Chromophobe renal cell carcinoma
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.
Overview
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.
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.
Anatomy and lymph node drainage
Renal cell carcinoma comes from the kidney's filtering cortex, urothelial cancer from the lining of the collecting system and bladder, and the adrenal on top hosts cortical and medullary (neuroblastoma) tumours.
- Renal cortex (RCC)Classic chromophobe · Eosinophilic chromophobe · Chromophobe with sarcomatoid change (aggressive)
- Renal pelvis and ureter (upper tract urothelial)
- Bladder lining (non-muscle-invasive)
- Bladder muscle wall (muscle-invasive)
- Adrenal cortex
- Adrenal medulla and sympathetic chain (neuroblastoma)
- Developing kidney (Wilms tumour)
- renal hilar
- para-aortic and paracaval
- obturator and iliac (bladder)
Same organ: Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer
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.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Partial nephrectomy or ablation; active surveillance for small tumours; no adjuvant therapy.
Sunitinib, cabozantinib, everolimus or lenvatinib plus everolimus; immunotherapy has low response rates outside sarcomatoid disease; trials preferred.
Kidney surveillance with MRI, nephron-sparing surgery at 3 cm, and genetic counselling.
Subtypes & biomarkers
top- Classic chromophobe
- Eosinophilic chromophobe
- Birt-Hogg-Dube-associated (germline FLCN, hybrid oncocytic tumours)
- Chromophobe with sarcomatoid change (aggressive)
- Multiple whole-chromosome losses
- TP53 and PTEN mutations (poor outlook)
- CK7 and KIT positive, distinguishing it from oncocytoma
- Germline FLCN testing when syndromic features are present
How often this target appears
- 1985Thoenes describes chromophobe renal cell carcinoma
- 2002FLCN identified as the Birt-Hogg-Dube gene
- 2014TCGA maps chromosome losses and TP53 and PTEN mutations
What is in development for Chromophobe renal cell carcinoma, drawn from the whole corpus: 0 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Nothing recorded in development for this cancer yet.
Open problems and what is being done
No randomised trial has ever been run in chromophobe cancer.
and how the field plans to fix it →What is being done about thisRare cancers and small trialsAvailable now- AI trial matching & clinical decision supportEstablished
- Comprehensive genomic profilingStandard of care
- Dinutuximab (ch14.18) / dinutuximab betaApproved
- Eflornithine (DFMO)Approved
- LarotrectinibApproved
- Limb-salvage surgery and endoprosthetic reconstructionStandard of care
In trials- ACTIONRecruiting
- COG AALL1731Positive
- COG ANBL0032Positive
- DeFiPositive
- Euro Ewing 2012Positive
- Functional (ex vivo) drug testingEmerging
Ideas and roadmaps- A digital second-opinion network answering community oncologists within 72 hours
- A DRUP-style protocol for off-label generic targeted drugs in rare tumours
- A funded expert second opinion for every new high-stakes or rare cancer diagnosis
- A global first-in-human network for academic cancer trials with single ethics review
- A global open trials operating system any hospital can plug into
- A live 'seats available' feed for trial slots, like airline inventory
Background: Basket, umbrella, and platform trials, Centralisation and high-volume centres, FNCLCC grade (soft-tissue sarcoma), Histotype-tailored therapy, INRG staging and risk groups. Also on OnCo: Find a trial · Expert centres.
Immunotherapy rarely works and the reason is not understood.
Sarcomatoid change has no effective treatment.
Trials
topTrials recruiting now
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Expert centres
topExpert centres
- via Renal cell carcinoma
- via Renal cell carcinoma
- via Renal cell carcinoma
- via Renal cell carcinoma
- via Renal cell carcinoma
- ANZUP Cancer Trials GroupSydney, AUvia Renal cell carcinoma
- Cedars-Sinai CancerLos Angeles, CA, USvia Renal cell carcinoma
- via Renal cell carcinoma
- via Renal cell carcinoma
- Zhongshan Hospital, Fudan UniversityShanghai, CNvia Thermal ablation (RFA, microwave, cryo)
Questions to ask
topQuestions to ask your oncologist about Chromophobe renal cell carcinoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Multiple whole-chromosome losses, TP53 and PTEN mutations, CK7 and KIT positive, distinguishing it from oncocytoma, Germline FLCN testing when syndromic features are present), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Classic chromophobe, Eosinophilic chromophobe, Birt-Hogg-Dube-associated.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Localised
- For my situation (localised), which of the standard options do you recommend and why?Why: Guideline options include: Partial nephrectomy or ablation; active surveillance for small tumours; no adjuvant therapy.
Metastatic
- For my situation (metastatic), which of the standard options do you recommend and why?Why: Guideline options include: Sunitinib, cabozantinib, everolimus or lenvatinib plus everolimus; immunotherapy has low response rates outside sarcomatoid disease; trials preferred.
- Am I a candidate for Sunitinib, Cabozantinib, Everolimus or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Birt-Hogg-Dube syndrome
- For my situation (birt-hogg-dube syndrome), which of the standard options do you recommend and why?Why: Guideline options include: Kidney surveillance with MRI, nephron-sparing surgery at 3 cm, and genetic counselling.
Any stage
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “No randomised trial has ever been run in chromophobe cancer”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Immunotherapy rarely works and the reason is not understood”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
4targets
7drugs
4companies
6Latest papers
topQuery for this cancer: (TITLE:"Chromophobe renal cell carcinoma" OR ABSTRACT:"Chromophobe renal cell carcinoma" OR TITLE:"chRCC" OR ABSTRACT:"chRCC") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Chromophobe renal cell carcinoma, not a curated reading list.
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