Paediatric Gastrointestinal Stromal Tumours Arising From the Stomach: A Clinicopathologic Review of 85 Cases

Aim of the study: Gastrointestinal stromal tumors (GISTs) may now be considered to encompass mesenchymal tumors that express Kit protein (CD117) or have activating mutations of receptor tyrosine kinase genes (PDGFRA). The purpose of this study is to define clinicopathologic characteristics of gastric GISTs in children. Materials and Methods: We conducted a review of the literature from 1962 and enrolled 85 patients (younger than 16 years of age) with gastric GISTs, including two cases treated in our pediatric surgery unit. All the patients’ clinical data and followup information were reviewed, including age, gender, tumour size and localization, presence of Kit mutation and outcome. Case 1: A 16-year-old girl with a history of weakness and pallor. A computer tomography scan confirmed 4 gastric lesions. The lesion in the fundus was excised and an inferior-middle gastric resection was perfomed. At 3 years follow-up the patient presents a progression of liver metastasis. Case 2: A 13-year-old boy was referred to our pediatric surgery unit because of an ulcerated neoformation of 3-cm-sized located in the gastric antrum. The patient underwent laparotomy gastric wedge resection. At the follow-up of 9 years the patient was disease free. Results: Our results are based on our systematic review of 85 cases. The multifocal disease was identified in 21 children (25%). 10 patients are died, all patients presented liver metastases but only 1 had multiple nodules. Conclusions: The majority of GISTs occur in girls that suggests a developmentally related sex difference. There are no relationship between multiple locations and worst prognosis. The presence of liver metastases is associated with a shorter survival. Paediatric Gastrointestinal Stromal Tumours Arising From the Stomach: A Clinicopathologic Review of 85 Cases - ResearchGate. Available from: http://www.researchgate.net/publication/268449480_Paediatric_Gastrointestinal_Stromal_Tumours_Arising_From_the_Stomach_A_Clinicopathologic_Review_of_85_Cases [accessed Apr 25, 2015]