Renal cell carcinoma classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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
| 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 |
Hereditary Forms
| 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 |
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.[2]
| 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 |
TNM System
The TNM classification system for staging of clear cell renal carcinoma is commonly used.
| T.N.M | Stage |
| Primary Tumor (T) | T0: No evidence of primary tumor T1: Tumor 2.5 cm or less limited to kidney T2: Tumor more than 2.5 cm limited to kidney T3: Tumor extends into major veins or invades adrenal gland or perinephric tissues, but not beyond Gerota's fascia T3a: Tumor invades adrenal gland or perinephric tissues, but not beyond Gerota's fascia T3b: Tumor grossly extends into renal vein(s) or vena cava T4: Tumor grossly extends invades beyond Gerota's fascia |
| Regional Lymph Nodes (N) | N0: No regional lymph nodes (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 |
References
- ↑ 1.0 1.1 Cohen HT, McGovern FJ (2005). "Renal-cell carcinoma". N Engl J Med. 353 (23): 2477–90. doi:10.1056/NEJMra043172. PMID 16339096.
- ↑ 2.0 2.1 Robson CJ, Churchill BM, Anderson W (1969). "The results of radical nephrectomy for renal cell carcinoma". J Urol. 101 (3): 297–301. PMID 5765875.