Korean J Urol.  2010 Oct;51(10):729-732.

Two Different Renal Cell Carcinomas and Multiple Angiomyolipomas in a Patient with Tuberous Sclerosis

• Kang SG
• Ko YH
• Kang SH
• Kim J
• Kim CH
• Park HS
• Moon DG
• Lee JG
• Kim JJ
• Cheon J

Abstract

We report a case of tuberous sclerosis associated with two histologically different renal cell carcinomas (RCCs) and multiple angiomyolipomas (AMLs) in the same kidney. A 43-year-old female was admitted to our hospital with left flank pain and a huge palpable mass in the left flank area. Abdominal computed tomography revealed two concurrent RCCs and multiple AMLs in the left kidney. Because of the clinical suspicion of RCC, the patient underwent left radical nephrectomy. On gross examination, the total size of the resected left kidney was 30.5x17x8 cm. Microscopically, the upper pole tumor features were consistent with chromophobe RCC and the midpole tumor was a clear-cell RCC. The multifocal masses in the remaining remnant parenchyma were AMLs. Six months after surgery, the patient is healthy without signs of tumor recurrence.

Keyword

Angiomyolipoma; Clear cell renal carcinoma; Renal cell carcinoma; Tuberous sclerosis

MeSH Terms

Adult
Angiomyolipoma
Carcinoma, Renal Cell
Female
Flank Pain
Humans
Kidney
Nephrectomy
Recurrence
Tuberous Sclerosis

Figure

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  FIG. 1 Coronal reconstruction of a contrast-enhanced CT scan showing two mass lesions in the upper pole and the midportion of the left kidney with distinct radiologic appearances. The upper mass (black arrow), pathologically diagnosed as chromophobe renal cell carcinoma, demonstrates a relatively homogeneous enhancement pattern. The lower mass (curved arrow), pathologically diagnosed as clear-cell renal carcinoma, demonstrates a predominantly heterogeneous enhancement pattern. The CT image also shows relatively small and multiple fat-containing tumors (white arrows) suggesting angiomyolipomas in the kidneys.

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  FIG. 2 On gross examination, three different renal masses were found. The cut surface of the largest mass (B) in the midportion had a heterogeneous appearance, composed of hemorrhagic, necrotic, and focal golden yellow soft areas. The cut surface of the smaller mass (C) showed focal hemorrhagic areas in the diffuse yellowish-grayish soft area. In the remaining renal parenchyma, there were multifocal poorly demarcated yellowish, glistening mass-like lesions (A).

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  FIG. 3 (A) Renal cell carcinoma (RCC), clear-cell type, composed of nests of cells with clear cytoplasm, surrounded by abundant thin-walled blood vessels. (B) RCC, chromophobe type, composed of nests of cells with abundant cytoplasm, sharply outlined cell membranes, raisinoid nuclei, and perinuclear halos.

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  FIG. 4 Renal cell carcinoma (RCC), chromophobe type, showing intense immunoreactivity for cytokeratin 7.

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  FIG. 5 Renal cell carcinoma (RCC), chromophobe type, showing complete absence of reactivity for vimentin within the tumor cells, except for sinusoidal blood vessels.

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