Pediatric intestinal ganglioneuroma: case report and literature review

Abstract

Objective To explore the diagnosis and treatment of pediatric intestinal ganglioneuroma. Methods Two cases were selected from our center while another 15 cases were collected from the databases of Wanfang, Weipu, CNKI, Sinomed, PubMed, Cochrane Library and Medline prior to May 2017. Results Belonging to neither multiple endocrine neoplasia type 2B (MEN2B) or neurofibromatosis type 1 (NF1), 2 cases from our center were not operated. There was no relief of symptoms during a follow-up period of 1 year and 2.5 years respectively. Among them, there were 9 boys and 8 girls with an age range of 14 days to 17 years. The clinical manifestations included diarrhea (n=6), constipation (n=5), hematochezia (n=3) and intestinal obstruction (n=3). 82.4% cases involved colon and rectum. The diagnosis was polypoidganglioneuromas (PG, n=3) and 1 had MEN2B. One case was diagnosed as ganglioneuromatous polyposis (GP). Among 13 cases of diffuse ganglioneuromatosis (DG), 4 had MEN2B and 1 NF1. Among 5 MEN2B cases with RET mutation, 4 cases underwent thyroidectomy with a pathological diagnosis of medullary thyroid cancer. Ten cases with removed lesions had an excellent prognosis while another 4 patients had no removal of lesions and their symptoms persisted. Conclusions Pediatric intestinal ganglioneuroma has an excellent postoperative prognosis. Patients should be carefully examined to exclude MEN2B or NF1 and close surveillance is essential. Key words: Child; Digestive tract; Ganglioneuroma