Papillary renal cell carcinoma (papillary RCC) is a kidney cancer subtype with distinct type 1 and type 2 patterns. The statistics below describe how often it occurs, who is represented in reported cohorts, how tumors present, and how outcomes vary by stage and subtype. Because the figures come from different studies and populations, they should not be treated as one universal rate.
Contents
- How common papillary RCC is
- Type 1 and type 2 differences
- Who was represented in reported cohorts
- Tumor size, stage, and metastasis
- Treatment patterns
- Survival statistics
- Genomic and prognostic findings
How common papillary RCC is
The National Cancer Institute (NCI) Rare Tumors page reports that papillary renal cell carcinoma makes up about 15% of all renal cell carcinoma. In an NCI The Cancer Genome Atlas (TCGA) summary, papillary carcinoma was estimated at approximately 8% of kidney cancer cases analyzed in that specific context. These percentages use different reference populations, so they are not interchangeable.
The 2024 BMC Urology study of an Iceland national cohort identified 1,725 renal cell carcinoma cases. Clear cell RCC accounted for 74.4% of the cohort, chromophobe RCC for 2.1%, and papillary RCC for 9.2%. Within the study period, the proportion of papillary RCC increased from 3.7% in the first study interval to 11.5% in the last interval. The Iceland figures describe one national population and a defined study period, rather than all kidney cancers worldwide.
In that Iceland cohort, age-standardized papillary RCC incidence was 1.97 per 100,000 for males and 0.5 per 100,000 for females. Age-standardized cancer-specific mortality was 0.6 per 100,000 for males and 0.19 per 100,000 for females. The same study reported an annual average increase of 3.6% in age-standardized incidence for type 1 papillary RCC.
Type 1 and type 2 differences
The NCI Rare Tumors page describes type 1 papillary RCC as more common than type 2. It also describes type 1 as slow-growing, while type 2 is more aggressive and grows more quickly. The Iceland cohort provides several measured differences between the groups:
| Measure | Type 1 papillary RCC | Type 2 papillary RCC |
|---|---|---|
| Patients in Iceland cohort | 103 | 40 |
| Mean age | 65.4 years | 64.8 years |
| Male-to-female ratio | 4.4 | 2.3 |
| Average tumor size | 58.8 mm | 73.7 mm |
| Metastasis at diagnosis | 8.7% | 30.0% |
| Estimated 5-year cancer-specific survival | 86.3% | 66.0% |
The type 1 and type 2 groups had similar mean ages in Iceland, but their tumor and outcome measures differed. Average tumor size was 14.9 mm greater for type 2 disease than for type 1 disease. Metastasis at diagnosis was reported in 30.0% of type 2 cases compared with 8.7% of type 1 cases. These are cohort findings and do not predict an individual patient’s outcome.
Who was represented in reported cohorts
The Iceland study included 103 patients with type 1 papillary RCC and 40 with type 2. Right-sided tumors accounted for 43% of type 1 cases and 45% of type 2 cases. Bilateral disease occurred in 2% of type 1 cases and 0% of type 2 cases.
The 2009 Journal of Cancer Research and Clinical Oncology surgical RCC cohort included 744 patients. In its papillary RCC group, 73.8% of patients were male, compared with 60.3% in the clear-cell RCC group. The study reported that papillary RCC presented more often with smaller tumors and low-grade tumors than clear-cell RCC. It also found organ-confined disease more often in papillary RCC, while pT3b/c tumors were more common in clear-cell RCC.
A separate SEER/COSMIC papillary RCC analysis reported a median age at diagnosis of 64 years. Men accounted for 77% of cases, and White patients accounted for 68%. In that analysis, tumor grades I through IV were distributed as 13%, 53%, 31%, and 3%, respectively. Age over 60 years, Black race, poor histologic differentiation, distant metastases, and tumor size over 10 cm were identified as independent mortality risk factors in the analysis.
Tumor size, stage, and metastasis
The Iceland data show different stage distributions for the two papillary RCC types. Stage I disease accounted for 58% of type 1 cases and 40% of type 2 cases. Stage IV disease accounted for 9% of type 1 cases and 33% of type 2 cases. Fuhrman grade 2 accounted for 56% of type 1 cases and 40% of type 2 cases.
Lung metastases were present in 5% of type 1 papillary RCC cases and 13% of type 2 cases in the Iceland cohort. The SEER/COSMIC analysis reported that localized-to-kidney disease accounted for 85% of known-stage cases. Tumors were 7 cm in 84% of known-size cases, and no metastasis was recorded in 97% of cases with known metastatic status. The wording of these measures matters: the denominators were limited to cases with known stage, size, or metastatic status.
The German surgical cohort also showed a strong relationship between extent of disease and survival. Among 555 non-metastatic evaluable patients, 5-year survival for pT1–4 papillary RCC was 89.2%. For pT ≤ 2 disease, 5-year survival was 97.4%, while for pT ≥ 3 disease it was 54.0%. These stage-group figures should be read as results from that non-metastatic evaluable group, not as a general survival rate for every papillary RCC diagnosis.
Treatment patterns
In Iceland, nephrectomy was performed in 69% of type 1 papillary RCC cases and 65% of type 2 cases. Partial nephrectomy was performed in 23% of type 1 cases and 15% of type 2 cases. The Iceland study also reported that 50% of type 1 cases and 35% of type 2 cases were incidental diagnoses.
In the SEER/COSMIC analysis, surgical resection was the most common treatment and was used in 9% of cases as reported by that analysis. Five-year overall survival was 79% across the analysis, and 5-year overall survival after surgery was 90.6%. Because treatment use and survival were reported for that particular analysis, these figures should not be used to estimate the benefit of surgery for a specific person.
Survival statistics
The Iceland national cohort estimated overall papillary RCC cancer-specific survival at 90.9% after 1 year, 83.6% after 3 years, and 80.7% after 5 years. When divided by subtype, estimated 5-year cancer-specific survival was 86.3% for type 1 papillary RCC and 66.0% for type 2.
The 2009 German surgical cohort reported mean estimated tumor-specific survival of 143.8 months for papillary RCC and 147.8 months for clear-cell RCC. Five-year survival was 78% for papillary RCC and 77% for clear-cell RCC. In metastatic disease, median tumor-specific survival was 18.4 months for papillary RCC and 25.3 months for clear-cell RCC. In advanced disease, 5-year survival was 35% for papillary RCC and 57% for clear-cell RCC.
These comparisons use different endpoints and study designs. The Iceland figures are cancer-specific survival from a national cohort, while the German study reports tumor-specific survival in a surgical RCC cohort. The SEER/COSMIC analysis reports overall survival, including 79% at 5 years overall and 90.6% after surgery. A cancer-specific or tumor-specific measure is not the same as overall survival, so the numbers should be compared only within their original context.
Genomic and prognostic findings
The NCI TCGA summary estimated that 81% of type 1 papillary tumors contained a MET alteration. The same NCI source described type 2 papillary carcinoma as heterogeneous, with multiple distinct subtypes, and described type 1 and type 2 papillary RCC as distinct in their genomic profiles.
The CpG island methylation phenotype subtype was found almost exclusively in type 2 papillary carcinoma and was associated with the least favorable outcomes in the NCI summary. Loss of CDKN2A expression in type 2 papillary carcinoma was associated with poor prognosis. The TCGA summary also identified clear cell and papillary carcinoma as the two common renal cell carcinoma subtypes analyzed in that context.
Taken together, the available statistics describe papillary RCC as a heterogeneous disease rather than one single-risk category. Type 1 was more common in the cited NCI description and had lower measured tumor size, stage IV frequency, and metastasis-at-diagnosis frequency than type 2 in the Iceland cohort. Type 2 was linked with more aggressive clinical features in the NCI description and with lower estimated 5-year cancer-specific survival in the Iceland data. Individual prognosis still depends on the tumor’s stage, grade, spread, molecular features, and treatment context.