Surgical management of adolescent and young adults with gastrointestinal stromal tumors: it is of value?

Gastrointestinal stromal tumors (GIST) are the most common sarcomas of the gastrointestinal tract and are characterized by constitutive activation of the KIT or PDGFRA receptor tyrosine kinase (1,2). According the Surveillance, Epidemiology and End Results (SEER) database, the incidence of GIST was 0.32 per 100,000 persons in the United States. The median age was 62 years and primary tumors are located mainly in the stomach (58.7%) and small bowel (31.2%). Up-front surgery is still standard therapy for located GIST (except for located GIST tumors of <2 cm diameter in which observation is an option), while imatinib (a c-KIT tyrosine-kinase inhibitor), is currently used in unresectable or metastatic GIST. Despite that 50% of patients respond to Imatinib therapy, the vast majority of patients develop secondary resistance, with a median time of 2 years (3-5). In metastatic GIST without imatinib progression (either generalized or local progression), several studies suggest that surgical rescue would improve overall survival (6-13). Unfortunately, also in this favourable subset of patients, the value of surgical rescue is on debate, because the lack of a control group (patients treated with imatinib therapy with response and not treated with surgical rescue) to adequately contextualize the clinical benefit.