A case of ganglioneuroma of the colon during routine colonoscopy

Arzu Buyuk,Donald Kotler,Gabriel Ionescu,Jean Abed,Michael Herman,Pavan Kumar Mankal,Wenjing Shi

doi:10.5348/ijcri-201590-cr-10551

Abstract

We present a case of solitary colonic ganglioneuroma, a rare benign peripheral nerve tumor, found during a routine colonoscopy, in a patient without clinical symptomatology of systemic disease. Case Presentation: A 57-year-old African-American male with a history of untreated chronic hepatitis C cirrhosis with viral load of over 4 million copies, seizure disorder, mild mental retardation, hypothyroidism, hypertension, diabetes mellitus type 2, presented to the gastroenterology clinic for scheduling of a screening colonoscopy. At the time, he was completely asymptomatic. On colonoscopy, one 4mm sessile polyp was resected in the sigmoid colon and was histologically diagnosed as a ganglioneuroma (GN). Colonic ganglioneuromas are classified as hamartomatous polyps that are composed of ganglion cells, nerve fibers, and enteric nervous system cells. GNs of the gastrointestinal tract can be classified into three groups based on the size and the number of polyps: polypoid GN, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. Polypoid GNs, seen in patients with Cowden's syndrome, are small, sessile or pedunculated polyps that have a similar appearance to hyperplastic and adenomatous polyps. Ganglioneuromatous polyposis, seen most commonly in patients with MEN IIB, NF1, Cowden's syndrome, usually manifests as more than 20 sessile or pedunculated polyps. Lastly, diffuse ganglioneuromatosis, seen in MEN IIB7 and NF1, involves proliferation of neuronal cells in the entire colon, but does not extend into the ileum. Conclusion: The finding of an asymptomatic, solitary GN in our patient does not warrant more frequent colon cancer screening given its benign nature.

Highlights

Colonic ganglioneuromas are classified as hamartomatous polyps that are composed of ganglion cells, nerve fibers, and enteric nervous system cells
The GNs of the gastrointestinal tract can be classified into three groups based on the size and the number of polyps: polypoid GN, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis
Both of them are highlighted by immunostain S100 (Figure 5), but negative for AE1/AE3 and EMA or Colonic GNs are classified as hamartomatous polyps that are composed of ganglion cells, nerve fibers, and enteric nervous system cells

Summary

INTRODUCTION

Ganglioneuromas (GNs) are a subset of neuroblastomas They are rare, slow growing, welldifferentiated large tumors that arise from sympathetic ganglion cells. The patient was asymptomatic and did not report any fever, chills, nausea, vomiting, abdominal pain, diarrhea or constipation. He never had any alarming symptoms such as weight loss, melena and hematochezia. In addition to denying toxic habits (i.e., tobacco, alcohol, drugs), he denied any personal or family history of colon or small bowel cancer His physical examination was unremarkable, as he did not have scleral icterus, murmurs, wheezing, abdominal distention, organomegaly, café au lait spots or fibromas on his skin. Both of them are highlighted by immunostain S100 (Figure 5), but negative for AE1/AE3 and EMA (markers for 69 epithelium) or Figure 1: Endoscopy view of ganglioneuroma

DISCUSSION

CONCLUSION