RESUMO
INTRODUCTION: Gastrointestinal stromal tumors (GISTs) represent <1% of all gastrointestinal (GI) tumors. Extra-gastrointestinal stromal tumors (EGISTs) are mesenchymal tissue neoplasm arising outside the GI tract. This rare group comprises only 5% of all GISTs. This case demonstrates a rare entity in a patient with non-specific symptoms, a large tumor size and unremarkable past personal and family history. PRESENTATION OF CASE: We present a 45-year old man with non-specific symptoms who was diagnosed with a primary EGIST arising in the small bowel mesentery after surgery. The tumor was not compromising the GI tract and it was completely resected. The tumor was sent for pathological examination that confirmed the diagnosis. Histological examination revealed a 15 cm in diameter mass, comprised of spindle cells and high mitotic activity. Treatment with imatinib mesylate was initiated. DISCUSSION: There have been only a few previous reports of EGISTs arising from the small bowel mesentery. It is believed that EGISTs originate from cells with similar pathological characteristics and biological behaviour as the intestinal cells of Cajal. Such tumors are associated with poorer prognosis, lager tumor size and younger presentation than their GI counterparts. The preferred treatment is complete surgical resection. The addition of specific tyrosine kinase inhibitors such as imatinib mesylate is recommended for high risk patients. Even though morphological and immunohistochemical similarities between GISTs and EGISTs are described, their pathogenesis, incidence, genetic background, complications and prognosis are not completely known because they are extremely rare. CONCLUSION: EGISTs are very rare tumors which originate from cells outside the GI tract and are associated with a more aggressive biological behavior than their GI counterparts. These tumors may grow without any clinical implications and should be kept in mind in the differential diagnosis for patients presenting with an abdominal mass. Further studies are needed due to lack of large patient cohort studies and long-term follow-up regarding the prognosis and management of this rare pathology.