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Succinate dehydrogenase-deficient renal cell carcinoma is a very rare kidney cancer of younger adults who carry a faulty SDH gene, the same fault that causes hereditary paraganglioma and some stomach stromal tumours. Most are low grade and cured by surgery, but a minority spread years later, so patients and relatives need gene testing and follow-up.
The 2016 WHO classification recognised succinate dehydrogenase-deficient renal cell carcinoma as a type, kept in 2022 (Moch 2016; Moch 2022). It arises in people with germline mutations of SDH subunit genes, who are prone to paraganglioma, gastrointestinal stromal tumour and, rarely, renal cell carcinoma. In the defining series of 11 tumours from 10 patients (median age 40; two with paragangliomas, one with bilateral tumours), tumours were 2 to 20 cm, WHO/ISUP grade 2 in ten, stage pT1a to pT2b, and composed of uniform eosinophilic cells with vacuoles or flocculent cytoplasmic inclusions, entrapped renal tubules and mast cells; SDHB immunohistochemistry was negative in all; one patient developed widespread metastases 16 years after nephrectomy and died six years later (Modern Pathology 2015). A low-grade FH-deficient carcinoma can mimic it (Histopathology 2017).
How it differs from its parent: it is a hereditary metabolic cancer of young adults, diagnosed by loss of SDHB staining, indolent in most but with a long tail of late metastasis, and its finding should prompt germline testing and screening for paraganglioma.
How common: no registry share; under 0.2 percent of renal cell carcinomas is the usual estimate in the pathology literature, but the sources read give case series only.
Treatment: surgery, with long-term follow-up for late metastasis; germline SDH testing and paraganglioma surveillance for the patient and relatives; advanced disease treated on the renal cell carcinoma page without a dedicated standard.
Very rare: the defining series holds 11 tumours from 10 patients aged 22 to 72 (median 40) (Modern Pathology 2015). No registry share exists.
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), TFE3-rearranged (translocation) renal cell carcinoma, Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), 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
No subtypes recorded beyond the ones named in the family strip above.
Surgery with long follow-up; germline SDH testing and paraganglioma surveillance for patient and relatives; advanced disease on the renal cell carcinoma page.
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Query for this cancer: (TITLE:"Succinate dehydrogenase-deficient renal cell carcinoma" OR ABSTRACT:"Succinate dehydrogenase-deficient renal cell carcinoma" OR TITLE:"SDH-deficient renal cell carcinoma" OR ABSTRACT:"SDH-deficient renal cell carcinoma" OR TITLE:"SDHB-deficient renal cell carcinoma" OR ABSTRACT:"SDHB-deficient renal cell carcinoma" OR TITLE:"Succinate dehydrogenase-deficient RCC" OR ABSTRACT:"Succinate dehydrogenase-deficient RCC") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Succinate dehydrogenase-deficient renal cell carcinoma, not a curated reading list.
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