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Table of Contents Purpose of This PDQ Summary
General Information
Cellular Classification
Stage Information
Small Intestine Adenocarcinoma
Small Intestine Leiomyosarcoma
Recurrent Small Intestine Cancer
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Changes to This Summary (05/22/2008)
More Information
Purpose of This PDQ Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of small intestine cancer. This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board.
Information about the following is included in this summary:
- Epidemiology and prognosis.
- Cellular classification.
- Staging.
- Treatment options for different types of tumors.
This summary is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Some of the reference citations in the summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations. Based on the strength of the available evidence, treatment options are described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for reimbursement determinations.
This summary is available in a patient version, written in less technical language, and in Spanish.
Back to Top General Information
Note: Estimated new cases and deaths from small intestine cancer in the United States in 2008:[1]
- New cases: 6,110.
- Deaths: 1,110.
Adenocarcinoma, lymphoma, sarcoma, and carcinoid tumors
account for the majority of small intestine malignancies which, as a whole,
account for only 1% to 2% of all gastrointestinal malignancies.[2-5] As in
other gastrointestinal malignancies, the predominant modality of treatment is
surgery when resection is possible, and cure relates to the ability to
completely resect the cancer. The overall 5-year survival rate for resectable
adenocarcinoma is only 20%. The 5-year survival rate for resectable
leiomyosarcoma, the most common primary sarcoma of the small intestine, is
approximately 50%. Carcinoid tumors of the small intestine are covered
elsewhere as a separate cancer entity. (Refer to the PDQ summary on Gastrointestinal Carcinoid Tumor Treatment for more information.)
References
-
American Cancer Society.: Cancer Facts and Figures 2008. Atlanta, Ga: American Cancer Society, 2008. Also available online. Last accessed October 1, 2008.
-
Zeh H III: Cancer of the small intestine. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. 7th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2005, pp 1035-48.
-
Serour F, Dona G, Birkenfeld S, et al.: Primary neoplasms of the small bowel. J Surg Oncol 49 (1): 29-34, 1992.
[PUBMED Abstract]
-
Matsuo S, Eto T, Tsunoda T, et al.: Small bowel tumors: an analysis of tumor-like lesions, benign and malignant neoplasms. Eur J Surg Oncol 20 (1): 47-51, 1994.
[PUBMED Abstract]
-
Chow JS, Chen CC, Ahsan H, et al.: A population-based study of the incidence of malignant small bowel tumours: SEER, 1973-1990. Int J Epidemiol 25 (4): 722-8, 1996.
[PUBMED Abstract]
Back to Top Cellular Classification
Tumors that occur in the small intestine include:
The majority of malignant neoplasms of the small intestine are adenocarcinomas (50% or more) and are more commonly located in the duodenum and jejunum than in the
ileum.[1] Small intestine carcinomas may occur synchronously or metachronously at
multiple sites.
Leiomyosarcomas occur most often in the ileum.
Some 20% of malignant lesions of the small intestine are carcinoid tumors,
which occur more frequently in the ileum than in the duodenum or jejunum and
may be multiple.
It is uncommon to find malignant lymphoma as a solitary small intestine lesion.
References
-
Small intestine. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 107-112.
Back to Top Stage Information
The treatment sections of this summary are organized according to
histopathologic type rather than stage.
The American Joint Committee on Cancer (AJCC) has designated staging by TNM
classification.[1]
TNM Definitions
Primary Tumor (T)
- TX: Primary tumor cannot be assessed
- T0: No evidence of primary tumor
- Tis: Carcinoma in situ
- T1: Tumor invades lamina propria or submucosa
- T2: Tumor invades muscularis propria
- T3: Tumor invades through the muscularis propria into the subserosa or into
the nonperitonealized perimuscular tissue (mesentery or
retroperitoneum) with extension 2 cm or less*
- T4: Tumor perforates the visceral peritoneum or directly invades other
organs or structures (includes other loops of the small intestine,
mesentery, or retroperitoneum more than 2 cm, and the abdominal wall by
way of the serosa; for the duodenum only, includes invasion of the pancreas)
* [Note: The nonperitonealized perimuscular tissue is, for the jejunum and
ileum, part of the mesentery and, for the duodenum in areas where serosa is
lacking, part of the retroperitoneum.]
Regional lymph nodes (N)
- NX: Regional lymph nodes cannot be assessed
- N0: No regional lymph node metastasis
- N1: Regional lymph node metastasis
Distant metastasis (M)
- MX: Distant metastasis cannot be assessed
- M0: No distant metastasis
- M1: Distant metastasis
AJCC Stage Groupings
Stage 0
Stage I
Stage II
Stage III
Stage IV
References
-
Small intestine. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 107-112.
Back to Top Small Intestine Adenocarcinoma
Standard treatment options:
- For resectable primary disease:
- Radical surgical resection.[1,2]
- For unresectable primary disease:
- Surgical bypass of obstructing lesion.
- Palliative radiation therapy.
Treatment options under clinical evaluation:
- For unresectable primary disease:
- Clinical trials evaluating methods to improve local control, such as the
use of radiation therapy with radiosensitizers with or without systemic
chemotherapy.
- For unresectable metastatic disease:
- Clinical trials evaluating the value of new anticancer drugs and
biologicals (phase I and phase II studies).
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with small intestine adenocarcinoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
References
-
Rose DM, Hochwald SN, Klimstra DS, et al.: Primary duodenal adenocarcinoma: a ten-year experience with 79 patients. J Am Coll Surg 183 (2): 89-96, 1996.
[PUBMED Abstract]
-
North JH, Pack MS: Malignant tumors of the small intestine: a review of 144 cases. Am Surg 66 (1): 46-51, 2000.
[PUBMED Abstract]
Back to Top Small Intestine Leiomyosarcoma
Standard treatment options:
- For resectable primary disease:
- Radical surgical resection.
- For unresectable primary disease:
- Surgical bypass of obstructing lesion and radiation therapy.
- For unresectable metastatic disease:
- Palliative surgery.
- Palliative radiation therapy.
- Palliative chemotherapy.
Treatment options under clinical evaluation:
- For unresectable primary or metastatic disease:
- Clinical trials evaluating the value of new anticancer drugs and
biologicals.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with small intestine leiomyosarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Back to Top Recurrent Small Intestine Cancer
Standard treatment options:
- For metastatic adenocarcinoma or leiomyosarcoma:
- No standard effective chemotherapy exists for patients with recurrent metastatic
adenocarcinoma or leiomyosarcoma of the small intestine. These types of patients
should be considered candidates for clinical trials evaluating the use of new
anticancer drugs or biologicals in phase I and phase II trials.
- For locally recurrent disease:
- Surgery.
- Palliative radiation therapy.
- Palliative chemotherapy.
- Clinical trials evaluating ways of improving local control, such as the use
of radiation therapy with radiosensitizers with or without systemic
chemotherapy.
Current Clinical Trials
Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent small intestine cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
Back to Top Get More Information From NCI
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Back to Top Changes to This Summary (05/22/2008)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Editorial changes were made to this summary.
Back to Top More Information
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