Renal cell carcinoma classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

Sporadic vs. Hereditary

The following tables classify renal cell carcinoma according to histologic appearance and differentiates sporadic from hereditary forms of renal cell carcinoma.

Sporadic Forms

Sporadic Forms of Renal Cell Carcinoma

Histologic Appearance	Incidence	Gene	Frequency
Conventional	75	VHL	60 %
Papillary	12	MET TFE3	13 % < 1 %
Chromophobe	4
Oncocytoma	4
Collecting Duct	< 1
Unclassified	3 - 5

^{Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90}

Hereditary Forms

Hereditary Forms of Renal Cell Carcinoma

Syndrome	Associated Gene	Common Histological Appearance
VHL Disease	VHL	Conventional
FCRC	Chromosome 3p translocation	Conventional
Hereditary Paraganglioma	SDHB	Conventional
HPRC	MET	Papillary
HLRCC	FH	Papillary
Birt-Hogg-Dube Syndrome	BHD	Chromophobe / Oncocytoma

^{Adapted from Cohen HT, McGovern FJ. Renal-cell carcinoma.N Engl J Med. 2005; 353:2477-90}

Staging

Clear cell renal carcinomas can be classified according to staging or anatomic spread of the disease. Two commonly implemented systems are the Modified Robson's System (1969) and the TNM (Tumor - Nodes - Metastasis) System:

Robson's System

The Modified Robson's staging system has been proposed in 1969. It emphasizes the anatomic distribution of the tumor and proximity to important vasculature and lymph nodes with prognostic implications, reporting a 52% 5-year survival and a 66% 5-year survival in patients with localized tumor.

Robson's Classification System of Renal Cell Carcinoma Staging

Stage	Definition
Stage I	Tumor confined within parenchyma
Stage II	Tumor involves perinephric fat, but still confined within Gerota's fascia
Stage III	A) Tumor involves main renal vein or vena cava B) Tumor involves regional lymph nodes C) Tumor involves both local vessels and lymph nodes
Stage IV	A) Tumor involves adjacent organs other than adrenal gland B) Distant metastasis

^{Adapted from Robson CJ, Churchill BM, Anderson W. The results of radical nephrectomy for renal cell carcinoma. J Urol. 1969; 101(3):297-301.}

References

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