Review of succinate dehydrogenase-deficient renal cell carcinoma with focus on clinical and pathobiological aspects

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11140%2F16%3A10323895" target="_blank" >RIV/00216208:11140/16:10323895 - isvavai.cz</a>

Result on the web

<a href="http://www.termedia.pl/Review-of-succinate-dehydrogenase-deficient-renal-cell-carcinoma-with-focus-on-clinical-and-pathobiological-aspects,55,27341,1,1.html" target="_blank" >http://www.termedia.pl/Review-of-succinate-dehydrogenase-deficient-renal-cell-carcinoma-with-focus-on-clinical-and-pathobiological-aspects,55,27341,1,1.html</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.5114/PJP.2016.59227" target="_blank" >10.5114/PJP.2016.59227</a>

Result language

angličtina

Original language name

Review of succinate dehydrogenase-deficient renal cell carcinoma with focus on clinical and pathobiological aspects

Original language description

Succinate dehydrogenase (SDH)-deficient renal cell carcinoma (RCC) was first identified in 2004 and has been integrated into the 2016 WHO classification of RCC. Succinate dehydrogenase (SDH) is an enzyme complex composed of four protein subunits (SDHA, SDHB, SDHC and SDHD). The tumor which presents this enzyme mutation accounts for 0.05 to 0.2% of all renal carcinomas. Multiple tumors may occur in approximately 30% of affected patients. SDHB-deficient RCC is the most frequent, and the tumor histologically consists of cuboidal cells with eosinophilic cytoplasm, vacuolization, flocculent intracytoplasmic inclusion and indistinct cell borders. Ultrastructurally, the tumor contains abundant mitochondria. Immunohistochemically, tumor cells are positive for SDHA, but negative for SDHB in SDHB-, SDHC- and SDHD-deficient RCCs. However, SDHA-deficient RCC shows negativity for both SDHA and SDHB. In molecular genetic analyses, a germline mutation in the SDHB, SDHC or SDHD gene (in keeping with most patients having germline mutations in an SDH gene) has been identified in patients with or without a family history of renal tumors, paraganglioma/pheochromocytoma or gastrointestinal stromal tumor. While most tumors are low grade, some tumors may behave in an aggressive fashion, particularly if they are high nuclear grade, and have coagulative necrosis or sarcomatoid differentiation.

Czech name

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Czech description

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Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

FP - Other medical fields

OECD FORD branch

—

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2016

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Polish Journal of Pathology

ISSN

1233-9687

e-ISSN

—

Volume of the periodical

67

Issue of the periodical within the volume

1

Country of publishing house

PL - POLAND

Number of pages

5

Pages from-to

3-7

UT code for WoS article

000375804800002

EID of the result in the Scopus database

2-s2.0-84966687527