SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib

Succinate dehydrogenase deficient gastrointestinal stromal tumors (dSDH GIST) is a unique group of GISTs with an energy metabolism defect as the key oncogenic mechanism without mutations in the proto-oncogene receptor tyrosine kinase (KIT) and platelet-derived growth factor receptor a (PDGFRA). SDH-...

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Auteurs principaux:	Daria A. Filonenko, Andrey A. Meshcheryakov, Petr P. Arkhiri, Maxim P. Nikulin, Evgeniia S. Kolobanova
Format:	article
Langue:	RU
Publié:	IP Habib O.N. 2020
Sujets:	gastrointestinal stromal tumors succinate dehydrogenase carney triad imatinib Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Accès en ligne:	https://doaj.org/article/553e488d924d466a9a73765baf3532ec
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spelling	oai:doaj.org-article:553e488d924d466a9a73765baf3532ec2021-11-30T17:03:34ZSDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib1815-14341815-144210.26442/18151434.2020.2.200053https://doaj.org/article/553e488d924d466a9a73765baf3532ec2020-07-01T00:00:00Zhttps://modernonco.orscience.ru/1815-1434/article/viewFile/35196/23619https://doaj.org/toc/1815-1434https://doaj.org/toc/1815-1442Succinate dehydrogenase deficient gastrointestinal stromal tumors (dSDH GIST) is a unique group of GISTs with an energy metabolism defect as the key oncogenic mechanism without mutations in the proto-oncogene receptor tyrosine kinase (KIT) and platelet-derived growth factor receptor a (PDGFRA). SDH-deficiency is a result of mutations in SDHA, SDHB, SDHC, SDHD. There are three variants of dSDH GIST: sporadic dSDH GIST, Carney triad or Carney-Stratakis syndrome. dSDH GISTs are characterized by young age, female prevalence, gastric location, multiple tumors, lymph node metastases, indolent behavior and poorly response to imatinib. Despite the literature data, we report the response to imatinib in patient with dSDH GIST. 21 year old female patient presented with incomplete Carney triad (multiply gastric GIST with liver and peritoneal metastases, left lung chondroma). The patient received imatinib with clinical response in a month and radiological response in three months-cystic transformation of primary gastric tumor and liver metastases. The duration of response was 8 months.Daria A. FilonenkoAndrey A. MeshcheryakovPetr P. ArkhiriMaxim P. NikulinEvgeniia S. KolobanovaIP Habib O.N.articlegastrointestinal stromal tumorssuccinate dehydrogenasecarney triadimatinibNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282RUСовременная онкология, Vol 22, Iss 2, Pp 133-136 (2020)
institution	DOAJ
collection	DOAJ
language	RU
topic	gastrointestinal stromal tumors succinate dehydrogenase carney triad imatinib Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	gastrointestinal stromal tumors succinate dehydrogenase carney triad imatinib Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Daria A. Filonenko Andrey A. Meshcheryakov Petr P. Arkhiri Maxim P. Nikulin Evgeniia S. Kolobanova SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
description	Succinate dehydrogenase deficient gastrointestinal stromal tumors (dSDH GIST) is a unique group of GISTs with an energy metabolism defect as the key oncogenic mechanism without mutations in the proto-oncogene receptor tyrosine kinase (KIT) and platelet-derived growth factor receptor a (PDGFRA). SDH-deficiency is a result of mutations in SDHA, SDHB, SDHC, SDHD. There are three variants of dSDH GIST: sporadic dSDH GIST, Carney triad or Carney-Stratakis syndrome. dSDH GISTs are characterized by young age, female prevalence, gastric location, multiple tumors, lymph node metastases, indolent behavior and poorly response to imatinib. Despite the literature data, we report the response to imatinib in patient with dSDH GIST. 21 year old female patient presented with incomplete Carney triad (multiply gastric GIST with liver and peritoneal metastases, left lung chondroma). The patient received imatinib with clinical response in a month and radiological response in three months-cystic transformation of primary gastric tumor and liver metastases. The duration of response was 8 months.
format	article
author	Daria A. Filonenko Andrey A. Meshcheryakov Petr P. Arkhiri Maxim P. Nikulin Evgeniia S. Kolobanova
author_facet	Daria A. Filonenko Andrey A. Meshcheryakov Petr P. Arkhiri Maxim P. Nikulin Evgeniia S. Kolobanova
author_sort	Daria A. Filonenko
title	SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
title_short	SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
title_full	SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
title_fullStr	SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
title_full_unstemmed	SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
title_sort	sdh-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib
publisher	IP Habib O.N.
publishDate	2020
url	https://doaj.org/article/553e488d924d466a9a73765baf3532ec
work_keys_str_mv	AT dariaafilonenko sdhdeficientgastrointestinalstromaltumorsparadoxicaleffectofimatinib AT andreyameshcheryakov sdhdeficientgastrointestinalstromaltumorsparadoxicaleffectofimatinib AT petrparkhiri sdhdeficientgastrointestinalstromaltumorsparadoxicaleffectofimatinib AT maximpnikulin sdhdeficientgastrointestinalstromaltumorsparadoxicaleffectofimatinib AT evgeniiaskolobanova sdhdeficientgastrointestinalstromaltumorsparadoxicaleffectofimatinib
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SDH-deficient gastrointestinal stromal tumors: paradoxical effect of imatinib

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