Clinical and molecular characteristics of pediatric gastrointestinal stromal tumors (GISTs)

AbstractGastrointestinal stromal tumors (GISTs) are rare in occurrence, but comprise the most common mesenchymal tumors of the gastrointestinal tract and affect between 15 and 20 individuals per million per year. Due to recent advancements in molecular classification of these tumors, medical therapy has provided improved outcomes to a historically surgically managed disease. This review article briefly discusses the molecular characteristics, medical and surgical therapies, and future of GIST management.

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Clinical and molecular characteristics of pediatric gastrointestinal stromal tumors (GISTs)

Journal of Clinical Oncology ◽

10.1200/jco.2004.22.14_suppl.8537 ◽

2004 ◽

Vol 22 (14_suppl) ◽

pp. 8537-8537

Author(s):

V. Price ◽

M. Zielenska ◽

C. Smith ◽

S. Chilton-Macneill ◽

D. Malkin ◽

...

Keyword(s):

Gastrointestinal Stromal Tumors ◽

Molecular Characteristics ◽

Stromal Tumors

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Paragangliomas in Carney–Stratakis Syndrome

Hormone and Metabolic Research ◽

10.1055/a-0918-8340 ◽

2019 ◽

Vol 51 (07) ◽

pp. 437-442 ◽

Cited By ~ 2

Author(s):

Arushi Khurana ◽

Lin Mei ◽

Anthony C. Faber ◽

Steven C. Smith ◽

Sosipatros A. Boikos

Keyword(s):

Gastrointestinal Stromal Tumors ◽

Relative Frequency ◽

Autosomal Dominant ◽

Molecular Characteristics ◽

Incomplete Penetrance ◽

Dominant Inheritance ◽

Therapeutic Implications ◽

Stromal Tumors ◽

Carney Triad ◽

Inherited Susceptibility

AbstractCarney-Stratakis Syndrome (CSS) comprises of paragangliomas (PGLs) and gastrointestinal stromal tumors (GISTs). Several of its features overlap with Carney Triad (CT) - PGLs, GISTs, and pulmonary chondromas. CSS has autosomal dominant inheritance, incomplete penetrance, and greater relative frequency of PGL over GISTs. The PGLs in CSS are multicentric and GISTs are multifocal in all the patients, suggesting an inherited susceptibility and associating the two manifestations. In this review, we highlight the clinical, pathological, and molecular characteristics of CSS, along with its diagnostic and therapeutic implications.

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Mutational Testing in Gastrointestinal Stromal Tumor

Current Cancer Drug Targets ◽

10.2174/1568009619666190326123945 ◽

2019 ◽

Vol 19 (9) ◽

pp. 688-697 ◽

Cited By ~ 3

Author(s):

Yu Wang ◽

Jerry Call

Keyword(s):

Gastrointestinal Stromal Tumors ◽

Molecular Characteristics ◽

Optimal Management ◽

Cancer Drugs ◽

Testing Methods ◽

Mesenchymal Tumors ◽

Stromal Tumors ◽

Cancer Management ◽

Specific Cancer ◽

Cancer Network

Targeted treatment has become a major modality in cancer management. Such cancer drugs are generally designed to treat tumors with certain genetic/genomic makeups. Mutational testing prior to prescribing targeted therapy is crucial in identifying who can receive clinical benefit from specific cancer drugs. Over the last two decades, gastrointestinal stromal tumors (GISTs) have evolved from histogenetically obscure to being identified as distinct gastrointestinal mesenchymal tumors with well-defined clinical and molecular characteristics, for which multiple lines of targeted therapies are available. Although the National Comprehensive Cancer Network (NCCN) strongly recommends mutational testing for optimal management of GIST, many GIST patients still have neither a mutation test performed or any mutation-guided cancer management. Here, we review the mutation-guided landscape of GIST, mutational testing methods, and the recent development of new therapies targeting GIST with specific mutations.

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