I tumori stromali gastrointestinali (GIST). Esperienza personale su tre casi a localizzazione nel tenue mesenteriale trattati in urgenza.

INTRODUCTION: GISTs, a new nosological entity recently described, represent a peculiar model of solid tumor: the identification of the molecular mechanism responsible for the oncogenesis led to the development of a new drug (imatinib) active on the specific molecular target, represented by the product of the mutated proto-oncogene c-kit which is a tyrosine kinase receptor that becomes constitutively active by mutation. Surgical resection, nevertheless, is still the primary treatment and it has to be as complete as possible. These two treatments can be integrated. GISTs are not uniformly kit-positive, and they can be alternatively due to mutations of the PDGFRA gene or, in patients with neurofibromatosis type 1 (NF-1), to generally isolated mutations of the NF-1 gene. PATIENTS AND METHODS: We describe 3 cases of kit-positive GISTs of the small intestine (SISTs), complicated and emergency surgically treated: case 1--53 years, female, with small bowel obstruction and concomitant acute intestinal bleeding; case 2--71 years, male, with NF-1 and acute intestinal bleeding; case 3--47 years, male, with perforation of the Treitz tract. The first two cases have been treated with intestinal resection and immediate mechanical anastomosis; the third one with resection of the pedunculated tumor at its base, where is situated the perforation too. CONCLUSIONS: SISTs (20-30%), with little or no symptoms in the initial phases, show notable diagnostic difficulties. Their aspecific and late clinical presentation--typical of this site and of the pathology that we are talking about--and the difficult physical-instrumental approach to small bowel limit the possibility of an accurate diagnosis and expose the patient to potentially fatal acute complications and to risks related to emergency surgery treatment that decreases the possibility of a radical resection.