Cancer Types   /   Gastrointestinal Cancers

NCI/PDQ^® Health professionals: Small Intestine Cancer Treatment (PDQ®)

National Cancer Institute
Last Modified: July 20, 2012

TABLE OF CONTENTS

• General Information About Small Intestine Cancer
  • Incidence and Mortality

• Cellular Classification of Small Intestine Cancer

• Stage Information for Small Intestine Cancer
  • Definitions of TNM

• Small Intestine Adenocarcinoma
  • Current Clinical Trials

• Small Intestine Leiomyosarcoma
  • Current Clinical Trials

• Recurrent Small Intestine Cancer
  • Current Clinical Trials

• Changes to This Summary (07/20/2012)

• About This PDQ® Summary
  • Purpose of This Summary
  • Reviewers and Updates
  • Levels of Evidence
  • Permission to Use This Summary
  • Disclaimer
  • Contact Us

• Get More Information From NCI

General Information About Small Intestine Cancer

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Incidence and Mortality

Estimated new cases and deaths from small intestine cancer in the United States in 2012: 1

• New cases: 8,070.
• Deaths: 1,150.

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 3 4 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:

1. American Cancer Society.: Cancer Facts and Figures 2012. Atlanta, Ga: American Cancer Society, 2012. Available online [PUBMED Abstract]
2. Zureikat AH, Heller MT, Zeh HJ III: Cancer of the small intestine. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 1048-59. [PUBMED Abstract]
3. Serour F, Dona G, Birkenfeld S, et al.: Primary neoplasms of the small bowel. J Surg Oncol 49 (1): 29-34, 1992. [PUBMED Abstract]
4. 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]
5. 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]

Cellular Classification of Small Intestine Cancer

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Tumors that occur in the small intestine include the following:

• Adenocarcinoma (majority of cases).
• Lymphoma (uncommon), which is usually of the non-Hodgkin type. (Refer to the PDQ® summary on Adult Non-Hodgkin Lymphoma Treatment for more information.)
• Sarcoma (most commonly leiomyosarcoma and more rarely angiosarcoma or liposarcoma).
• Carcinoid. (Refer to the PDQ® summary on Gastrointestinal Carcinoid Tumor Treatment for more information.)
• Gastrointestinal stromal tumors. (Refer to the PDQ® summary on Gastrointestinal Stromal Tumors Treatment for more information.)

Approximately 25% to 50% of the primary malignant tumors in the small intestine are adenocarcinomas, and most occur in the duodenum. 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:

1. Small intestine. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 127-32. [PUBMED Abstract]

Stage Information for Small Intestine Cancer

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Note: This Stage Information section has been updated to include information from the seventh edition (2010) of the American Joint Committee on Cancer's AJCC Cancer Staging Manual. The PDQ® Adult Treatment Editorial Board, which is responsible for maintaining this summary, is currently reviewing the new staging categories to determine whether additional changes need to be made to other parts of the summary. Any necessary changes will be made as soon as possible.

The treatment sections of this summary are organized according to histopathologic type rather than stage.

Definitions of TNM

The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define small intestine cancer. 1

Table 1. Primary Tumor (T)^a

^aReprinted with permission from AJCC: Small intestine. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 127-32.^bThe nonperitonealized perimuscular tissue is, for jejunum and ileum, part of the mesentery and, for duodenum in areas where serosa is lacking, part of the interface with the pancreas.

TX	Primary tumor cannot be assessed.
T0	No evidence of primary tumor.
Tis	Carcinoma in situ.
T1a	Tumor invades lamina propria.
T1b	Tumor invades submucosa.b
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.b
T4	Tumor perforates the visceral peritoneum or directly invades other organs or structures (includes other loops of small intestine, mesentery, or retroperitoneum >2 cm, and abdominal wall by way of serosa; for duodenum only, invasion of pancreas or bile duct).

Table 2. Regional Lymph Nodes (N)^a

^aReprinted with permission from AJCC: Small intestine. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 127-32.

NX	Regional lymph nodes cannot be assessed.
N0	No regional lymph node metastasis.
N1	Metastasis in 13 regional lymph nodes.
N2	Metastases in 4 regional lymph nodes.

Table 3. Distant Metastasis (M)^a

^aReprinted with permission from AJCC: Small intestine. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 127-32.

M0	No distant metastasis.
M1	Distant metastasis.

Table 4. Anatomic Stage/Prognostic Groups^a

^aReprinted with permission from AJCC: Small intestine. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 127-32.

Stage	T	N	M
0	Tis	N0	M0
I	T1	N0	M0
	T2	N0	M0
IIA	T3	N0	M0
IIB	T4	N0	M0
IIIA	Any T	N1	M0
IIIB	Any T	N2	M0
IV	Any T	Any N	M1

References:

1. Small intestine. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 127-32. [PUBMED Abstract]

Small Intestine Adenocarcinoma

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Standard treatment options:

1. For resectable primary disease:
   • Radical surgical resection. 1 2

2. For unresectable primary disease:
   • Surgical bypass of obstructing lesion.
   • Palliative radiation therapy.

Treatment options under clinical evaluation:

1. 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.

2. 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 list of cancer clinical trials 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:

1. 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]
2. North JH, Pack MS: Malignant tumors of the small intestine: a review of 144 cases. Am Surg 66 (1): 46-51, 2000. [PUBMED Abstract]

Small Intestine Leiomyosarcoma

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Standard treatment options:

1. For resectable primary disease:
   • Radical surgical resection.

2. For unresectable primary disease:
   • Surgical bypass of obstructing lesion and radiation therapy.

3. 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 list of cancer clinical trials 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.

Recurrent Small Intestine Cancer

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Standard treatment options:

1. 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.

2. 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 list of cancer clinical trials 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.

Changes to This Summary (07/20/2012)

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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.

This summary is written and maintained by the PDQ® Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ® Editorial Boards in maintaining the PDQ® summaries can be found on the About This PDQ® Summary and PDQ® NCI's Comprehensive Cancer Database pages.

About This PDQ® Summary

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Purpose of This Summary

This PDQ® cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of small intestine cancer. It 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.

Reviewers and Updates

This summary is reviewed regularly and updated as necessary by the PDQ® Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).

Board members review recently published articles each month to determine whether an article should:

• be discussed at a meeting,
• be cited with text, or
• replace or update an existing article that is already cited.

Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.

The lead reviewers for Small Intestine Cancer Treatment are:

• David P. Ryan, MD (Massachusetts General Hospital)
• Jennifer Wo, MD (Massachusetts General Hospital)

Any comments or questions about the summary content should be submitted to Cancer.gov through the Web site's Contact Form. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.

Levels of Evidence

Some of the reference citations in this 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.

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The preferred citation for this PDQ® summary is:

National Cancer Institute: PDQ® Small Intestine Cancer Treatment. Bethesda, MD: National Cancer Institute. Date last modified . Available at: http://cancer.gov/cancertopics/pdq/treatment/smallintestine/HealthProfessional. Accessed .

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