# Succinate dehydrogenase-deficient renal cell carcinoma

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

## TL;DR

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.

## Summary

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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: SDH-deficient renal cell carcinoma; SDHB-deficient renal cell carcinoma; Succinate dehydrogenase-deficient RCC
- Tags: subtype-page; wave4; kidney; rare
- Group: genitourinary
- Burden: Very rare: the defining series holds 11 tumours from 10 patients aged 22 to 72 (median 40) (Modern Pathology 2015). No registry share exists.
- Biomarkers: Loss of SDHB immunohistochemistry; Germline SDHB (or SDHC, SDHA, SDHD) mutation; Flocculent cytoplasmic inclusions and entrapped tubules; WHO/ISUP grade (usually 2)

## 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/sdh-deficient-renal-cell-carcinoma/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/sdh-deficient-renal-cell-carcinoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/sdh-deficient-renal-cell-carcinoma/#what-it-is
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/sdh-deficient-renal-cell-carcinoma/#finding-it [4 biomarkers]
- Treating it (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/sdh-deficient-renal-cell-carcinoma/#treating-it [1 setting]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/sdh-deficient-renal-cell-carcinoma/#evidence
- The science (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/sdh-deficient-renal-cell-carcinoma/#science [1 target]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/sdh-deficient-renal-cell-carcinoma/where-you-are/
- Living with it (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/sdh-deficient-renal-cell-carcinoma/#living-with-it [6 questions]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/sdh-deficient-renal-cell-carcinoma/coming/
- 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/sdh-deficient-renal-cell-carcinoma/data/ [4 connected records]

## Standard of care

- All stages: Surgery with long follow-up; germline SDH testing and paraganglioma surveillance for patient and relatives; advanced disease on the renal cell carcinoma page. ([Renal cell carcinoma](https://onco.cc/cancers/rcc/), [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/), [Nephrectomy](https://onco.cc/terms/nephrectomy/), [Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)](https://onco.cc/cancers/hereditary-ppgl/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Renal_cell_carcinoma
- Moch 2016, European Urology: the 2016 WHO classification of urinary and male genital tumours, part A: https://doi.org/10.1016/j.eururo.2016.02.029
- Moch 2022, European Urology: the 2022 WHO classification of urinary and male genital tumours, part A (renal, penile, testicular): https://doi.org/10.1016/j.eururo.2022.06.016
- Modern Pathology 2015: succinate dehydrogenase-deficient renal cell carcinoma, 11 tumours: https://doi.org/10.1038/modpathol.2014.86
- Histopathology 2017: a low-grade oncocytic FH-deficient RCC resembling SDH-deficient RCC: https://doi.org/10.1111/his.13183

## Connected records

- cancers: [Chromophobe renal cell carcinoma](https://onco.cc/cancers/chromophobe-rcc/), [Eosinophilic solid and cystic renal cell carcinoma](https://onco.cc/cancers/eosinophilic-solid-cystic-renal-cell-carcinoma/), [Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated)](https://onco.cc/cancers/fh-deficient-renal-cell-carcinoma/), [Gastrointestinal stromal tumour (GIST)](https://onco.cc/cancers/gist/), [Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127)](https://onco.cc/cancers/hereditary-ppgl/), [Renal cell carcinoma](https://onco.cc/cancers/rcc/)
- technologies: [Germline (hereditary) testing](https://onco.cc/technologies/germline-testing/)
- targets: [SDHB](https://onco.cc/targets/sdhb/)
- terms: [Hereditary cancer syndromes](https://onco.cc/terms/hereditary-cancer-syndromes/), [Nephrectomy](https://onco.cc/terms/nephrectomy/)

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