Reference work entry
Diffuse ganglioneuromatosis; Ganglioneuromatosis; Ganglioneuromatous polyposis; Isolated (solitary) polypoid ganglioneuroma; Mucosal ganglioneuroma
Ganglioneuroma (GN) of gastrointestinal tract is an extremely rare neuroectodermal tumor that is characterized by the presence of mucosal ganglion cells and spindled Schwann cells often with numerous eosinophils. Gastrointestinal GNs are divided into three groups defined as polypoid GN, ganglioneuromatous polyposis (GP), and diffuse ganglioneuromatosis (DG). The most common form is polypoid GNs form which show a mixture of ganglion cells and nerve fibrils within mucosa, extending to submucosa. In GP, most polyps resemble typical polypoid GN, whereas others show filiform mucosal projections containing groups of ganglion cells with little neural stroma. GP affects patients with familial adenomatous polyposis (FAP), Cowden’s disease, tuberous sclerosis, multiple endocrine neoplasia type 2b (MEN type 2b) syndrome, colorectal...
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