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5 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| 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. | not mapped |