Gastrointestinal autonomic nerve tumor: immunohistochemical and molecular identity with gastrointestinal stromal tumor.

摘要: Gastrointestinal autonomic nerve tumor (GANT) is a gastrointestinal neoplasm that ultrastructurally recapitulates the enteric neural plexus. This study identifies and defines features of 10 cases this rare mesenchymal compares its clinicopathologic molecular genetic with data on stromal (GIST). The majority patients in series presented at an older age (mean 64 years). Tumors arose from stomach (6), small intestine (2), retroperitoneum (2). Mean size was 14 cm; however, four neoplasms were <6 cm. Histologically, tumors spindled or epithelioid; one epithelioid demonstrated previously undescribed rhabdoid histologic phenotype. All positive for CD117 (KIT), while eight CD34. In contrast, only two S-100, all negative actin desmin. Five GANTs GIST-specific gain-of-function mutations juxtamembrane domain c-kit gene (50%). Three died disease 22-30 months, patient postoperative period, complications CML. clinicopathologic, histologic, immunohistologic, GANT are similar to GIST, indicating merely represents phenotypic variant GIST.