• Humans
• Crohn Disease/diagnosis
• Crohn Disease/therapy
• Gastroenterology/standards
• Germany

• adenocarcinoma of the ileum
• ileal adenocarcinoma
• no risk factors
• partial ileal resection
• small bowel adenocarcinoma
• small bowel neoplasm

Small bowel adenocarcinoma (SBA) is an unusual complication of Crohn’s disease (CD).

To describe the incidence rate, clinical features, and outcomes of SBA in a group of subsequent patients with CD.

We retrospectively analysed outpatient and hospital records and identified a group of patients with diagnosed CD. Then we reviewed all medical records of patients who reported for follow-up visits in a 14-year period. We identified a group of 103 patients, whose medical records were evaluated for the presence of SBA.

Long-term follow-up carried out in the group of consecutively treated patients with CD revealed an 0.97% incidence rate of SBA. Cancer was located in the ileum and the clinical presentation was subileus and anaemia. The patient underwent ileocaecal resection, and the postoperative period was uneventful.

SBA is a rare complication of CD. Although the authors are aware that the number of patients enrolled in the study is insufficient to draw far-reaching conclusions, the results obtained are significant for determination of the incidence rate of SBA in the Caucasian population of patients with CD. The key issue of effective treatment of patients with SBA was early detection of the lesion and R0 resection with proper lymphadenectomy. However, it is worth noting that in more advanced stages of SBA the future belongs to, and outcome improvement depends on, new regimes of adjuvant personalised chemotherapies. Further studies on the mechanisms of carcinogenesis in patients with CD are essential.

• Crohn’s disease
• bowel
• cancer
• inflammatory bowel disease
• small bowel adenocarcinoma

• colon cancer
• crohn's disease
• gastrointestinal surgery
• surgical oncology

Small bowel adenocarcinoma is a rare but well-known complication of Crohn's disease. The diagnosis of small bowel adenocarcinoma remains difficult since its presentation is highly variable and mimics active or obstructive Crohn's disease. The diagnosis is often delayed and typically detected only at surgery in an advanced stage with a poor prognosis. We report a case of metastatic ileal adenocarcinoma in a patient with Crohn's disease with prolonged survival. Our case describes serial promising treatment options of these advanced malignancies and raises a possible role for checkpoint immunotherapy.

• CD
• NET
• UC
• intestinal cancer

Background: Small bowel adenocarcinoma (SBA) is associated with a poor prognosis. It is an uncommon malignancy and therefore difficult to study. Randomized phase III trials are not available to guide best approaches. The Provincial Cancer Registry of the British Columbia Cancer Agency contains long-term data on patients with SBA. The authors analyzed characteristics and treatment outcomes for SBA patients diagnosed between 1990 and 2008.

Material and methods: Charts of 150 patients with a histological diagnosis of SBA were retrospectively analyzed. Epidemiological and treatment data were collected. Disease-free survival (DFS) and overall survival (OS) were estimated by the Kaplan-Meier method.

Results: Baseline characteristics, such as median age at diagnosis (64.5 years), tumor stage (I-II 33%, III-IV 58%, unknown 9%), and location (duodenum 48%, jejunum 31%, ileum 21%) were consistent with published data. 55% of patients had a positive family history of cancer. DFS and OS of 29 patients treated with adjuvant chemotherapy were not significantly different to that of 47 patients without (p = 1 and p = 0.211, respectively). In the palliative setting patients treated with polychemotherapy (21 patients) had statistically better OS than patients treated with monochemotherapy (12 patients) (p = 0.0228).

Conclusions: Our study suggests a survival benefit for advanced-stage SBA patients treated with poly- versus monochemotherapy. This, however, was a retrospective analysis with several potential confounders. Nevertheless, our study adds to the evidence suggesting that chemotherapy may be beneficial for patients with SBA, at least in the palliative setting.

• adenocarcinoma
• adjuvant
• chemotherapy
• palliative.
• small bowel

Although Crohn's disease has been associated with an increased risk of small bowel adenocarcinoma, primary adenocarcinoma arising from an ileostomy is a complication that has been rarely documented in Crohn's disease. Chronic small bowel inflammation may lead to development of malignancy through the dysplasia-carcinoma sequence. We report a case of a 61-year-old woman with Crohn's ileocolitis diagnosed with a primary adenocarcinoma at the ileostomy with metastases to the liver 47 years after proctocolectomy, and review the literature.

Adenocarcinoma of small intestines (SBA) is a relatively rare malignancy with poor outcomes due to delayed diagnosis. Fifty percent of patients have metastases on presentation and therefore early detection and treatment offers the best long term outcomes. Certain genetic polyposis syndromes and familial diseases are associated with increased risks for SBA. These include familial adenomatous polyposis (FAP), Lynch syndromes (LS), Juvenile polyposis syndrome, Peutz-Jeghers syndrome, Crohn’s disease (CD) and celiac disease. Mutations in APC gene, Mismatch repair genes, STK11 gene, and SMAD4 gene have been implicated for the genetic diseases respectively. While there are no specific inherited genetic mutations for CD, genome-wide association studies have established over 140 loci associated with CD. CpG island mutations with defects in mismatch repair genes have been identified in celiac disease. Significant diagnostic advances have occurred in the past decade and intuitively, it would seem beneficial to use these advanced modalities for surveillance of these patients. At present it is debatable and no clear data exists to support this approach except for established guidelines to diagnose duodenal polyps in FAP, and LS. Here we discuss the genetic alterations, cancer risks, signaling mechanisms and briefly touch the surveillance modalities available for these genetic and clinical syndromes. English language articles from PubMed/Medline and Embase was searched were collected using the phrases “small-bowel adenocarcinoma, genetics, surveillance, familial adenomatous polyposis, lynch syndromes, Peutz-Jeghers syndrome, juvenile polyposis syndrome, CD and celiac disease”. Figures, tables and schematic diagram to illustrate pathways are included in the review.

• Small intestinal adenocarcinoma
• Genetic risks
• Mutations
• Signaling pathways
• Surveillance

Crohn’s disease (CD) is a chronic remittent idiopathic disease. Although the early phase of the disease is commonly characterized by inflammation-driven symptoms, such as diarrhea, the frequency of fibrostenotic complications in patients with CD increases over the long-term course of the disease. This review presents the current diagnostic options for assessing CD-associated strictures. In addition to the endoscopic evaluation of CD strictures, this review summarizes the currently available imaging modalities, including ultrasound and cross-sectional imaging techniques. In addition to stricture detection, differentiating between the primarily inflammatory strictures and the predominantly fibrotic ones is essential for selecting the appropriate treatment strategy (anti-inflammatory medical treatment vs endoscopical or surgical approaches). Therefore, recent imaging advances, such as contrast-enhanced ultrasound and ultrasound elastography, contribute to the development of non-invasive non-radiating imaging of CD-associated strictures. Finally, novel magnetic resonance imaging techniques, such as diffusion-weighted, motility and magnetization transfer imaging, as well as ¹⁸F-FDG PET/CT, molecular imaging approaches and biomarkers, are critically reviewed with regard to their potential role in assessing stricturing CD.

• Crohn’s disease
• Inflammatory bowel disease
• Intestinal stricture
• Endoscopy
• Magnetic resonance imaging
• Ultrasound
• Computed tomography
• ¹⁸F-FDG PET/CT CEUS
• Elastography

• Constriction, Pathologic
• Crohn Disease/complications
• Crohn Disease/diagnosis
• Diagnostic Imaging/methods
• Endoscopy, Gastrointestinal/methods
• Humans
• Intestinal Obstruction/diagnostic imaging
• Intestinal Obstruction/etiology
• Intestines/diagnostic imaging
• Predictive Value of Tests
• Prognosis

This review of the literature on small bowel carcinoma associated with Crohn’s disease specifically addresses the incidence, risk factors, and protective factors which have been identified. It also reviews the clinical presentation, the current modalities of diagnosis, the pathology, treatment, and surveillance. Finally, the prognosis and future direction are addressed. Our experience with small bowel adenocarcinoma in Crohn’s disease is reported. Readers will be provided with a better understanding of this rare and often poorly recognized complication of Crohn’s disease.

• Crohn’s disease
• Small bowel adenocarcinoma
• Cancer risk
• Cancer malignancy
• Incidental carcinoma
• Late complications of Crohn’s disease
• Inflammatory bowel disease

• Adenocarcinoma/diagnosis
• Adenocarcinoma/epidemiology
• Adenocarcinoma/therapy
• Crohn Disease/diagnosis
• Crohn Disease/epidemiology
• Crohn Disease/therapy
• Humans
• Incidence
• Intestinal Neoplasms/diagnosis
• Intestinal Neoplasms/epidemiology
• Intestinal Neoplasms/therapy
• Intestine, Small/drug effects
• Intestine, Small/pathology
• Intestine, Small/surgery
• Predictive Value of Tests
• Prognosis
• Protective Factors
• Risk Assessment
• Risk Factors

• Humans
• Inflammatory Bowel Diseases/diagnosis

• Adenocarcinoma
• Carcinoid tumours
• Diagnosis of small bowel tumours
• Gastrointestinal stromal tumours
• Management of small bowel tumours
• Small bowel cancer
• Small bowel tumours

• Consensus
• Crohn's disease
• Histopathology
• Inflammatory bowel disease
• Ulcerative colitis

• Adenocarcinoma/complications
• Adenocarcinoma/secondary
• Adenocarcinoma/therapy
• Adult
• Crohn Disease/etiology
• Crohn Disease/pathology
• Crohn Disease/therapy
• Diarrhea/complications
• Humans
• Ileitis/etiology
• Ileitis/pathology
• Ileitis/therapy
• Liver Neoplasms/complications
• Liver Neoplasms/pathology
• Liver Neoplasms/therapy
• Magnetic Resonance Imaging
• Male

A 40-year-old male, diagnosed with mild Crohn’s disease (CD) 11 years ago but with no prior abdominal surgeries, was diagnosed with a small bowel stricture, due to ongoing abdominal pain and intolerance of enteral diet, and referred for surgical treatment. Exploratory laparoscopy revealed a white solid mass causing a near total jejunal obstruction with significant proximal dilatation. An adjacent small node was sampled for frozen biopsy, revealing a lymph node infiltrated with adenocarcinoma. Laparoscopic assisted small bowel resection and appendectomy were carried out. Final pathological results supported the initial report of diffuse small bowel adenocarcinoma. In conclusion, once a small bowel stricture associated with CD is suspected, rapid action should be considered to avoid late diagnosis of a neoplasia.

• Inflammatory bowel disease
• Crohn’s disease
• Small bowel obstruction
• Small bowel stricture
• Small bowel adenocarcinoma

Small bowel tumors and Crohn’s disease are common causes of small bowel obstruction. Early stage neoplasms can easily be mistaken for Crohn’s disease. Therefore, thorough work-ups including imaging studies and endoscopic evaluation with biopsies are critical for accurate diagnosis. Here we report a case of an otherwise healthy female with progressive onset of multiple, recurrent obstructive symptoms secondary to terminal ileal narrowing who was referred for management of steroid-dependent Crohn’s disease. After thorough evaluation, the diagnosis was revised to myeloid granulocytic sarcoma involving the terminal ileum. In this case, a delay in diagnosis can be detrimental for prognosis, as myeloid granulocytic sarcoma is highly predictive of underlying acute myeloid leukemia and needs urgent referral for chemotherapy and/or resection.

• Crohn’s disease
• Myeloid sarcoma
• Ileum
• Steroids

• Adenocarcinoma/etiology
• Antineoplastic Agents/therapeutic use
• Chromosomal Instability
• Colorectal Neoplasms/diagnosis
• Colorectal Neoplasms/etiology
• Colorectal Neoplasms/prevention & control
• CpG Islands/physiology
• DNA Methylation
• Disease Progression
• Humans
• Inflammatory Bowel Diseases/complications
• Inflammatory Bowel Diseases/drug therapy
• Inflammatory Bowel Diseases/genetics
• Intestinal Neoplasms/etiology
• Lymphoma/etiology
• Lymphoma/prevention & control
• Microsatellite Instability
• Risk Factors

Cancer of the small intestine is very uncommon. There are 4 main histological subtypes: adenocarcinomas, carcinoid tumors, lymphoma and sarcoma. The incidence of small intestine cancer has increased over the past several decades with a four-fold increase for carcinoid tumors, less dramatic rises for adenocarcinoma and lymphoma and stable sarcoma rates. Very little is known about its etiology. An increased risk has been noted for individuals with Crohn’s disease, celiac disease, adenoma, familial adenomatous polyposis and Peutz-Jeghers syndrome. Several behavioral risk factors including consumption of red or smoked meat, saturated fat, obesity and smoking have been suggested. The prognosis for carcinomas of the small intestine cancer is poor (5 years relative survival < 30%), better for lymphomas and sarcomas, and best for carcinoid tumors. There has been no significant change in long-term survival rates for any of the 4 histological subtypes. Currently, with the possible exceptions of obesity and cigarette smoking, there are no established modifiable risk factors which might provide the foundation for a prevention program aimed at reducing the incidence and mortality of cancers of the small intestine. More research with better quality and sufficient statistical power is needed to get better understanding of the etiology and biology of this cancer. In addition, more studies should be done to assess not only exposures of interest, but also host susceptibility.

• Cancer of the small intestine
• Histology
• Incidence
• Risk factors
• Survival

• Animals
• Celiac Disease/complications
• Celiac Disease/immunology
• Colorectal Neoplasms/etiology
• Disease Models, Animal
• Humans
• Inflammation/etiology
• Inflammatory Bowel Diseases/complications
• Inflammatory Bowel Diseases/immunology
• Mice

• R01 ES009519 NIEHS NIH HHS
• R01 ES009519-11 NIEHS NIH HHS
• ES09519 NIEHS NIH HHS