Renal cell carcinoma classification

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

Classification

Sporadic vs. Hereditary Renal Cell Carcinoma

The following tables classify renal cell carcinoma according to histologic appearance and differentiates sporadic from hereditary forms of renal cell carcinoma. Conventional/Clear type renal cell carcinoma is considered the most common type of renal carcinoma.

Sporadic Forms of Renal Cell Carcinoma

Sporadic Forms of Renal Cell Carcinoma

Histologic Appearance	Incidence	Gene	Frequency
Conventional/Clear Cell	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 of Renal Cell Carcinoma

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 Clear 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.}

TNM System

The TNM classification system for staging of renal clear cell carcinoma is commonly used. The following TNM classification is based on the American Joint Committee Cancer (AJCC), the American College of Radiology (ACR), and the Union for International Cancer Control (UICC).

TNM Classification System of Renal Clear Cell Carcinoma Staging

T.N.M	Stage
Primary Tumor (T)	TX: Primary tumor cannot be assessed T0: No evidence of primary tumor T1: Tumor 7 cm or less in greatest dimension, limited to the kidney T1a: Tumor 4 cm or less in greatest dimension, limited to the kidney T1b: Tumor more than 4 cm but not more than 7 cm in greatest dimension, limited to the kidney T2: Tumor more than 7 cm in greatest dimension, limited to the kidney T2a: Tumor more than 7 cm but less than or equal to 10 cm in greatest dimension, limited to the kidney T2b: Tumor more than 10 cm, limited to the kidney T3: Tumor extends into major veins or perinephric tissues, but not into the ipsilateral adrenal gland and not beyond Gerota's fascia T3a: Tumor grossly extends into the renal vein or its segmental (muscle containing) branches, or tumor invades perirenal and/or renal sinus fat but not beyond Gerota's fascia T3b: Tumor grossly extends into the vena cava below the diaphragm T3c: Tumor grossly extends into the vena cava above the diaphragm or invades the wall of the vena cavaT4: Tumor invades beyond Gerota's fascia (including contiguous extension into the ipsilateral adrenal gland)
Regional Lymph Nodes (N)	NX: Regional lymph nodes (LN) cannot be assessed N0: No regional LN metastases N1: Metastasis in a single LN 2 cm or less N2: Metastasis in a single LN greater than 2 cm, but less than 5 cm, or multiple LN none greater than 5 cm N3: Metastasis in LN greater than 5 cm
Distant Metastasis (M)	M0: No distant metastasis M1: Distant metastasis

To note, proposals in December 2013 to amend the TNM staging of renal clear cell carcinoma have emerged following a 41.7 month follow-up of 122 patients with pT3a renal clear cell carcinoma. Baccos and colleagues concluded that fat and/or renal vein invasion are important prognostic factors that play a major role in patient survival and should be considered in the TNM staging. Patients with both fat invasion and renal vein thrombosis have worse survival rates than those with only one element.

References

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