No abstract

Standards for the management of gastrointestinal stromal tumors (GIST) in children do presently not exist. Thus a systematic review and summary of the current literature was conducted serving as a basis for the further development of optimal management strategies for childhood GIST within a cooperative network. Presently 21 cases with familial GIST, and more than 100 pediatric cases each with Carney triad or sporadic GIST have been published so far. An international prospective registration based on national registries has recently started to acquire more clinical and molecular data and to develop appropriate management strategies for children and adolescents with GIST.

Following the SIOP protocol, pulmonary RT can be omitted for a majority of patients with PM and results in a relatively good outcome.