scholarly journals A bittersweet symphony

2014 ◽  
Vol 21 (4) ◽  
pp. C7-C9
Author(s):  
Adrian F Daly ◽  
Albert Beckers
Keyword(s):  
Succinate Dehydrogenase ◽  
Gastrointestinal Stromal Tumors ◽  
New Work ◽  
Stromal Tumors ◽  
Carney Triad ◽  
Cancer Syndromes

This issue analyzes new work expanding the range of how genetic dysregulation of succinate dehydrogenase subunit (SDHx) genes can cause cancer syndromes with a prominent endocrine component, in this case Carney triad, which is characterized by gastrointestinal stromal tumors, paraganglioma, and pulmonary chondromas.

scholarly journals SDHB immunohistochemistry: a useful tool in the diagnosis of Carney–Stratakis and Carney triad gastrointestinal stromal tumors

2010 ◽  
Vol 24 (1) ◽  
pp. 147-151 ◽  
Author(s):  
José Gaal ◽  
Constantine A Stratakis ◽  
J Aidan Carney ◽  
Evan R Ball ◽  
Esther Korpershoek ◽  
...  
Keyword(s):  
Gastrointestinal Stromal Tumors ◽  
Stromal Tumors ◽  
Carney Triad

Expression of IGF-1 receptor in KIT/PDGF receptor-α wild-type gastrointestinal stromal tumors with succinate dehydrogenase complex dysfunction

2013 ◽  
Vol 9 (1) ◽  
pp. 121-126 ◽  
Author(s):  
Margherita Nannini ◽  
Annalisa Astolfi ◽  
Paola Paterini ◽  
Milena Urbini ◽  
Donatella Santini ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Gastrointestinal Stromal Tumors ◽  
Pdgf Receptor ◽  
Wild Type ◽  
Stromal Tumors ◽  
Succinate Dehydrogenase Complex ◽  
Dehydrogenase Complex

scholarly journals Succinate Dehydrogenase-Deficient Gastrointestinal Stromal Tumors: Small Steps Toward Personalized Medicine?

2019 ◽  
Vol 12 ◽  
pp. 251686571984253 ◽  
Author(s):  
Gloria Ravegnini ◽  
Riccardo Ricci
Keyword(s):  
Succinate Dehydrogenase ◽  
Gastrointestinal Stromal Tumors ◽  
Dna Methyltransferase ◽  
Kinase Inhibitors ◽  
Alkylating Agents ◽  
Mgmt Methylation ◽  
Wild Type ◽  
Stromal Tumors ◽  
Methylguanine Dna Methyltransferase ◽  
Specific Alternative

Various molecular triggers define heterogeneous subsets of gastrointestinal stromal tumors (GISTs), differing in clinical behavior and drug sensitivity. KIT/PDGFRA-wild-type GISTs, including those succinate dehydrogenase (SDH)-deficient, are overall unresponsive to the tyrosine kinase inhibitors commonly used, fostering the development of specific alternative therapeutic strategies. Epigenetic inactivation of O6-methylguanine-DNA methyltransferase (MGMT) through promoter methylation leads to effectiveness of alkylating agents in several human cancers. SDH-deficient GISTs typically feature widespread DNA methylation. However, the actual occurrence of MGMT methylation in these tumors, potentially predisposing them to respond to alkylating drugs, has not been investigated so far. Here we discuss the recent findings concerning the occurrence of MGMT methylation in different GIST subgroups, including SDH-deficient ones, as a premise for a possible reappraisal of alkylating agents specifically targeting these small, otherwise overall chemorefractory, GIST subgroups.

scholarly journals Current management of succinate dehydrogenase–deficient gastrointestinal stromal tumors

2019 ◽  
Vol 38 (3) ◽  
pp. 525-535 ◽  
Author(s):  
Pushpa Neppala ◽  
Sudeep Banerjee ◽  
Paul T. Fanta ◽  
Mayra Yerba ◽  
Kevin A. Porras ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Gastrointestinal Stromal Tumors ◽  
Current Management ◽  
Stromal Tumors

scholarly journals Plexiform architecture in gastrointestinal stromal tumors is not restricted to succinate dehydrogenase-deficient cases

2018 ◽  
Vol 13 ◽  
pp. 69-72
Author(s):  
Maria Cristina Giustiniani ◽  
Valerio Papa ◽  
Maurizio Martini ◽  
Federica Castri ◽  
Tonia Cenci ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Gastrointestinal Stromal Tumors ◽  
Stromal Tumors

Identification of SDHA (subunit A of the succinate dehydrogenase) mutations in KIT/PDGFRA WT gastrointestinal stromal tumors (GISTs).

2011 ◽  
Vol 29 (15_suppl) ◽  
pp. 10045-10045 ◽  
Author(s):  
M. A. Pantaleo ◽  
A. Astolfi ◽  
V. Indio ◽  
P. Paterini ◽  
S. Formica ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Gastrointestinal Stromal Tumors ◽  
Subunit A ◽  
Stromal Tumors

scholarly journals Immunohistochemical Loss of Succinate Dehydrogenase Subunit A (SDHA) in Gastrointestinal Stromal Tumors (GISTs) Signals SDHA Germline Mutation

2013 ◽  
Vol 37 (2) ◽  
pp. 234-240 ◽  
Author(s):  
Markku Miettinen ◽  
Jonathan Keith Killian ◽  
Zeng-Feng Wang ◽  
Jerzy Lasota ◽  
Christopher Lau ◽  
...  
Keyword(s):  
Succinate Dehydrogenase ◽  
Germline Mutation ◽  
Gastrointestinal Stromal Tumors ◽  
Subunit A ◽  
Stromal Tumors
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