Celiac.com 9/26/2019 - Small bowel adenocarcinoma is a rare abnormal tissue growth, that can happen alone, or can also be the result of predisposing conditions, including hereditary syndromes and immune-mediated intestinal disorders, such as celiac disease.
However, researchers still don't know very much about small bowel adenocarcinoma in the context of celiac disease. To get some answers, a research team recently set out to show the main clinical features, diagnostic procedures and management options of small bowel adenocarcinoma cases detected in a large cohort of celiac patients diagnosed in a single tertiary care center.
They are variously affiliated with the Department of Medical Sciences, University of Ferrara, St. Anna Hospital in Ferrara, Italy; the Mucosal Immunology and Biology Research Center and Celiac Center, Massachusetts General Hospital- Harvard Medical School, Boston, MA, USA; the Department of Medical and Surgical Sciences, University of Bologna in Bologna, Italy; and the Department of Medical Sciences, University of Ferrara, St. Anna Hospital, Ferrara, Italy.
The team retrospectively reviewed all small bowel adenocarcinoma cases from a group of 770 celiac disease patients of the Celiac Disease Referral Center at the University Hospital in Bologna, Italy from January 1995 to December 2014. The group included nearly 600 females, spanned 18 to 80 years of age, and averaged 36 years old at diagnosis.
A total of five of the 770 celiac disease patients developed small bowel adenocarcinoma. All were female, and about 53 years old on average, though the individuals ranged from 38 to 72 years old.
The small bowel adenocarcinoma was diagnosed along with the celiac disease in three cases. It was localized to the jejunum in four cases, and to the duodenum in one case.
The clinical presentation of small bowel adenocarcinoma was characterized by intestinal sub-occlusion in two cases, while the main presentation in the other three cases were iron deficiency anaemia, abdominal pain and acute intestinal obstruction, respectively.
All the patients underwent surgery, while three patients with advanced stage neoplasia also received chemotherapy. The overall survival rate at 5 years was 80% for the group.
The observed celiac disease-related small bowel adenocarcinoma cases were marked by a younger age of onset, were mainly female, and faced better odds of survival, compared with sporadic, Crohn- and hereditary syndrome-related small bowel adenocarcinoma.