Malignant Neoplasms of the Small Intestine Clinical Presentation

  • Author: N Joseph Espat, MD, MS, FACS; Chief Editor: Jules E Harris, MD   more...
 
Updated: Dec 2, 2011
 

History

Small-bowel cancer is typically asymptomatic in its early stages, but more than 90% of patients eventually develop symptoms as the disease progresses. This unfortunately reflects advanced disease.

  • Because of the nonspecific nature of symptoms, a significant delay between the onset of symptoms and diagnosis often occurs, averaging 6-8 months.
  • Nausea, vomiting, and intestinal obstruction are common presenting symptoms. Half of these patients undergo emergency surgery for intestinal obstruction.
  • Abdominal pain and weight loss complicate the clinical presentation.
  • Bleeding is less common.
  • The few published series on small bowel neoplasms that are available cannot be used as generalizations for presentation of the individual histologic subtypes. However, it does appear that adenocarcinomas are more frequently associated with pain and obstruction when compared to sarcomas and carcinoids. Sarcomas (GIST) present more commonly with acute GI bleeding.
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Physical

Patients with small-bowel malignancies may present with fairly unremarkable physical examination findings.

  • A tender and distended abdomen may be found due to obstruction.
  • Peritoneal signs indicate perforation.
  • Jaundice from biliary obstruction or liver metastases may occur rarely.
  • Guaiac-positive stool or acute GI bleeding, suggests intestinal bleeding, although this occurs more frequently in persons with benign small-bowel tumors.
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Causes

  • Genetic risk factors
    • Familial adenomatous polyposis: Patients with this condition develop multiple adenomas throughout the small bowel and colon that may lead to adenocarcinomas. After the colon, the duodenum is the most common site of adenocarcinoma. A 1993 study from Johns Hopkins by Offerhaus et al found that patients with familial adenomatous polyposis have a relative risk of more than 300 for duodenal adenocarcinoma but no elevated risk for gastric or nonduodenal small-bowel cancer.[9] Molecular genetic studies of duodenal polyps in patients with familial adenomatous polyposis performed by Kashiwagi et al in 1997 found a high frequency of p53 overexpression in dysplastic adenomas, although the frequency of TP53 and k-ras gene mutations was much lower.[10]
    • Hereditary nonpolyposis colorectal cancer: Aside from colorectal carcinoma, patients with this genetic syndrome also develop endometrial, gastric, small bowel, upper urinary tract, and ovarian carcinomas. The lifetime risk of small-bowel adenocarcinoma in patients with hereditary nonpolyposis colorectal cancer is 1-4%, which is more than 100 times the risk in the general population. Small bowel adenocarcinomas in persons with hereditary nonpolyposis colorectal cancer are distributed fairly evenly throughout the small bowel. They occur at younger age and appear to have a better prognosis than sporadic small-bowel cancers. The most commonly mutated genes in the germline of patients with hereditary nonpolyposis colorectal cancer are HMLH1 and HMSH2, which are involved in DNA mismatch repair.
  • Environmental risk factors
    • Diet: A 1977 study by Lowenfels and Sonni found animal fat intake to be correlated with small-bowel cancer.[11] Another study, in 1993 by Chow et al, reported that consumption of red meat and salt-cured or smoked foods raised the risk of small-bowel cancer 2-3 times.[12]
    • Tobacco and alcohol: Studies from 1994 by Chen et al found an association between smoking and small-bowel adenocarcinoma and between alcohol consumption and small-bowel adenocarcinoma, but this has not been confirmed in other studies.[13]
  • Predisposing medical conditions
    • Crohn disease: The relative risk of small-bowel adenocarcinoma is estimated to be between 15 and more than 100 in patients with Crohn disease. Unlike most small-bowel adenocarcinomas, Crohn-related tumors generally occur in the ileum, reflecting the distribution of Crohn disease. The risk of adenocarcinoma does not begin until at least 10 years after the onset of Crohn disease, and the adenocarcinoma typically occurs more than 20 years afterwards.
    • Celiac disease (nontropical sprue): Patients with celiac disease appear to be at increased risk of small-bowel lymphoma and adenocarcinoma. A 2001 survey of adult celiac disease patients in the United States performed by Green et al found a relative risk of 300 for the development of lymphoma and 67 for the development of adenocarcinoma. Small-bowel adenocarcinomas associated with celiac disease appear to have an increased incidence of defective DNA mismatch repair compared with those not associated with celiac disease and are also associated with an earlier stage at diagnosis and a better prognosis.[14]
    • Peutz-Jeghers syndrome: Hemminki has reported an approximately 18-fold increase in the incidence compared to that in the general population.[15]
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Contributor Information and Disclosures
Author

N Joseph Espat, MD, MS, FACS  Harold J Wanebo Professor and Chief of Surgical Oncology, Director, Adele R Decof Cancer Center, Vice-Chair of Department of Surgery, Roger Williams Medical Center, Boston University School of Medicine

N Joseph Espat, MD, MS, FACS is a member of the following medical societies: Alpha Omega Alpha, American Association for Cancer Research, American College of Surgeons, American Hepato-Pancreato-Biliary Association, American Medical Association, American Society for Parenteral and Enteral Nutrition, American Society of Clinical Oncology, Association for Academic Surgery, Central Surgical Association, Chicago Medical Society, International Hepato-Pancreato-Biliary Association, Pancreas Club, Sigma Xi, Society for Leukocyte Biology, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Society of Surgical Oncology, Society of University Surgeons, Southeastern Surgical Congress, Southern Medical Association, and Surgical Infection Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ponnandai S Somasundar, MD, FACS  Assistant Chief of Surgical Oncology, Assistant Professor of Surgery, Boston University; Hepatopancreatobiliary/Surgical Oncologist, Roger Williams Medical Center

Ponnandai S Somasundar, MD, FACS is a member of the following medical societies: American College of Surgeons, American Hepato-Pancreato-Biliary Association, Association for Academic Surgery, Association of Surgeons of India, and Society of Surgical Oncology

Disclosure: Nothing to disclose.

Piero Marco Fisichella, MD  Assistant Professor of Surgery, Stritch School of Medicine, Loyola University; Director, Esophageal Motility Center, Loyola University Medical Center

Piero Marco Fisichella, MD is a member of the following medical societies: American College of Surgeons, American Medical Association, Association for Academic Surgery, Society for Surgery of the Alimentary Tract, and Society of American Gastrointestinal and Endoscopic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Lodovico Balducci, MD  Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Benjamin Movsas, MD  Vice-Chairman, Department of Radiation Oncology, Fox Chase Cancer Center

Benjamin Movsas, MD is a member of the following medical societies: American College of Radiology, American Radium Society, and American Society for Therapeutic Radiology and Oncology

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Jules E Harris, MD  Clinical Professor of Medicine, Division of Hematology/Medical Oncology, Department of Internal Medicine, University of Arizona College of Medicine; Consulting Staff, Arizona Cancer Center

Jules E Harris, MD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Immunologists, American Society of Hematology, and Central Society for Clinical Research

Disclosure: GlobeImmune Salary Consulting

Additional Contributors

eMedicine extends its thanks to Alfred I Neugut, MD, PhD , Head, Cancer Prevention and Control, Herbert Irving Comprehensive Cancer Center; Professor, Department of Medicine and Public Health, Columbia University College of Physicians and Surgeons and Allen C Chen, MD, MS, Assistant Professor, Department of Medicine, Division of Medical Oncology, New York University School of Medicine for previous versions of this article.

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