The DOI matches its Crossref record; 47 of the 47 references it cites were found in a registry or an index.
Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients
Open accessAbstract
Succinate dehydrogenase (SDH)-deficient renal carcinoma has been accepted as a provisional entity in the 2013 International Society of Urological Pathology Vancouver Classification. To further define its morphologic and clinical features, we studied a multi-institutional cohort of 36 SDH-deficient renal carcinomas from 27 patients, including 21 previously unreported cases. We estimate that 0.05% to 0.2% of all renal carcinomas are SDH deficient. Mean patient age at presentation was 37 years (range, 14 to 76 y), with a slight male predominance (M:F=1.7:1). Bilateral tumors were observed in 26% of patients. Thirty-four (94%) tumors demonstrated the previously reported morphology at least focally, which included: solid or focally cystic growth, uniform cytology with eosinophilic flocculent cytoplasm, intracytoplasmic vacuolations and inclusions, and round to oval low-grade nuclei. All 17 patients who underwent genetic testing for mutation in the SDH subunits demonstrated germline mutations (16 in SDHB and 1 in SDHC). Nine of 27 (33%) patients developed metastatic disease, 2 of them after prolonged follow-up (5.5 and 30 y). Seven of 10 patients (70%) with high-grade nuclei metastasized as did all 4 patients with coagulative necrosis. Two of 17 (12%) patients with low-grade nuclei metastasized, and both had unbiopsied contralateral tumors, which may have been the origin of the metastatic disease. In conclusion, SDH-deficient renal carcinoma is a rare and unique type of renal carcinoma, exhibiting stereotypical morphologic features in the great majority of cases and showing a strong relationship with SDH germline mutation. Although this tumor may undergo dedifferentiation and metastasize, sometimes after a prolonged delay, metastatic disease is rare in the absence of high-grade nuclear atypia or coagulative necrosis.
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Cites
The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia
Srigley, J. R., Delahunt, B., Eble, J. N., Egevad, L., Epstein, J. I., Grignon, D., Hes, O., Moch, H., Montironi, R., Tickoo, S. K., et al. (2013) · American Journal of Surgical Pathology · doi:10.1097/pas.0b013e318299f2d1 · Cited by 1,047
Self-citationNeumann, H. P. H. (2004) · JAMA · doi:10.1001/jama.292.8.943 · Cited by 893
Janeway, K. A., Kim, S. Y., Lodish, M., Nosé, V., Rustin, P., Gaal, J., Dahia, P. L. M., Liegl, B., Ball, E. R., Raygada, M., et al. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1009199108 · Cited by 628
van Nederveen, F. H., Gaal, J., Favier, J., Korpershoek, E., Oldenburg, R. A., de Bruyn, E. M., Sleddens, H. F., Derkx, P., Rivière, J., Dannenberg, H., et al. (2009) · The Lancet Oncology · doi:10.1016/s1470-2045(09)70164-0 · Cited by 538
Vanharanta, S., Buchta, M., McWhinney, S. R., Virta, S. K., Peçzkowska, M., Morrison, C. D., Lehtonen, R., Januszewicz, A., Järvinen, H., Juhola, M., et al. (2004) · The American Journal of Human Genetics · doi:10.1086/381054 · Cited by 389
Miettinen, M., Wang, Z.-F., Sarlomo-Rikala, M., Osuch, C., Rutkowski, P., & Lasota, J. (2011) · American Journal of Surgical Pathology · doi:10.1097/pas.0b013e3182260752 · Cited by 363
Self-citationGermline SDHB Mutations and Familial Renal Cell Carcinoma
Ricketts, C., Woodward, E. R., Killick, P., Morris, M. R., Astuti, D., Latif, F., & Maher, E. R. (2008) · JNCI Journal of the National Cancer Institute · doi:10.1093/jnci/djn254 · Cited by 342
Korpershoek, E., Favier, J., Gaal, J., Burnichon, N., van Gessel, B., Oudijk, L., Badoual, C., Gadessaud, N., Venisse, A., Bayley, J.-P., et al. (2011) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2011-1043 · Cited by 277
Gill, A. J., Benn, D. E., Chou, A., Clarkson, A., Muljono, A., Meyer-Rochow, G. Y., Richardson, A. L., Sidhu, S. B., Robinson, B. G., & Clifton-Bligh, R. J. (2010) · Human Pathology · doi:10.1016/j.humpath.2009.12.005 · Cited by 277
Self-citationGimm, O., Armanios, M. Y., Dziema, H., Neumann, H. P. H., & Eng, C. (2000) · PubMed · Cited by 274
Cited by
Mitochondrial complex ii: At the crossroads
Bezawork-Geleta, A., Rohlena, J., Dong, L., Pacak, K., & Neuzil, J. (2017) · Trends in Biochemical Sciences · doi:10.1016/j.tibs.2017.01.003 · Cited by 295
Kamilaris, C. D. C., & Stratakis, C. A. (2018) · HORMONES · doi:10.1007/s42000-018-0072-y · Cited by 7
Metadata
- Authors
- Gill, A. J., Hes, O., Papathomas, T., Šedivcová, M., Tan, P. H., Agaimy, A., Andresen, P. A., Kedziora, A., Clarkson, A., Toon, C. W., Sioson, L., Watson, N., Chou, A., Paik, J., Clifton-Bligh, R. J., Robinson, B. G., Benn, D. E., Hills, K., Maclean, F., Niemeijer, N. D., Vlatkovic, L., Hartmann, A., Corssmit, E. P. M., van Leenders, G. J. L. H., Przybycin, C., McKenney, J. K., Magi-Galluzzi, C., Yilmaz, A., Yu, D., Nicoll, K. D., Yong, J. L., Sibony, M., Yakirevich, E., Fleming, S., Chow, C. W., Miettinen, M., Michal, M., & Trpkov, K.
- Venue
- American Journal of Surgical Pathology
- Published
- 2014-12
- Volume
- 38
- Issue
- 12
- Pages
- 1588-1602
- ISSN
- 0147-5185 (ISSN-L)
- OpenAlex
- W1457625536
- Cited by
- 347
- References
- 47
- FWCI
- 19.39
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- CoChat. (2026). Citation check: Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients. https://app.cochat.ai/papers/10.1097/pas.0000000000000292
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- Gill, A. J., Hes, O., Papathomas, T., Šedivcová, M., Tan, P. H., Agaimy, A., Andresen, P. A., Kedziora, A., Clarkson, A., Toon, C. W., Sioson, L., Watson, N., Chou, A., Paik, J., Clifton-Bligh, R. J., Robinson, B. G., Benn, D. E., Hills, K., Maclean, F., . . . Trpkov, K. (2014). Succinate Dehydrogenase (SDH)-deficient Renal Carcinoma: A Morphologically Distinct Entity A Clinicopathologic Series of 36 Tumors From 27 Patients. American Journal of Surgical Pathology. https://doi.org/10.1097/pas.0000000000000292
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
