Case Report
Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Jun 21, 2008; 14(23): 3759-3762
Published online Jun 21, 2008. doi: 10.3748/wjg.14.3759
Abdominal neurenteric cyst
Radoje Colović, Marjan Micev, Miodrag Jovanović, Slavko Matić, Nikica Grubor, Henry Dushan E Atkinson
Radoje Colović, Marjan Micev, Miodrag Jovanović, Slavko Matić, Nikica Grubor, Clinical Center of Serbia, Institute for Digestive Diseases, Belgrade 11000, Serbia
Henry Dushan E Atkinson, Surgical Directorate, Imperial College School of Medicine, St Mary’s Hospital, Praed St, London W2 1NY, United Kingdom
Author contributions: Colović R took part at the operation and wrote the article; Micev M did the histology and immunohistochemistry investigation; Jovanović M operated the patient; Matić S did the literature analysis, made an original translation to English, did all the corrections of the manuscript and the correspondence; Grubor N did the literature analysis; Atkinson HDE obtained some of the cited references and corrected the final English article.
Correspondence to: Slavko Matić, MD, PhD, Surgeon, Assistant professor of Surgery, Clinical Center of Serbia, Institute for Digestive Diseases, K. Todorovića 6 Street, Belgrade 11000, Serbia.
Telephone: +381-64-2181949
Fax: +381-11-3031830
Received: December 24, 2007
Revised: April 30, 2008
Accepted: May 7, 2008
Published online: June 21, 2008

Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystogastrostomy for a loculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomography (CT) scan revealed a 16 cm × 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive single-layered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuro ganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.

Keywords: Neurenteric cyst, Congenital, Abdomen, Pancreas, Surgical excision