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Background/Aims: Owing to the low prevalence of small-bowel adenocarcinoma (SBA), data on the impact of Crohn’s disease (CD) on the survival of patients with SBA are lacking. Therefore, we investigated this issue in this study. Methods: In this bicenter cohort study, patients with histologically confirmed SBA were retrospectively enrolled and classified into two groups: sporadic SBA and CD-associated SBA. Patients with duodenal SBA were excluded. Overall survival, disease-free survival, and factors associated with survival were analyzed. Results: Of 128 patients with SBA, 115 had sporadic SBA and 13 had CD-associated SBA. Ileal involvement and poorly differentiated tumors were more common in the CD-associated SBA group than in the sporadic SBA group (ileal involvement, 53.8% vs 22.6%; poor differentiation, 46.2% vs 14.8%; both p

Cancers of the small bowel could account for less than 5% of all gastrointestinal malignancies. Of these tumors, adenocarcinomas were the major histologic subtype and generally carried a poor prognosis. High expression of vascular epithelial growth factor (VEGF) could be seen in small bowel adenocarcinomas. A systematic review was conducted here to determine if bevacizumab, a recombinant humanized antibody against VEGF, could offer clinical benefit among patients with metastatic small bowel adenocarcinoma when combined with chemotherapy. A search for relevant published and unpublished studies was performed using PubMed, ScienceDirect, Google Scholar, the American Society of Clinical Oncology meetings library, ClinicalTrials.gov, and ISRCTN registry. Information on study design, methods, intervention, and outcomes were extracted from selected eligible studies. Methodological quality was then assessed using the Newcastle-Ottawa Scale. There was a significant improvement in mean overall survival with the addition of bevacizumab with chemotherapy versus chemotherapy alone. The use of bevacizumab with chemotherapy, likewise improved progression-free survival and objective response rate compared to chemotherapy alone. Continued use of bevacizumab beyond first progression also appeared to show benefit. The conduct of prospective controlled studies by consortia to offset the rarity of small bowel adenocarcinomas could further elucidate the efficacy of bevacizumab in the treatment of this disease. [ABSTRACT FROM AUTHOR]

Small-bowel tumors represent a rare entity comprising 0.6% of all new cancer cases in the US, and only 3% of all gastrointestinal neoplasms. They are a heterogenous group of neoplasms comprising of about forty different histological subtypes with the most common being adenocarcinoma, neuroendocrine tumors, stromal tumors and lymphomas. Their incidence has been reportedly increasing over recent years, partly owing to the advances and developments in the diagnostic modalities. Small-bowel capsule endoscopy, device assisted enteroscopy and dedicated small-bowel cross-sectional imaging are complimentary tools, supplementing each other in the diagnostic process. Therapeutic management of small-bowel tumors largely depends on the histological type and staging at diagnosis. The aim of the present review article is to discuss relevant advances in the diagnosis and management of small-bowel tumors., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this article., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Small-bowel adenocarcinoma (SBA) is a rare tumor of poor prognosis. Data on the efficacy of chemotherapy for advanced SBA are scarce. Patients and methods: All patients with advanced SBA who received frontline chemotherapy from 1996 to 2008 were eligible for this retrospective multicenter study. Results: Ninety-three consecutive patients were included. In the entire population, the median progression-free survival (PFS) and overall survival (OS) times were 6.6 and 15.1 months, respectively. Median PFS times among patients treated with LV5FU2 (n = 10), FOLFOX (n = 48), FOLFIRI (n = 19) and LV5FU2-cisplatin (n = 16) were 7.7, 6.9, 6.0 and 4.8 months, respectively, while median OS times were 13.5, 17.8, 10.6 and 9.3 months, respectively. In multivariate analysis, World Health Organization performance status (PS) (P < 0.0001) and elevated serum levels of carcinoembryonic antigen (CEA) (P = 0.02) and carbohydrate antigen 19-9 (CA 19-9) (P = 0.03) were the only variables significantly associated with poor OS. In the subgroup of patients treated with platinum-based chemotherapy, multivariate analysis showed that LV5FU2-cisplatin was associated with poorer PFS (P < 0.0001) and OS (P = 0.02) compared with FOLFOX. Conclusions: This is the largest study of chemotherapy in advanced SBA. Baseline PS and CEA and CA 19-9 levels were the main prognostic factors. FOLFOX seems to be the most effective platinum-based chemotherapy regimen. [ABSTRACT FROM AUTHOR]

Objective: Patients with Von Recklinghausen's disease (neurofibromatosis type 1) are at increased risk of developing various tumours. However, the coexistence of neurofibromatosis with small-bowel adenocarcinoma is exceedingly rare. We present an uncommon case of neurofibromatosis type 1, involving the small bowel in a 73-year-old man, who was admitted to our department with signs of acute abdomen. At laparotomy, multiple mesenteric and intramural nodules were seen in the distal ileum. These nodules obstructed ileal lumen, while the intestine wall was perforated in one point. A wide resection of the affected ileum together with all visible nodules in the adjacent mesentery was performed. Histology revealed neurofibromatosis type 1 with malignant transformation to small-bowel adenocarcinoma. The patient had no additional therapy. In a follow-up of 2 years, the patient is very well and there was no recurrence of the disease. We suggest that adenocarcinoma of small bowel should be considered in the evaluation of acute abdominal pain in neurofibromatosis patients. [ABSTRACT FROM AUTHOR]

Patients affected with Crohn's disease (CD) have a recognized, but low relative risk of developing small-bowel adenocarcinoma (SBA). In fact, SBA develops in 2.2% of patients who have long-standing CD and it is seldom diagnosed preoperatively because of its rarity. A retrospective analysis of all cases of SBA in CD patients since 1980 was carried out in Rouen University Hospital. Three patients with known or unknown CD who presented with SBA with long-term follow-up were analysed. In our first case, the occlusive syndrome revealed SBA and CD simultaneously. Most ileal carcinomas in CD are located in strictures and are often incidentally diagnosed postoperatively, as in our three cases. Digestive surgeons and gastroenterologists must be aware that the diagnosis of SBA in CD is often made fortuitously on histological examination after surgical resection for an occlusive syndrome. Failure to detect SBA in patients with CD results in late diagnosis, with poor survival. [ABSTRACT FROM AUTHOR]

Strictureplasties have proven useful and safe in Crohn's disease. Concerns have been raised, however, about the potential of carcinoma arising at the strictureplasty site. Here the authors report a case of a small-bowel adenocarcinoma developing at the site of a prior strictureplasty in a middle-aged male patient seven years postoperatively in the absence of any other preneoplastic disease of the small bowel. Presenting symptoms were of progressive obstruction after a long period of quiescent disease. With this report comes stronger evidence that adenocarcinoma does Occur at strictureplasty sites, raising questions of its long-term safety. [ABSTRACT FROM AUTHOR]

A 60-year-old woman with a medical history of celiac disease was evaluated due to recurrent episodes of small bowel obstruction. Upper and lower endoscopies were normal. The small bowel pathology consisted of celiac disease, and the anti-endomysial antibody was positive. Dilatation of small bowel loops was demonstrated on abdominal computed tomography. Further evaluation was conducted using video capsule endoscopy that demonstrated regional narrowing and severe ulceration in the middle of the small bowel. Upper and lower double balloon endoscopies failed to demonstrate the lesion. On explorative laparotomy a small bowel mass in the proximal ileum was excised. Pathology revealed ulcerated, well to moderately differentiated adenocarcinoma without regional nodal involvement. We discuss the etiology and treatment of small bowel carcinoma. This case emphasizes that a high level of suspicion is required in order to diagnose early stage small bowel adenocarcinoma in celiac patients. [ABSTRACT FROM AUTHOR]

Background: As small-bowel adenocarcinoma (SBA) is scarce, no standard systemic regimen in metastatic disease has been defined., Objective: To obtain insights into the use and effects of palliative chemotherapy in patients with metastatic SBA in a population-based setting., Methods: Data from the Netherlands Cancer Registry of patients with metastatic SBA between 2007 and 2016 were used ( n  = 522). For patients treated with palliative chemotherapy, differences in treatment regimens and survival were evaluated., Results: Palliative chemotherapy was received by 38% of patients ( n  = 199). First-line combination chemotherapy was administered to 80% of patients, mainly CAPOX/FOLFOX. Single-agent chemotherapy mostly consisted of capecitabine. Second-line treatment, mostly irinotecan-based (58%), was prescribed to 27% of patients. Age 70 years or older was an adverse predictive factor for receiving first-line combination chemotherapy (odds ratio (OR) 0.2, 95% confidence interval (CI) 0.08-0.62) and second-line therapy (OR 0.3, 95% CI 0.10-0.72). Median overall survival with palliative chemotherapy was 9.3 months, compared with 3.0 months without. In subanalyses, patients who received only first-line treatment had a median overall survival of 5.6 and 7.0 months after single-agent and combination chemotherapy, respectively., Conclusion: A minority of patients were treated with palliative chemotherapy. First-line treatment consisted predominantly of oxaliplatin-based combination chemotherapy, whereas second-line treatment was mainly irinotecan-based. Population-based median overall survival for selected patients treated with chemotherapy amounted to nine months., (© Author(s) 2019.)