Gastrointestinal autonomic nerve tumors: a clinical review

Gastrointestinal autonomic nerve tumors (GANTs) are believed to be rare accounting for 1 % of all malignant gastrointestinal tumors. Many gastrointestinal surgeons and gastroenterologists are unaware of this entity. This review aims to highlight the salient clinical features and prognosis of GANTs.

Using the common search engines and manual cross-referencing, a search of the English literature was conducted for "gastrointestinal autonomic nerve tumor."

All of the published literature on GANTs is either case reports or small case series. From 49 retrieved articles, a total of 107 GANT cases were collected with a mean age of 54 years and equal male to female preponderance. The most commonly affected site was small bowel followed by stomach. Esophageal and colorectal GANTs were less frequent. Clinical presentation was variable ranging from non-specific symptoms, abdominal pain, weight loss, iron-deficiency anemia, to obstruction and gastrointestinal bleeding. Acute presentation due to free rupture or perforation with subsequent peritonitis was extremely rare. Endoscopic and radiological investigations were valuable in tumor localization and determination of distant spread. Thirteen patients were lost to or had no follow-up, leaving 94 patients for long-term outcome analysis. All patients were treated by radical surgical resection of the involved organ as this offered the only hope of cure. Local recurrence, metastases, or both developed in 40 % of cases despite radical surgical resection. Resection for local recurrences and hepatic metastases was feasible in some selected cases. Response to adjuvant chemoradiation was poor and imatinib mesilate was effective in cases of metastatic or inoperable CD117-positive GANTs.

Radical surgical resection of GANTs is the mainstay of treatment. The aggressive behavior after radical resection coined with the poor response to adjuvant chemotherapy call for the urgent need to develop new adjuvant therapies.