scholarly journals Multifocal gastric gastrointestinal stromal tumors (GISTs) with lymph node metastases in children and young adults: A comparative clinical and histomorphological study of three cases including a new case of Carney triad

2011 ◽  
Vol 6 (1) ◽  
Author(s):  
Claudia Otto ◽  
Abbas Agaimy ◽  
Alexander Braun ◽  
Jochen Rädecke ◽  
Jens Hoeppner ◽  
...  
2020 ◽  
Vol 22 (2) ◽  
pp. 133-136
Author(s):  
Daria A. Filonenko ◽  
Andrey A. Meshcheryakov ◽  
Petr P. Arkhiri ◽  
Maxim P. Nikulin ◽  
Evgeniia S. Kolobanova

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.


2005 ◽  
Vol 27 (4) ◽  
pp. 179-187 ◽  
Author(s):  
Sonam Prakash ◽  
Lisa Sarran ◽  
Nicholas Socci ◽  
Ronald P DeMatteo ◽  
Jonathan Eisenstat ◽  
...  

Apmis ◽  
2013 ◽  
Vol 121 (10) ◽  
pp. 938-944 ◽  
Author(s):  
Guhyun Kang ◽  
Young Soo Park ◽  
Eun-Sun Jung ◽  
Mee Joo ◽  
Mi Seon Kang ◽  
...  

2005 ◽  
Vol 173 (4S) ◽  
pp. 359-359
Author(s):  
Marta Sanchez-Carbayo ◽  
Lee Richstone ◽  
Nicholas Socci ◽  
Wentian Li ◽  
Nille Behrendt ◽  
...  

2001 ◽  
Vol 45 (6) ◽  
pp. 581
Author(s):  
Ji Seon Joo ◽  
Hyung Jin Kim ◽  
Kyung Jin Kang ◽  
Young Kuk Cho ◽  
Myung Kwan Lim ◽  
...  

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