Small bowel adenocarcinoma: Results from a nationwide prospective ARCAD‐NADEGE cohort study of 347 patients

Abstract

Small bowel adenocarcinoma (SBA) is a rare tumour. We conducted a prospective cohort to describe the prevalence, survival and prognostic factors in unselected SBA patients. The study enrolled patients with all stages of newly diagnosed or recurrent SBA at 74 French centres between January 2009 and December 2012. In total, 347 patients were analysed; the median age was 63 years (range 23–90). The primary tumour was in the duodenum (60.6%), jejunum (20.7%) and ileum (18.7%). The prevalence of predisposing disease was 8.7%, 6.9%, 1.7%, 1.7% and 0.6% for Crohn disease, Lynch syndrome, familial adenomatous polyposis, celiac disease and Peutz‐Jeghers syndrome, respectively. At diagnosis, 58.9%, 5.5% and 35.6% of patients had localised and resectable, locally advanced unresectable and metastatic disease, respectively. Crohn disease was significantly associated with younger age, poor differentiation and ileum location, whereas Lynch syndrome with younger age, poor differentiation, early stage and duodenum location. Adjuvant chemotherapy (oxaliplatin‐based in 89.9%) was performed in 61.5% of patients with locally resected tumours. With a 54‐months median follow‐up, the 5‐year overall survival (OS) was 87.9%, 78.2% and 55.5% in Stages I, II and III, respectively. The median OS of patients with Stage IV was 12.7 months. In patients with resected tumours, poor differentiation (p = 0.047) and T4 stage (p = 0.001) were associated with a higher risk of death. In conclusion, our study showed that the prognosis of advanced SBA remains poor. Tumour characteristics differed according to predisposing disease. In SBA‐resected tumours, the prognostic factors for OS were grade and T stage.

Efficacy of surgery and chemotherapy for stage IV small bowel adenocarcinoma: A population‐based analysis using Surveillance, Epidemiology, and End Result Program database

“…Besides, chemotherapy also significantly associated with better survival in our analysis, no matter surgery was performed or not. For stage IV or unresectable SBA patients, several analyses found that palliative chemotherapy could prolong survival compared with best supportive care or without treatment 5,18,19,24,26 . Among different chemotherapy regimens, combination chemotherapy including platinum and 5‐FU are primarily recommended as yet, with well tolerance and better OS 6,8‐11,21,22,25‐27 .…”

“…For stage IV or unresectable SBA patients, several analyses found that palliative chemotherapy could prolong survival compared with best supportive care or without treatment 5,18,19,24,26 . Among different chemotherapy regimens, combination chemotherapy including platinum and 5‐FU are primarily recommended as yet, with well tolerance and better OS 6,8‐11,21,22,25‐27 . As distinct genomic profiling of SBA was revealed compared with CRC and gastric carcinoma, 28 it seems unreasonable that the approach proposed for CRC or gastric carcinoma was completely adopted for the treatment of SBA.…”

Loss of HES-1 Expression Predicts a Poor Prognosis for Small Intestinal Adenocarcinoma Patients

“…Proximal small intestinal carcinomas are sometimes accompanied by background gastric metaplasia, suggesting a gastric metaplasia-dysplasia-carcinoma sequence, or pancreaticobiliary differentiation. In contrast, distal small intestine carcinomas are significantly associated with Crohn's disease (4).…”

“…This discrepancy might be due to the small proportion of non-tubular type of tumors (9.2%, 17/185) in this study, comparing to higher proportion of them (44.7%, 34/76) in the study of Vanoli et al Further studies utilizing large numbers of non-tubular type of small intestinal adenocarcinomas are needed to establish the prognostic power of different histologic features. It has been hypothesized that the pathogenesis of small intestinal adenocarcinoma varies depending upon the tumor location (4). Proximal small intestinal carcinomas are sometimes accompanied by background gastric metaplasia, suggesting a gastric metaplasia-dysplasia-carcinoma sequence, or pancreaticobiliary differentiation.…”

“…The majority of small intestinal adenocarcinoma patients are diagnosed at an advanced disease stage because of a lack of early detection tools, low incidence, and nonspecific clinical symptoms (1,3). A prospective ARCAD-NADEGE cohort study found that 35.6% of patients with small intestinal adenocarcinomas were diagnosed with metastatic disease, compared to 15.6% of those with colorectal cancer (4). The incidence of small intestinal adenocarcinoma is increasing while that of colorectal cancer is declining (5).…”