The CD diagnosis was made according to duodenal histopathology, serology and Human Leukocyte Antigen (HLA) genotyping (HLA-DQ2 -DQA1*05 and DQB1*02 with detection of homozygosity and HLA-DQ8 - DQB1*0302). Orsola-Malpighi University Hospital (Bologna, Italy). Also, the information derived from this study should aid an early recognition of a very aggressive tumor usually associated with a poor quality of life and a significantly reduced life expectancy for CD patients.įrom January 1995 to December 2014, 770 CD patients (599 females F / M ratio 3.5:1, median age at diagnosis 36 years, range 18–80 years) were diagnosed at the Celiac Disease Referral Center of the St. In the present study, we aimed to provide physicians with the main clinical features, diagnostic procedures and management options of our cases of SBA detected in a large cohort of CD patients observed in a single tertiary care center in a time-frame of 20 years. As most intestinal tumors, also SBA may recognize the classic ‘adenoma-to-carcinoma’ sequence, although this hypothesis is still debated. The link between SBA and CD has been explored in a few case reports implying that a variety of putative mechanisms, e.g., chronic inflammation, increased permeability to cancerogenic factors, malabsorption of anti-cancerogenic substances (e.g., vitamins) as well as impaired immune surveillance can contribute to the pathogenesis of SBA arising from CD. Similarly, the prevalence of CD is spreading worldwide, accounting for more than 1% of the general population. In the last years the incidence of SBA is increasing according to the data from the surveillance epidemiology and end results (SEER) showing an overall incidence of 22.7 cases per million in the US population in 2004. Another study involving a metanalysis of 79,000 CD patients estimated an odd ratio of 14.41 for developing SBA.
CELIAC DISEASE COLONOSCOPY FINDINGS FREE
Nowadays it is largely agreed that a delay in CD diagnosis and a low compliance with gluten free diet (GFD) are two major factors leading to a 60–80 fold relative risk of developing SBA. The association between CD and SBA was first described more than 40 years ago and, in contrast to the general population, CD patients have a markedly increased risk of developing SBA. familial adenomatous polyposis, Lynch syndrome Peutz-Jeghers syndrome and others, and immune-related intestinal disorders (i.e., Crohn’s disease and celiac disease, CD). SBA can be detected in patients without any associated intestinal disorder (i.e., sporadic SBA) or it can occur in association with predisposing conditions, including hereditary conditions, e.g. The duodenum is the most common localization for this neoplasia accounting for 55–82% of cases, followed by jejunum (11–25%) and ileum (7–17%). Similar findings were described in the US population, with 5300 new cases and 1100 deaths per year. In Europe the estimated rate of new cases per year is 3600, with a median age in the seventh decade of life. Nonetheless, the small bowel adenocarcinoma (SBA) is an extremely rare neoplasia, with an incidence of 5.7 cases per million people. The small bowel accounts for more than 75% of the whole gastrointestinal tract and 90% of its mucosal surface. ConclusionsĪlthough in a limited series, herein presented CD-related SBA cases were characterized by a younger age of onset, a higher prevalence in female gender and a better overall survival compared to sporadic, Crohn- and hereditary syndrome-related SBA. The overall survival rate at 5 years was 80%. All the patients were referred to surgery, and three cases with advanced stage neoplasia were also treated with chemotherapy. The clinical presentation of SBA was characterized by intestinal sub-occlusion in two cases, while the predominant manifestation of the remaining three cases was iron deficiency anaemia, abdominal pain and acute intestinal obstruction, respectively. SBA, diagnosed at the same time of the CD diagnosis in three cases, was localized in the jejunum in four cases and in the duodenum in one case. All of them were female with a mean age of 53 years (range 38–72 years). Resultsįive (0.65%) out of our 770 CD patients developed SBA. We retrospectively reviewed all the SBA cases detected in a cohort of 770 CD patients (599 females F / M ratio: 3.5:1 median age at diagnosis 36 years, range 18–80 years), diagnosed at the Celiac Disease Referral Center of our University Hospital (Bologna, Italy) from January 1995 to December 2014. This study was aimed to show the main clinical features, diagnostic procedures and management options of SBA cases detected in a large cohort of celiac patients diagnosed in a single tertiary care center. However, the features of SBA in the context of CD remain only partly understood.
Small bowel adenocarcinoma (SBA) is a rare neoplasm, which can occur in a sporadic form or can be associated with a number of predisposing conditions such as hereditary syndromes and immune-mediated intestinal disorders, e.g.
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