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Succinate Dehydrogenase-Deficient Renal Cell Carcinoma

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  • First Online:
Uropathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 5 Accesses

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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References and Further Reading

  • Gill, A. J. (2018). Succinate dehydrogenase (SDH)-deficient neoplasia. Histopathology, 72, 106–116.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  Google Scholar 

  • 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.

    Article  PubMed  PubMed Central  Google Scholar 

  • 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.

    Article  CAS  PubMed  Google Scholar 

  • 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.

    Article  CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy

    Anna Caliò & Guido Martignoni

  2. Department of Pathology, Pederzoli Hospital, Peschiera del Garda, Italy

    Diego Segala & Guido Martignoni

Authors
  1. Anna Caliò
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  2. Diego Segala
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  3. Guido Martignoni
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Corresponding author

Correspondence to Guido Martignoni .

Editor information

Editors and Affiliations

  1. Histopathology and Molecular Diagnostics, University Hospital Careggi, Florence, Italy

    Maria Rosaria Raspollini

  2. Pathology Service, Champalimaud Clinical Center, Lisbon, Portugal

    Antonio Lopez-Beltran

  3. Unit of Anatomic Pathology, Department of Surgery, Cordoba University Medical School, Cordoba, Spain

    Antonio Lopez-Beltran

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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

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-41893-9

  • Online ISBN: 978-3-030-41894-6

  • eBook Packages: MedicineReference Module Medicine

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