Urol Radiol 11:30-32 (1989)

Urologic Radiology

© Springer-Verlag New York Inc. 1989

Primary Renal Angiosarcoma Mimicking a Renal Cell Carcinoma

M. B. Desai, 1 Q. Chess, 2 J. B. Naidich, 3 and R. Weiner 4 ~Division of Nuclear Medicine, Department of Medicine, 2Department of Pathology, 3Department of Radiology, 4Department of Medicine, North Shore University Hospital, CorneU University Medical College, Manhasset, New York, USA

Abstract. Primary renal angiosarcoma is a very rare malignant tumor that closely mimics the more common renal cell adenocarcinoma (hypernephro- ma) in radiologic appearance. Both tumors are hy- pervascular masses and the diagnosis must be con- firmed histologically. However, in the presence of a tumor with a capsular blood supply, and in the ab- sence of venous invasion, the diagnosis of renal an- giosarcoma may be suggested, especially in a patient with a new onset of consumption coagulopathy.

Key words: Angiosarcoma -- Renal neoplasia.

Maglignant neoplasms of vascular endothelium are uncommon tumors that may be classified as 1 of the following 4 distinct clincopathologic entities: he- mangiopericytoma, lymphangiosarcoma, Kaposi's sarcoma, and angiosarcoma [1]. Angiosarcoma oc- curs in both sexes at any age; the most frequent primary sites are skin, liver, spleen, and bone [2]. Primary renal angiosarcoma is very rare and it may be difficult to differentiate radiographically from renal cell carcinoma. We report a case that was clin- ically and radiographically mistaken for a renal cell carcinoma.

Case Report

A 54-year-old white man presented with flank pain, fatigue, and gingival bleeding. Physical examination showed multiple ecchy-

moses and a left upper quadrant mass. The coagulogram indi- cated disseminated intravascular coagulopathy (DIC). Urine analysis showed numerous red cells.

Abdominal computed tomographic (CT) scan (Fig. 1) showed a 21 x 16 x 14 cm capsular left renal mass with central necrosis displacing the left kidney anteriorly. Abdominal aortography showed a large hypervascular left renal mass. The renal capsular artery appeared stretched. An inferior vena cavogram failed to reveal involvement of the left renal vein or inferior vena cava. The diagnosis of renal cell carcinoma was suggested. Left neph- rectomy revealed a small pale kidney adherent to and surrounded by a cavernous vascular mass weighing 820 g. Microscopic sec- tions showed a diffusely infiltrating high-grade vascular neoplasm consisting of anastomosing channels lined by pleomorphic en- dothelial cells (Fig. 2). Immunoperoxidase stain demonstrated cytoplasmic factor VIII antigen, thus confirming the vascular nature of the tumor. Electron microscopy showed poorly formed

Address reprint requests to." M.B. Desai, M.D., Division of Nu- clear Medicine, North Shore University Hospital, 300 Com- munity Drive, Manhasset, NY 11030, USA

Fig. 1. A C T scan of the abdomen shows a large mass in the left renal fossa. Note the anterior displacement of the kidney (arrow).

M.B. Desai et al.: Renal Angiosarcoma Mimicking Carcinoma 31

Fig. 2. Histopathologic appearance of renal angiosarcoma: anastomosing cleftlike vascular spaces (arrowheads) and invasion of perinephric adipose tissue (x 40).

Weibel-Palade bodies. The diagnosis of primary renal angio- sarcoma was made. The patient briefly responded to chemo- therapy, but was readmitted within 4 months for anemia. Repeat CT scan showed tumor recurrence in the left renal fossa. Bone scintigraphy revealed hypervascular metastases. The patient died shortly thereafter.

Discuss ion

According to the A r m e d Forces Insti tute o f Pa- thology [3], about 2% of all adult neoplasms are o f kidney origin; renal cell carc inomas account for 80 - 85% followed by renal pelvic tumors (8-10%). The rest are miscel laneous sarcomas (2-3%). Le iomy- osarcomas are the mos t frequent renal sarcomas. Very few cases o f renal angiosarcomas have been repor ted [5-9]. Clinically, a lmost every pat ient has had D I C at presentation, and all have suffered a rapidly fatal progression with widespread metas ta- ses. It is difficult to distinguish this t u m o r radio- graphically f rom renal cell carc inoma, since bo th neoplasms can have similar CT and angiographic features [4, 10, 11]. However , features that suggest the possibili ty o f renal capsular sa rcoma include a t u m o r with p redominan t capsular b lood supply, dis- p lacement o f the kidney, and absence o f renal vein or inferior vena cava invo lvemen t [4].

Dissemina t ion o f angiosarcomas is hematoge- nous with hypervascular metastases to liver, lung, and bone [12]. In summary , the diagnosis o f renal angiosarcoma m a y be suggested when a pat ient with a new onset o f D I C has a vascular renal capsular neop lasm with a dominan t capsular b lood supply and absence o f renal vein or inferior vena cava in- vo lvement . However , the diagnosis mus t be con- f irmed histologically. The prognosis, at this t ime o f writing, is un i formly rapidly fatal.

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