İleumun Yaygın Ganglionöromatozu
Abstract
A 33-year-old man presented with a two-year history of right lower quadrant pain and episodes of bloody diarrhea. He had previously undergone an appendectomy and an unspecified laparotomy without resolution of symptoms. Suspected Crohn’s disease prompted a third laparotomy at our center, during which 120 cm of terminal ileum and adjacent mesentery were resected. Grossly, the ileal wall was thickened up to 2 cm, the lumen narrowed, and the mucosa exhibited pseudopolypoid projections and superficial ulcers. Histologically, a diffuse proliferation of spindle-shaped cells and mature ganglion-like elements infiltrated from the submucosa through the muscularis into the serosa, consistent with ganglioneuromatosis. A cutaneous neurofibroma excised three months earlier suggests an association with neurofibromatosis. We review the clinical presentation, pathology and management of gastrointestinal ganglioneuromas.
Keywords
ganglioneuroma, ganglioneuromatosis, ileum, gastrointestinal nerve tumor
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