Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Sep 28, 2015; 21(36): 10461-10467
Published online Sep 28, 2015. doi: 10.3748/wjg.v21.i36.10461
Jejunitis and brown bowel syndrome with multifocal carcinogenesis of the small bowel
Martin Raithel, Tilman T Rau, Alexander F Hagel, Heinz Albrecht, Thomas de Rossi, Thomas Kirchner, Eckhart G Hahn
Martin Raithel, Alexander F Hagel, Heinz Albrecht, Thomas de Rossi, Eckhart G Hahn, Department of Medicine 1, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen D-91054, Germany
Tilman T Rau, Department of Pathology, Friedrich-Alexander-University Erlangen-Nuremberg, Erlangen D-91054, Germany
Thomas Kirchner, Department of Pathology, Ludwig-Maximilians-University Munich, Munich D-80337, Germany
Author contributions: Raithel M and Hahn EG conceived of the study and wrote and revised the paper; Rau TT and Kirchner T carried out the pathologic investigations; Hagel AF, de Rossi T and Albrecht H participated in data collection and design and coordination of the study; All authors read and approved the final manuscript.
Supported by Deutsche Forschungsgemeinschaft and Friedrich-Alexander-University Erlangen-Nuremberg within the funding program Open Access Publishing.
Institutional review board statement: As the case report was written retrospectively, it was not antecedently reviewed by the local ethics committee of the Friedrich-Alexander-University Erlangen-Nuremberg.
Informed consent statement: The patient provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare that they have no competing interests.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Heinz Albrecht, MD, Department of Medicine 1, Friedrich-Alexander-University Erlangen-Nuremberg, University Hospital Erlangen, Ulmenweg 18, Erlangen D-91054, Germany. heinz.albrecht@uk-erlangen.de
Telephone: +49-9131-8535000 Fax: +49-9131-8535252
Received: March 9, 2015
Peer-review started: March 11, 2015
First decision: March 26, 2015
Revised: May 15, 2015
Accepted: June 26, 2015
Article in press: June 26, 2015
Published online: September 28, 2015
Abstract

This is the first report describing a case where prolonged, severe malabsorption from brown bowel syndrome progressed to multifocally spread small bowel adenocarcinoma. This case involves a female patient who was initially diagnosed with chronic jejunitis associated with primary diffuse lymphangiectasia at the age of 26 years. The course of the disease was clinically, endoscopically, and histologically followed for 21 years until her death at the age 47 due to multifocal, metastasizing adenocarcinoma of the small bowel. Multiple lipofuscin deposits (so-called brown bowel syndrome) and severe jejunitis were observed microscopically, and sections of the small bowel showed dense lymphoplasmacytic infiltration of the lamina propria as well as blocked lymphatic vessels. After several decades, multifocal nests of adenocarcinoma cells and extensive, flat, neoplastic mucosal proliferations were found only in the small bowel, along with a loss of the mismatch repair protein MLH1 as a long-term consequence of chronic jejunitis with malabsorption. No evidence was found for hereditary nonpolyposis colon carcinoma syndrome. This article demonstrates for the first time multifocal carcinogenesis in the small bowel in a malabsorption syndrome in an enteritis-dysplasia-carcinoma sequence.

Keywords: Brown bowel syndrome, Carcinogenesis of the small bowel, Enteritis-dysplasia-carcinoma sequence, Malabsorption

Core tip: Severe malabsorption associated with brown bowel syndrome can progress to multifocally spread small bowel adenocarcinoma. This report describes the clinical course of a woman suffering from a long-lasting malabsorption syndrome who developed small bowel adenocarcinoma in an enteritis-dysplasia-carcinoma sequence. After several decades of chronic jejunitis with malabsorption, multifocal nests of adenocarcinoma cells were found only in the small bowel without evidence of hereditary nonpolyposis colon carcinoma syndrome.