Primary renal angiosarcoma mimicking urothelial carcinoma - A case report and literature reviews

Abstract

Angiosarcoma (AS) is a rare aggressive tumor originating from endothelial cells. We reported a 66-year-old female with primary renal angiosarcoma (PRA) who presented as urothelial carcinoma with hematuria and dysuria. Based on ureterorenoscopic tumor biopsy, the initial diagnosis suggested low-grade non-invasive urothelial carcinoma. However, the specimen retrieved from nephroureterectomy confirmed the diagnosis of primary renal angiosarcoma. Primary renal angiosarcoma could uncommonly present as urothelial carcinoma in renal pelvis. Surgical resection remains to be the most effective therapy but there is no consensus about adjuvant therapies. The overall prognosis of primary renal angiosarcoma is dismal.

Keywords: Adjuvant chemotherapy; Angiosarcoma; CT, Computed tomography; Nephroureterectomy; PA, Primary angiosarcoma; PET, Positron emission tomography; PRA, Primary renal angiosarcoma; Radiotherapy; Urothelial carcinoma.

Fig. 1

Contrast enhanced computed tomography (CT) of abdomen. Contrast enhanced computed tomography (CT). A CT image reveals a suspicious renal pelvis tumor with peripheral contrast enhancement (orange arrows). (A) Transverse section and (B) coronal section. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

Histological examination of specimen from ureterorenoscopic tumor biopsy. The findings of histological examination indicated papillary urothelial proliferation with mild cytologic abnormality mimic non-invasive low grade papillary urothelial carcinoma.

Fig. 3

The immunohistochemical staining. The immunohistochemical study revealed positive findings of CD31, CD34 and FLI-1 which are sensitive and specific for vascular tumors highlight the endothelial cells of blood vessels. Negative findings for HHV-8, HMB-45, MDM2 and CDK4.