Published online Sep 28, 2015. doi: 10.3748/wjg.v21.i36.10461
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
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.
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.