Definition
Succinate dehydrogenase (SDH)-deficient renal cell carcinoma is a rare emerging entity according to 2016 WHO, reported in patients with germline mutation of succinate dehydrogenase gene (SDH).
Clinical Features
It occurs in a syndrome characterized by paraganglioma/pheochromocytoma, gastrointestinal stromal tumor, and pituitary adenoma (Gill 2018).
Incidence
It comprises up to 0.2% of all resected renal cell tumors with an overall risk of developing renal cell carcinoma being about 14% by age 70 years.
Age
The range in age of patients with SDH-deficient renal cell carcinoma is broad. Therefore, the diagnosis cannot to be thought only in young patients.
Sex
There is no gender predilection.
Site
There is no site predilection.
Treatment
Since these tumors are usually small, partial nephrectomy is recommended.
Outcome
Although a small subset of SDH-deficient renal cell carcinomas behave aggressively, most of them show an indolent course after the complete resection (Ricketts et...
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Gill, A. J. (2018). Succinate dehydrogenase (SDH)-deficient neoplasia. Histopathology, 72, 106–116.
Gill, A. J., Pachter, N. S., Chou, A., et al. (2011). Renal tumors associated with germline SDHB mutation show distinctive morphology. The American Journal of Surgical Pathology, 35, 1578–1585.
Gill, A. J., Hes, O., Papathomas, T., et al. (2014). Succinate dehydrogenase (SDH)-deficient renal carcinoma: A morphologically distinct entity: A clinicopathologic series of 36 tumors from 27 patients. The American Journal of Surgical Pathology, 38, 1588–1602.
Ricketts, C. J., Shuch, B., Vocke, C. D., et al. (2012). Succinate dehydrogenase kidney cancer: An aggressive example of the Warburg effect in cancer. The Journal of Urology, 188, 2063–2071.
Williamson, S. R., Eble, J. N., Amin, M. B., et al. (2015). Succinate dehydrogenase-deficient renal cell carcinoma: Detailed characterization of 11 tumors defining a unique subtype of renal cell carcinoma. Modern Pathology, 28, 80–94.
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Caliò, A., Segala, D., Martignoni, G. (2020). Succinate Dehydrogenase-Deficient Renal Cell Carcinoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4954
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4954
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Publisher Name: Springer, Cham
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Online ISBN: 978-3-030-41894-6
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