• Xiaonan Yin, Xin Chen, Renjia Shu, Chaoyong Shen, Yuan Yin, Zhaolun Cai, Jian Wang, Zhou Zhao, Huijiao Chen, and Bo Zhang.
• Department of Gastrointestinal Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
• Medicine (Baltimore). 2019 Jul 1; 98 (27): e16305e16305.

RationaleIntestinal ganglioneuromatosis (IGNM) is a rare disease, defined by an abnormal proliferation of ganglion cells, nerve fibers and Schwann cells in the enteric nerve system.Patient ConcernsA 54-year-old woman presented with a one-year history of recurrent episodes of hypogastric pain, with vomiting, nausea, melena, and weight loss of 10 kg in recent 5 months.DiagnosesThe patient was diagnosed as a diffuse IGNM by pathological examination.InterventionsA complete excision of the tumor was performed.OutcomesOn follow-up after 26 months, the patient was asymptomatic without complications.LessonsThis report showed a rare case of diffuse IGNM not associated with NF1 or MEN2b. Preoperative radiological examination suggested an intestinal GIST, yet the final diagnosis of diffuse IGNM was made according to the pathological examination of the resected specimen. Although the prevalence of ganglioneuromatosis is low, this condition should be considered in the differential diagnosis of intestinal mass in adults.

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