# Chromophobe renal cell carcinoma

Source: https://onco.cc/cancers/chromophobe-rcc/  
OnCo record `chromophobe-rcc` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Chromophobe kidney cancer comes from a different cell of the kidney's tubules, usually behaves gently and is cured by surgery. Its rare metastatic form responds poorly to immunotherapy, so kinase and mTOR inhibitors are used, and it runs in families with Birt-Hogg-Dube syndrome.

## Summary

Chromophobe renal cell carcinoma arises from the intercalated cells of the collecting duct and is marked by loss of whole chromosomes (1, 2, 6, 10, 13, 17, 21) with TP53 and PTEN mutations in a minority; it must be told apart from the benign oncocytoma, which it resembles. It is a feature of Birt-Hogg-Dube syndrome, caused by germline FLCN mutations, along with skin fibrofolliculomas and lung cysts. Most tumours are found early and cured by partial nephrectomy, and surveillance is reasonable for small lesions. Metastatic disease is uncommon, responds poorly to PD-1 blockade and is treated with sunitinib or cabozantinib, everolimus, or lenvatinib plus everolimus, drawing on the mTOR pathway activity seen in the disease; sarcomatoid transformation carries the worst prognosis of any kidney cancer.

## Fields

- Kind: Cancer
- Last checked: 2026-09-17
- Also known as: chRCC
- Tags: subtype-page
- Group: genitourinary
- Burden: About five percent of kidney cancers, with the best outlook of the common types: fewer than one in ten spread, and those that do are often slow, but sarcomatoid change turns it deadly.
- Subtypes: Classic chromophobe; Eosinophilic chromophobe; Birt-Hogg-Dube-associated (germline FLCN, hybrid oncocytic tumours); Chromophobe with sarcomatoid change (aggressive)
- Biomarkers: Multiple whole-chromosome losses; TP53 and PTEN mutations (shorter survival); CK7 and KIT positive, distinguishing it from oncocytoma; Germline FLCN testing when syndromic features are present

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/chromophobe-rcc/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/chromophobe-rcc/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/chromophobe-rcc/#what-it-is [4 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/chromophobe-rcc/#finding-it [4 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/chromophobe-rcc/#treating-it [3 settings, 2 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/chromophobe-rcc/#evidence [3 key papers, 3 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/chromophobe-rcc/#science [10 targets]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/chromophobe-rcc/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/chromophobe-rcc/#living-with-it [13 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/chromophobe-rcc/coming/ [4 medicines, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/chromophobe-rcc/data/ [29 connected records]

## Standard of care

- Localised: Partial nephrectomy or ablation; active surveillance for small tumours; no adjuvant therapy. ([Thermal ablation (RFA, microwave, cryo)](https://onco.cc/technologies/thermal-ablation/), [Active surveillance](https://onco.cc/technologies/active-surveillance/))
- Metastatic: Sunitinib, cabozantinib, everolimus or lenvatinib plus everolimus; immunotherapy has low response rates outside sarcomatoid disease; trials preferred. ([Sunitinib](https://onco.cc/drugs/sunitinib/), [Cabozantinib](https://onco.cc/drugs/cabozantinib/), [Everolimus](https://onco.cc/drugs/everolimus/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/))
- Birt-Hogg-Dube syndrome: Kidney surveillance with MRI, nephron-sparing surgery at 3 cm, and genetic counselling. ([Renal cell carcinoma](https://onco.cc/cancers/rcc/))

## State of the art

- Recognition of mTOR pathway dependence gave chromophobe cancer a rational medical option after immunotherapy disappointed.
- Molecular tools now reliably separate chromophobe carcinoma from oncocytoma, sparing some patients surgery.
- Registries of rare kidney cancers are producing the first subtype-specific outcome data.

## Open problems

- No randomised trial has ever been run in chromophobe cancer.
- Immunotherapy rarely works and the reason is not understood.
- Sarcomatoid change has no effective treatment.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Chromophobe_renal_cell_carcinoma
- Wikipedia: https://en.wikipedia.org/wiki/Chromophobe_renal_cell_carcinoma

## Connected records

- cancers: [Clear cell renal cell carcinoma](https://onco.cc/cancers/clear-cell-rcc/), [Eosinophilic solid and cystic renal cell carcinoma](https://onco.cc/cancers/eosinophilic-solid-cystic-renal-cell-carcinoma/), [Papillary renal cell carcinoma](https://onco.cc/cancers/papillary-rcc/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/), [Succinate dehydrogenase-deficient renal cell carcinoma](https://onco.cc/cancers/sdh-deficient-renal-cell-carcinoma/)
- key papers: [ASPEN: everolimus versus sunitinib in metastatic non-clear cell renal cell carcinoma](https://onco.cc/key-papers/paper-aspen-armstrong-lancet-oncol-2016/), [The somatic genomic landscape of chromophobe renal cell carcinoma (The Cancer Genome Atlas)](https://onco.cc/key-papers/paper-tcga-chromophobe-davis-cancer-cell-2014/), [WHO classification of tumours of the urinary system and male genital organs, 2022: renal, penile and testicular tumours](https://onco.cc/key-papers/paper-who-2022-gu-moch-eur-urol-2022/)
- drugs: [Cabozantinib](https://onco.cc/drugs/cabozantinib/), [Everolimus](https://onco.cc/drugs/everolimus/), [Lenvatinib](https://onco.cc/drugs/lenvatinib/), [Sunitinib](https://onco.cc/drugs/sunitinib/)
- technologies: [Active surveillance](https://onco.cc/technologies/active-surveillance/), [Thermal ablation (RFA, microwave, cryo)](https://onco.cc/technologies/thermal-ablation/)
- terms: [PTEN loss](https://onco.cc/terms/pten-loss/)
- targets: [CDKN1A](https://onco.cc/targets/cdkn1a/), [GRM3](https://onco.cc/targets/grm3/), [VHL](https://onco.cc/targets/vhl/)

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JSON: https://onco.cc/api/v1/entities/chromophobe-rcc.json