Treatment of Gastrointestinal Stromal Tumors (GISTs): A Focus on Younger Patients

Abstract

Gastrointestinal stromal tumors (GISTs) originate from Cajal’s cells and are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs in young adults, i.e., patients before the age of 40, are rare and differ from those in older patients and GISTs in children in terms of the molecular and clinical features, including the location and type of mutations. They often harbor other molecular abnormalities than KIT and PDGFRA mutations (wild-type GISTs). The general principles of therapeutic management in young patients are the same as in the elderly. Considering some differences in molecular abnormalities, molecular testing should be the standard procedure to allow appropriate systemic therapy if needed. The optimal treatment strategy should be established by a multidisciplinary team experienced in sarcoma treatment. The impact of treatment on the quality of life and daily activities, including the impact on work, pregnancy, and fertility, in this patient population should be especially taken into consideration.

Tumor Microenvironment Remodeling in Gastrointestinal Cancer: Role of miRNAs as Biomarkers of Tumor Invasion

“…Gastrointestinal stromal tumors (GISTs) are a subgroup of rare mesenchymal GI tumors that arise most frequently in the stomach ( 60%) and small intestine ( 25%) and less frequently in the rectum ( 5%) and esophagus (<1%) [ 39 , 40 ]. Recently, the incidence has increased with 10–15 new cases per 100,000/year, mainly in older patients and rarely in younger patients [ 41 , 42 ]. GISTs develop from a particular type of cell called interstitial cells of Cajal (ICC).…”

Characterization of a Human Gastrointestinal Stromal Tumor Cell Line Established by SV40LT-Mediated Immortalization

“…Patients presenting with metastatic disease should receive tyrosine kinase inhibitor (TKI) therapy as the initial treatment rather than undergo upfront surgery [10]. Imatinib mesylate, a selective TKI that targets C-KIT and PDGFRα, is used in the treatment of unresectable or metastatic GISTs and can significantly improve the five-year survival rates of patients [11,12]. Unfortunately, approximately 50% of patients develop secondary resistance after two years of treatment, which is called imatinib secondary resistance [13,14].…”

Young-Onset Cancers—Early Steps in the Right Direction

“…The issue also features two review articles by Castle and colleagues [9] on paediatric neuroendocrine neoplasms and Dudzisz-Śledź and colleagues on the treatment of gastrointestinal stromal tumours in younger patients [10].…”