Concurrent Angiomyolipoma and Renal Cell Neoplasia: A Study of 36 Cases

Abstract

Little is known about the association of angiomyolipoma and adult renal-cell neoplasia. We studied the clinicopathologic features of 36 patients with concurrent angiomyolipoma and renal-cell neoplasia from the consultation and surgical pathology files of nine institutions. HMB-45 immunoreactivity was analyzed in both neoplasms. Twenty-five sporadic cases of patients with angiomyolipoma and renal-cell neoplasia and 11 cases of patients with tuberous sclerosis, as defined by Gomez' criteria, had mean ages of 59 and 53 years, respectively, and female-male ratios of 2:1 and 5:1, respectively. The mean size of the angiomyolipomas was 1 cm in the sporadic cases and 3 cm in those patients with tuberous sclerosis (medians: 0.5 and 3 cm, respectively, P ‫؍‬ .002). The mean sizes of the renal-cell neoplasms were 5 cm in sporadic cases and 6 cm in patients with tuberous sclerosis (medians: 4 and 5 cm, respectively; P ‫؍‬ .88). In both clinical settings, angiomyolipoma was more commonly the incidental tumor. Clear-cell (conventional) renal-cell carcinoma was the most common renal-cell neoplasm in both groups of patients, accounting for approximately two thirds of the tumors. In patients with tuberous sclerosis, 27% of renal-cell neoplasms were oncocytomas, compared with 8% in sporadic cases (P ‫؍‬ .15). Papillary neoplasia, chromophobe, and collecting-duct renal-cell carcinoma were found only in sporadic cases. All of the 22 renal-cell neoplasms studied were negative for HMB-45, whereas all 25 angiomyolipomas studied were positive.

Cathepsin K expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney

“…16 This differential is of interest because both oncocytomas and angiomyolipomas have been reported in the same kidney and because in patients with tuberous sclerosis there is some evidence that oncocytomas occur more frequently than in general population. 34 In our experience, the detection of HMB45 can be extremely focal in oncocytoma-like angiomyolipomas and, therefore, the strong and diffuse reaction for cathepsin K demonstrated in the present study can be diagnostically helpful.…”

Pseudosarcomatous fibroblastic/myofibroblastic proliferation in perinephric adipose tissue adjacent to renal cell carcinoma: a lesion mimicking well-differentiated liposarcoma

“…Synchronous occurrences of renal neoplasms and angiomyolipoma have been reported. [16][17][18] However, no characteristic thickwalled vessels or perivascular arrangement of the atypical stromal cells was found. Furthermore, the atypical cells were negative for HMB-45 and Melan A, essentially excluding this possibility.…”

Mutational Analysis of the von Hippel Lindau Gene in Clear Cell Renal Carcinomas from Tuberous Sclerosis Complex Patients

“…Renal cell carcinomas are known to occur in patients with angiomyolipomas who do not have TSC. In a recent study of 36 renal cell carcinomas associated with angiomyolipomas, 25 of which were from patients without TSC, clear cell was the most common type (10). These sporadic angiomyolipoma-associated renal carcinomas are of particular interest for future mutational analyses of the TSC1 and TSC2 genes.…”

“…This heterogeneity contrasts with renal cell carcinoma in von Hippel Lindau (VHL) disease or hereditary papillary renal carcinoma (HPRC), which are associated with clear cell and papillary tumors, respectively. Clear cell renal carcinoma appears to be the most common histologic subtype in TSC patients (10).…”