Gastroenterology Report 3 (2015) 86–89, doi:10.1093/gastro/gou052 Advance access publication 6 August 2014 Case report Mucinous cystadenoma of the appendix with enterocutaneous fistula: a therapeutic dilemma Nawal K. Jha, Dipendra K. Sinha, Abhinav Anand, Mrigendra K. Rai, Anjana Gandhi, Jitin Yadav and Sanjay K. Yadav* General Surgery, Rajendra Institute of Medical Sciences, Ranchi, India *Corresponding author: General Surgery, Rajendra Institute of Medical Sciences, 3/50 RIMS Ranchi, Jharkhand 834009, India. Tel: +91-857-9002240; Email: sky1508@gmail.com Submitted 25 May 2014; Revised 8 July 2014; Accepted 24 July 2014 Mucinous cystadenoma of appendix is a rare clinical entity with very few reported cases in the literature. Consensus on optimal surgical management has not been reached. We report the case of a 65-year-old female patient who presented with fistula over the right iliac fossa. Computed tomography (CT) of the abdomen suggested abscess of the parietal wall. Upon exploration, a mass was found to be arising from the tip of the retroperitoneal appendix and the retroperitoneum was studded with mucoid material. Appendectomy was carried out and final histopathology revealed mucinous cystade- noma with no evidence of malignancy. The patient was discharged uneventfully. The unusual presentation of this disease, as retroperitoneal psuedomyxoma without any intraperitoneal pathology, prompted us to report this case. Keywords: appendiceal tumours; mucinous cystadenoma; psuedomyxoma peritonei INTRODUCTION Tumours of the appendix are very rare and the usual mode of diagnosis is incidental means most patients operated for appendicitis and if specimen sent for HPE, few will be having neoplastic changes following histopathological ex- amination of an appendectomy specimen done for appen- dicitis. According to a report published by the National Cancer Institute USA using the Surveillance, Epidemiology and End Results (SEER) database, appendiceal neoplasms account for approximately 0.4% of gastrointestinal tumours [1]. Even though these tumours are rare, they are histologically diverse—mainly mucinous epithelial neo- plasms, also called adenomas, cystadenoma, and benign neoplastic mucocele. Primary mucinous neoplasms of the appendix are found in less than 2% of surgically resected appendices [2]. Mucoceles that are less than 2 cm in diameter are usually simple retention cysts, while hyperplastic epithelium, cystadenoma and cystadenocarcinoma are more likely to be larger than 2 cm [3]. In developing countries, where non-availability of frozen section is a limitation, optimal management of mucinous adenomas is debatable, some surgeons advocating appendectomy only while others prefer right hemicolectomy. We report a rare case with unusual presentation as enterocutaneous fistula and retro- peritoneal psuedomyxoma. CASE PRESENTATION A 65-year-old female patient was admitted with complaints of a pus-discharging wound over the right inguinal region for a duration of 15 days. A local doctor misdiagnosed the swelling over the right iliac fossa as an abscess and tried an incision and drainage procedure, leading to the formation of a fistula. She was then referred to us. On examination ß The Author(s) 2014. Published by Oxford University Press and the Digestive Science Publishing Co. Limited. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/ licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. Downloaded from https://academic.oup.com/gastro/article-abstract/3/1/86/589157 by guest on 20 November 2018