoma is a rare malignant tumor occurring in less than 2% of soft tissue sarcomas. Angiosarcoma involving the kidney usually represents metastasis from skin or visceral primary lesions, while angiosarcoma primarily occurring in the kidney is a very rare neoplasm. In the English literature, from 1942 to the present, only 19 cases of primary kidney angiosarcoma have been reported. A case of primary renal angiosarcoma presenting as a large kidney mass and flank pain occurring after traumatism is reported. The present case represents further evidence of a rare malignancy.

A Case of Primary Renal Angiosarcoma

A 78-year old man was diagnosed with a left bleeding renal cyst from CT scan results. Serial CT scans revealed the left kidney mass to be increasing in size and a new lesion in the liver. Renal cell carcinoma with liver metastasis was diagnosed and a radical nephrectomy performed. The initial pathological diagnosis was a benign chronic hematoma. However, the liver mass increased in size and multiplied, while another mass emerged in the twelfth thoracic vertebra with spinal paralysis and was immediately removed. Pathological findings for that specimen showed malignancy of stromal cell origin but low atypia. The renal specimen was re-evaluated using whole cross-section analysis and immunohistochemistry, and diagnosed as a primary renal angiosarcoma. Recombinant interleukin-2 therapy was started immediately; however, the patient died of metastatic disease 13 months after the initial operation. Although contrast imaging depicted the primary lesion as a non-specific hematoma with little focal pooling, and low-grade cytological atypia was shown pathologically, the angiosarcoma was extremely aggressive. This work is licensed under a Creative Commons Attribution 3.0 License (by-nc 3.0) ©Copyright K.

Angiosarcoma of the Kidney. A Case Report and Review of Literature

UROLOGY JOURNAL, 2009

Angiosarcoma is a rare high-grade malignant tumor accounting for less than 2% of the soft tissue sarcomas. They originate from the endothelium of the blood and lymphatic vessels and can be primary or metastatic, and localized or multicentric. Most angiosarcomas develop in the skin or soft tissue, and others, in the breast, liver, and bones. Commonly, metastases occur in the liver, lungs, and bones. Those involving the kidney usually represent metastasis from the skin or the primary visceral lesions. On the contrary, primary angiosarcoma of the kidney is a very rare neoplasm with less than 30 cases reported in the literature to date. To our knowledge, all previous reports described the origin of angiosarcoma in a normal kidney. We report a unique case of primary renal angiosarcoma arising from a multicystic kidney.

Primary renal angiosarcoma: A case report

Human Pathology, 1981

Figure 2. A, Section of skin with c)tomegalic inclusion in derntal capillary (arrow). B, Section of skin with cytomegalic inclusion in sweat gland epithelial cell (arrow). T h e nncleus is out of the plane of section, but cytoplasmic inclusions are clearly visible. (Hematoxylin and eosin stain. • 4O0.

Spontaneous Rupture of a Renal Angiosarcoma

Urologia Internationalis, 2002

Here we describe the clinical, radiologic, histopathological and immunohistochemical features of a rare spontaneous rupture of renal angiosarcoma detected in the left kidney of a 55-year-old male. Due to the rarity of this neoplasm (less than 15 cases have been reported), there is no unanimous concensus for therapy following radical nephrectomy. In our case there was no flank trauma and retroperitoneal haematoma around the left kidney was seen on computed tomography. The patient underwent left radical nephrectomy and died about 3 months after operation due to multiple bone and liver metastases. To our knowledge, this is the first report of spontaneous rupture of renal angiosarcoma and we suggest that it should be borne in mind that renal angiosarcoma may be a cause of retroperitoneal haematomas.