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Chromophobe Renal Cell Carcinoma

Name  l  Define  l  Risk  l  Detect  l  Treatment  l  Stage/Grade  l  Followup  l  Mets/Recur  l  Link

Names - Synonyms

ChC, CRCC, ChCRCC, chromophobe renal cell carcinoma, chromophobic renal cell carcinoma, renal cell carcinoma - chromophobe subtype, renal cell carcinoma - chromophobic

Definition

Chromophobe renal cell carcinoma is a malignant distinct subtype of renal cell carcinoma and comprises approximately 5% of neoplasms of the renal tubular epithelium, originating in the distal nephron.  It has been described as having a compact growth pattern of large tumor cells, having a translucent and reticular cytoplasm and distinct cell borders.

This type of renal cancer was described first in 1989 by Dr. Kovacs. Unlike most other RCC, these lesions are hypovascular or vascular on renal arteriography. The tumors of this type have different biologic and cytogenetic characteristics as compared with nonpapillary RCC. The tumor is classified as a true papillary RCC if at least 75% of the neoplasm shows papillary growth pattern, with remaining tumor having a tubulopapillary architecture composed of granular cells.

Risk Factors - Causes

The mean age of patients diagnosed is 59, as reported in one study.  It is diagnosed in man as often as women. The only known risk factor that I found in the literature was a congenital disease known as Birt-Hogg-Dubé Syndrome (BHD).  Patients diagnosed with this cancer should always be tested for this condition.

Detection

Although most patients show no symptoms of this cancer, some patients have presented with flank pain, followed by hematuria and/or a palpable mass. Because of it's rarity, pathologists may not familiar with this neoplasm or the neoplasm my have an atypical morphology.  Always request a second, independent, pathology opinion.  Radiographically, the tumor may show up as a hypervascular lesion with a central scar.

Treatment

See Renal Cell Carcinoma

Stage - Grade

See Renal Cell Carcinoma

This cancer may have a slightly better prognosis in grade 3 and 4 than other renal cell carcinomas, but outcome data are limited.  Tumors are more often localized to the kidney (stage I) at diagnosis. Histologic tumor necrosis was an indicator of a poorer prognosis.

Suggested Followups

See Renal Cell Carcinoma

Metastasis - Recurrence

See Renal Cell Carcinoma

Link

Chromophobe Renal Cell Carcinoma Support Forums