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Cancers
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Small Bowel Adenocarcinoma
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Small Bowel Adenocarcinoma

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Causes, Screening and Diagnosis".

Deadline for manuscript submissions: closed (15 December 2021) | Viewed by 15384

Special Issue Editor

Dr. Aziz Zaanan

E-Mail Website
Guest Editor
Gastrointestinal Oncology Department, European Georges Pompidou Hospital, APHP.Centre, University of Paris, 20 rue Leblanc, 75015 Paris, France
Interests: gastrointestinal cancers; biomarkers; microsatellite instability; immunotherapy; circulating tumor DNA

Special Issue Information

Dear Colleagues,

Small bowel adenocarcinomas (SBAs) are rare tumors, but their incidence is increasing. Its rarity restricts molecular understanding but presents unique diagnostic and therapeutic challenges. Even though these tumors are most often sporadic, some predisposing diseases have been identified, among which are Crohn’s disease and genetic syndromes. Early diagnosis of SBA remains difficult despite significant radiological and endoscopic progress. The therapeutic approach of SBA is often confused with those of colorectal adenocarcinoma, whereas these two tumors present distinct clinical and molecular entities. Recent advances in the molecular characterization of SBA have led to a better understanding of its pathogenesis and offer opportunities for the development of new targeted therapies. This Special Issue will summarize current data concerning the following topics: epidemiology, risk factors and diagnosis of SBA—molecular characteristics of SBA—and treatment of localized and advanced SBA with perspectives.

Dr. Aziz Zaanan
Guest Editor

Manuscript Submission Information

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Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • small bowel adenocarcinomas
  • molecular alterations
  • rare tumor
  • surgery
  • chemotherapy

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Published Papers (5 papers)

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Research

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11 pages, 423 KiB  
Article
Gall Bladder Disease and the Risk of Small Bowel Cancer—Results from a Nationwide Swedish Cohort Study
by Louise Emilsson, Cecilia Radkiewicz, Carol E. Semrad, Amit D. Joshi and Jonas F. Ludvigsson
Cancers 2022, 14(3), 469; https://doi.org/10.3390/cancers14030469 - 18 Jan 2022
Cited by 1 | Viewed by 2125
Abstract
Background and aims: Small bowel cancer is a rare but rising malignancy. The etiology is poorly understood and there is a need for large-scale studies. Gallbladder disease (GBD), inducing localized inflammation, has been suggested to increase small bowel cancer risk. Methods: We retrieved [...] Read more.
Background and aims: Small bowel cancer is a rare but rising malignancy. The etiology is poorly understood and there is a need for large-scale studies. Gallbladder disease (GBD), inducing localized inflammation, has been suggested to increase small bowel cancer risk. Methods: We retrieved nationwide data from Sweden’s 28 pathology departments on all adults (age 20–79) with pathology-confirmed GBD diagnosed in 1965–2017. In total 156,390 GBD patients were matched with up to 5 matched comparators from the general population and follow-up started one year after GBD diagnosis. We used stratified Cox regression to calculate hazard ratios (HRs) for small bowel adenocarcinoma, adenomas, and carcinoids. Results: During a median follow-up of 12 years, we identified 92 small bowel adenocarcinomas, 132 adenomas, and 81 carcinoid tumors in the GBD cohort. Corresponding incidence rates were 4.8, 6.9, and 4.2 per 100,000 person-years (PY), compared to 3.2, 3.2, and 1.8 in matched comparators. The adjusted HR was 1.42 (95% CI = 1.08–1.87) for small bowel adenocarcinoma, 1.79 (95% CI = 1.41–2.27) for adenoma, and 2.07 (95% CI = 1.52–2.81) for carcinoid. The excess cancer risk was most pronounced during the first year of follow-up for adenocarcinomas and during the first six years for adenomas while for carcinoids the HR peaked 10–15 years after start of follow-up. Conclusions: In this nationwide cohort study, GBD was associated with an increased risk of small bowel cancer. The excess risk of small bowel adenocarcinoma was mainly seen during the first years of follow-up while small bowel carcinoid risk peaked 11–16 years after GBD diagnosis. Full article
(This article belongs to the Special Issue Small Bowel Adenocarcinoma)
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Review

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18 pages, 336 KiB  
Review
Localized Small Bowel Adenocarcinoma Management: Evidence Summary
by Anthony Turpin, Mehdi El Amrani and Aziz Zaanan
Cancers 2022, 14(12), 2892; https://doi.org/10.3390/cancers14122892 - 11 Jun 2022
Cited by 12 | Viewed by 2424
Abstract
Small bowel cancers are rare diseases whose prognosis is poorer than that of colon cancers. Due to disease rarity, there is little data on small bowel adenocarcinoma (SBA) treatment, and most recommendations come from expert agreements or analogies to the management of colon [...] Read more.
Small bowel cancers are rare diseases whose prognosis is poorer than that of colon cancers. Due to disease rarity, there is little data on small bowel adenocarcinoma (SBA) treatment, and most recommendations come from expert agreements or analogies to the management of colon cancer. Although relatively high rates of local recurrence are observed for duodenal malignancies, distant metastatic relapse remains common and requires adjuvant systemic therapy. Given the similarities between SBA and colorectal cancer, radiotherapy and chemotherapy strategies used for the latter disease are frequently pursued for the former disease, specifically for tumors located in the duodenum. However, no previous randomized study has evaluated the benefit of adjuvant chemotherapy on the overall survival of SBA patients. Most previous studies on treatment outcomes and prognostic factors in this context were based on large international databases, such as the Surveillance, Epidemiology, and End Results or the National Cancer Database. Studies are required to establish and validate prognostic and predictive markers relevant in this context to inform the use of (neo) adjuvant treatment. Among those, deficient mismatch repair tumors represent 20% of SBAs, but their impact on chemosensitivity remains unknown. Herein, we summarize the current evidence on the management of localized SBA, including future perspectives. Full article
(This article belongs to the Special Issue Small Bowel Adenocarcinoma)
11 pages, 297 KiB  
Review
Epidemiology, Risk Factors and Diagnosis of Small Bowel Adenocarcinoma
by Thomas Aparicio, Atanas Pachev, Pierre Laurent-Puig and Magali Svrcek
Cancers 2022, 14(9), 2268; https://doi.org/10.3390/cancers14092268 - 2 May 2022
Cited by 19 | Viewed by 3233
Abstract
Adenocarcinomas of the small intestine are rare tumors but their incidence is increasing. There is a slight male predominance. The median age at diagnosis is the 6th decade. The most frequent primary location is the duodenum. There is no clearly identified environmental risk [...] Read more.
Adenocarcinomas of the small intestine are rare tumors but their incidence is increasing. There is a slight male predominance. The median age at diagnosis is the 6th decade. The most frequent primary location is the duodenum. There is no clearly identified environmental risk factor, but adenocarcinomas of the small intestine are associated in almost 20% of cases with predisposing diseases (Crohn’s disease, Lynch syndrome, familial adenomatous polyposis, Peutz–Jeghers syndrome and celiac disease). Full article
(This article belongs to the Special Issue Small Bowel Adenocarcinoma)
14 pages, 1516 KiB  
Review
Molecular Landscape of Small Bowel Adenocarcinoma
by Karan Pandya, Michael J. Overman and Pat Gulhati
Cancers 2022, 14(5), 1287; https://doi.org/10.3390/cancers14051287 - 2 Mar 2022
Cited by 10 | Viewed by 4505
Abstract
Small bowel adenocarcinoma (SBA) is a rare malignancy, with lower incidence, later stage at diagnosis, and poor overall prognosis compared to other cancers of the gastrointestinal tract. Owing to the rarity of the disease along with the paucity of high-quality tissue samples and [...] Read more.
Small bowel adenocarcinoma (SBA) is a rare malignancy, with lower incidence, later stage at diagnosis, and poor overall prognosis compared to other cancers of the gastrointestinal tract. Owing to the rarity of the disease along with the paucity of high-quality tissue samples and preclinical models, little is known about the molecular alterations characteristic of SBA. This is reflected by the fact that the clinical management of SBA is primarily extrapolated from colorectal cancer (CRC). Recent advances in genomic profiling have highlighted key differences between these tumors, establishing SBA as a molecularly unique intestinal cancer. Moreover, comprehensive molecular analysis has identified a relatively high incidence of potentially targetable genomic alterations in SBA, predictive of response to targeted and immunotherapies. Further advances in our knowledge of the mutational and transcriptomic landscape of SBA, guided by an increased understanding of the molecular drivers of SBA, will provide opportunities to develop novel diagnostic tools and personalized therapeutic strategies. Full article
(This article belongs to the Special Issue Small Bowel Adenocarcinoma)
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16 pages, 295 KiB  
Review
Therapeutic Strategies for Patients with Advanced Small Bowel Adenocarcinoma: Current Knowledge and Perspectives
by Emilie Moati, Michael J. Overman and Aziz Zaanan
Cancers 2022, 14(5), 1137; https://doi.org/10.3390/cancers14051137 - 23 Feb 2022
Cited by 6 | Viewed by 2215
Abstract
Small bowel adenocarcinoma (SBA) is diagnosed at an advanced (unresectable or metastatic) tumor stage in approximately one-third of cases. This is partly due to the non-specific symptomatology and limitations in endoscopic and radiologic detection methods. In this context, the prognosis remains poor and [...] Read more.
Small bowel adenocarcinoma (SBA) is diagnosed at an advanced (unresectable or metastatic) tumor stage in approximately one-third of cases. This is partly due to the non-specific symptomatology and limitations in endoscopic and radiologic detection methods. In this context, the prognosis remains poor and systemic chemotherapy appears to benefit patients when compared to best supportive care alone, despite the absence of randomized controlled trials. The results of a recent large prospective cohort (ARCAD-NADEGE) reported that the absence of chemotherapy was a predictive factor for a lower overall survival (OS) even though poor differentiation and SBA associated with Crohn’s disease correlate with poor prognosis. In retrospective series, the median OS ranges from approximately 9 to 18 months with current treatment approaches. A combination of a fluoropyrimidine and oxaliplatin (FOLFOX or CAPOX) appears to be the most utilized and effective first-line chemotherapy regimen. Other front-line alternatives are the combination of 5-FU and cisplatin or fluoropyrimidine and irinotecan (FOLFIRI). In second-line, FOLFIRI is an effective option after progression on platinum-based therapy. Taxane-based therapy appears to be an alternative option, but further evaluation in larger series is needed. To a limited extent, the role of surgical resection for metastatic disease appears to be a valid option, though this approach has not been evaluated in prospective clinical studies. Due to the rareness of the disease, inclusion in clinical trials should be prioritized, and there is hope that targeted therapies and immunotherapy may enter the therapeutic arsenal for these patients. Full article
(This article belongs to the Special Issue Small Bowel Adenocarcinoma)
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