Small Intestine Cancer Treatment (PDQ®): Treatment - Health Professional Information [NCI]

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Small Intestine Cancer Treatment

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.

General Information

Note: Estimated new cases and deaths from small intestine cancer in the United States in 2009:[1]

  • New cases: 6,230.
  • 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.[2,3,4,5] 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 2009. Atlanta, Ga: American Cancer Society, 2009. Also available online. Last accessed January 6, 2010.
2. 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.
3. Serour F, Dona G, Birkenfeld S, et al.: Primary neoplasms of the small bowel. J Surg Oncol 49 (1): 29-34, 1992.
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.
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.

Cellular Classification

Tumors that occur in the small intestine include:

  • 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 Adult Soft Tissue Sarcoma Treatment for more information.)

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:

1. Small intestine. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 107-112.

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)

*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

  • Tis, N0, M0

Stage I

  • T1, N0, M0
  • T2, N0, M0

Stage II

  • T3, N0, M0
  • T4, N0, M0

Stage III

  • Any T, N1, M0

Stage IV

  • Any T, any N, M1

References:

1. Small intestine. In: American Joint Committee on Cancer.: AJCC Cancer Staging Manual. 6th ed. New York, NY: Springer, 2002, pp 107-112.

Small Intestine Adenocarcinoma

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 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:

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

Small Intestine Leiomyosarcoma

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 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.

Recurrent Small Intestine Cancer

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 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.

Get More Information From NCI

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Changes to This Summary (07 / 01 / 2009)

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.

GENERAL INFORMATION

Updated statistics with estimated new cases and deaths for 2009 (cited American Cancer Society as reference 1).

More Information

ABOUT PDQ

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    Full description of the NCI PDQ database.

ADDITIONAL PDQ SUMMARIES

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    Treatment options for adult cancers.
  • PDQ® Cancer Information Summaries: Pediatric Treatment
    Treatment options for childhood cancers.
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    Side effects of cancer treatment, management of cancer-related complications and pain, and psychosocial concerns.
  • PDQ® Cancer Information Summaries: Screening/Detection (Testing for Cancer)
    Tests or procedures that detect specific types of cancer.
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    Risk factors and methods to increase chances of preventing specific types of cancer.
  • PDQ® Cancer Information Summaries: Genetics
    Genetics of specific cancers and inherited cancer syndromes, and ethical, legal, and social concerns.
  • PDQ® Cancer Information Summaries: Complementary and Alternative Medicine
    Information about complementary and alternative forms of treatment for patients with cancer.

IMPORTANT:

This information is intended mainly for use by doctors and other health care professionals. If you have questions about this topic, you can ask your doctor, or call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

Date Last Modified: 2009-07-01

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