Case of the Week

2020-29 / July 20 (Contributor: Varsha Manucha)

On follow up for a renal mass, a man in his early 60s presents with a fracture of his right arm. Imaging revealed a renal mass (5.5 x 1.7cm) in the lower pole, a destructive lesion involving humeral metaphysis, and small satellite nodules in liver.

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Quiz

1. What is the diagnosis?

a. Papillary renal cell carcinoma

b. Sarcomatoid renal cell carcinoma

c. Angiosarcoma

d. Urothelial carcinoma

e. Collecting duct carcinoma

Angiosarcoma is rare and exhibits remarkable clinical heterogeneity. Most cases develop in the skin or soft tissue, and involvement of the kidney is usually metastatic in nature. Primary renal angiosarcoma (PRA) accounts for 1% of all the cases and has an overall dismal prognosis. Its etiology is still unknown but development in a pre-existing renal angiomyolipoma or multicystic kidney disease has been reported. Presenting symptoms are the same as that of renal cell carcinomas. The tumor can range between 3.0cm to 37cm and forms a hemorrhagic and/or necrotic mass. Histologically, it is composed of irregular, anastomosing vascular spaces and channels lined by discrete and large endothelial cells with variable degrees of cytological pleomorphism, nuclear atypia, mitotic activity, and multilayering. Positivity for ERG, CD31, CD34, & factor VIII support the diagnosis. The mainstay of treatment of PRA is surgical resection. Recent therapeutic approaches such as immunotherapy, molecular targeting therapy and anti-angiogenic agents are promising and require further evaluation.

This tumor was associated with multiple foci of vascular invasion and microscopic tumor nodules in peri-renal fat. Biopsy of a liver nodule and the humerus also showed angiosarcoma.

About GUPS

The Genitourinary Pathology Society (GUPS) was formed with the vision of advancing the care of patients with urologic diseases through enhancements of best practices, research, and education in the subspecialty of Urological Pathology.

GUPS is an official Companion Society of the United States and Canadian Academy of Pathology (USCAP).

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