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Translocation renal cell carcinoma is a rare kidney cancer, more often seen in children and young adults, driven by a rearrangement of the TFE3 gene (or its relative TFEB). It can look like clear cell or papillary kidney cancer under the microscope, so it is found by testing for the gene. There is no standard treatment for advanced disease, which is treated with the usual kidney cancer drugs.
The 2022 WHO classification groups TFE3-rearranged and TFEB-altered (rearranged or amplified) renal cell carcinomas as the molecularly defined MiT family translocation carcinomas (Moch 2022). TFE3-translocation carcinoma is highly heterogeneous clinically and genetically: among 63 untreated primaries studied by whole-exome and RNA sequencing, the ASPSCR1-TFE3 fusion and copy-number changes including loss of 22q went with aggressive features and poor outcome, most tumours (except MED15-TFE3) showed low PD-L1 expression and low T-cell infiltration, and five transcriptional clusters with distinct angiogenesis, stroma, proliferation and KRAS-down signatures tracked with fusion type and prognosis (Nature Communications 2021). A mouse model expressing PRCC-TFE3 in the kidney reproduced the histology, showed elevated RET expression that the RET inhibitor vandetanib suppressed, and identified GPNMB as a diagnostic marker (Molecular Cancer Research 2019).
How it differs from its parent: it is defined by a fusion rather than by VHL loss, occurs at a younger age, is misdiagnosed as clear cell or papillary carcinoma without TFE3 immunohistochemistry or FISH, and lacks the immune-rich microenvironment that makes clear cell carcinoma respond to checkpoint inhibitors.
How common: no registry share was found in the sources read.
Treatment: surgery for localised disease; for advanced disease there is no standard, and the VEGF-directed and checkpoint combinations of the renal cell carcinoma page are used with retrospective evidence only (Nature Communications 2021). A registry trial of agnostic therapy in rare solid tumours lists translocation renal cell carcinoma among its conditions.
Rare, and commonest in children and young adults; the largest molecular series holds 63 untreated primary tumours (Nature Communications 2021). No registry share was found in the sources read.
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.
Same organ: Collecting duct carcinoma of the kidney, Renal medullary carcinoma (SMARCB1-deficient), Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), Succinate dehydrogenase-deficient renal cell carcinoma, Mucinous tubular and spindle cell carcinoma of the kidney, Eosinophilic solid and cystic renal cell carcinoma, Clear cell papillary renal cell tumour, Urothelial carcinoma of the urethra, Squamous cell carcinoma of the urethra, Adenocarcinoma of the urethra (including clear cell adenocarcinoma), Melanoma of the urethra, Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Low-risk neuroblastoma (INRG very low and low risk, including stage MS), Intermediate-risk neuroblastoma, High-risk neuroblastoma, Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer, Localised penile cancer (organ-confined, node-negative), Node-positive and metastatic penile cancer, Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable), Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127), Metastatic pheochromocytoma and paraganglioma
Surgery when localised; advanced disease treated on the renal cell carcinoma page's VEGF and checkpoint pathways without a dedicated standard.
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Query for this cancer: (TITLE:"TFE3-rearranged translocation renal cell carcinoma" OR ABSTRACT:"TFE3-rearranged translocation renal cell carcinoma" OR TITLE:"Translocation renal cell carcinoma" OR ABSTRACT:"Translocation renal cell carcinoma" OR TITLE:"Xp11 translocation renal cell carcinoma" OR ABSTRACT:"Xp11 translocation renal cell carcinoma" OR TITLE:"MiT family translocation renal cell carcinoma" OR ABSTRACT:"MiT family translocation renal cell carcinoma" OR TITLE:"TFE3-translocation RCC" OR ABSTRACT:"TFE3-translocation RCC" OR TITLE:"TFEB-altered renal cell carcinoma" OR ABSTRACT:"TFEB-altered renal cell carcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about TFE3-rearranged (translocation) renal cell carcinoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
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Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
Tablets: take on an empty stomach (no food 2 hours before or 1 hour after). Avoid grapefruit.
Avoid grapefruit.
Known QT prolongation. Avoid other QT-prolonging drugs where possible; check ECG and correct potassium and magnesium before and during treatment.
See all on the product pages:CabozantinibNivolumabSunitinib·Printable cards in the navigator
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