Papillary, chromophobe and the rarer renal cell carcinomas
- Lesion
Beyond clear cell carcinoma, other renal carcinomas have their own genes, looks and prognosis.
What it is
Renal cell carcinomas are classified by morphology plus genetics. Papillary carcinoma, the second commonest, has trisomies 7 and 17 and activation of MET; hereditary forms come from germline MET (type 1 features) and fumarate hydratase variants (HLRCC, aggressive). Chromophobe carcinoma arises from intercalated cells, has losses of many whole chromosomes and does well. Rare types include MiT family translocation carcinoma (TFE3 or TFEB fusions, children and young adults), collecting duct carcinoma (medulla, aggressive) and renal medullary carcinoma (sickle cell trait).
Structure
- Papillary: hemorrhagic and cystic tumours; fibrovascular cores lined by cuboidal cells with foamy histiocytes.
- Chromophobe: tan solid tumour; plant-cell-like borders, raisinoid nuclei, Hale colloidal iron positive.
- Collecting duct: infiltrating tubules in a desmoplastic stroma deep in the medulla.
Renal cell carcinomas beyond the clear cell type
| Type | Genetics | Features |
|---|---|---|
| Papillary | Trisomy 7, 17; MET activation | Papillae, foamy macrophages; second commonest |
| Chromophobe | Loss of multiple whole chromosomes | Distinct borders, perinuclear halos; good prognosis |
| FH-deficient | Fumarate hydratase loss (HLRCC) | Aggressive; leiomyomas of skin and uterus |
| MiT translocation | TFE3 or TFEB fusions | Children and young adults; clear and papillary cells |
| Collecting duct | Varied | Medullary, desmoplastic, poor prognosis |
| Renal medullary carcinoma | SMARCB1 (INI1) loss | Young people with sickle cell trait; very aggressive |
Clinical notes
- A young person of African descent with sickle cell trait and hematuria needs evaluation for renal medullary carcinoma.
- Hereditary renal cancer syndromes call for genetic counselling of relatives.
Test yourself
Think of the answer, then tap the question to see it.
Which chromosomal gains characterize papillary renal cell carcinoma?
Trisomies of chromosomes 7 and 17 (with MET activation in many).
Which renal carcinoma has the best prognosis among the common types?
Chromophobe renal cell carcinoma.
Which renal tumour is associated with sickle cell trait?
Renal medullary carcinoma.
Elsewhere in DurgiPedia
Connections
Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.
Sources: Hereditary leiomyomatosis and renal cell cancer: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2025); Renal Cell Cancer Treatment (PDQ®)–Patient Version (National Cancer Institute, 2025).
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Representative model, for educational purposes only.