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

Cancers (Basel). 2022 Jun 8;14(12):2831. doi: 10.3390/cancers14122831.

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.

Keywords: GIST; KIT; NF1; PDGFRA; SDH-competent; SDH-deficient; SDHB; TKI; gastrointestinal tumors; tyrosine kinase inhibitor; wild-type; young adult.

Publication types

  • Review

Grants and funding

This work was further supported by Maria Sklodowska-Curie National Research Institute of Oncology subsidy from Ministry of Science and Higher Education.