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PDQ Cancer Information Summaries [Internet]. Bethesda (MD): National Cancer Institute (US); 2002-.
PDQ Cancer Information Summaries [Internet].
Show detailsThis 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.
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).
General Information About Small Intestine Cancer
Incidence and Mortality
Estimated new cases and deaths from small intestine cancer in the United States in 2020:[1]
- New cases: 11,110.
- Deaths: 1,700.
Adenocarcinoma, lymphoma, sarcoma, and carcinoid tumors account for the majority of small intestine malignancies, which, as a whole, account for only 4% of all gastrointestinal malignancies.[2-5]
Follow-up and Survivorship
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.
Carcinoid tumors of the small intestine are covered elsewhere as a separate cancer entity. (Refer to the PDQ summary on Gastrointestinal Carcinoid Tumor Treatment [Adult] for more information.)
References
- American Cancer Society: Cancer Facts and Figures 2020. Atlanta, Ga: American Cancer Society, 2020. Available online. Last accessed January 12, 2021.
- Chamberlain R, Ghalyaie N, Patil S: Small bowel cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA, et al., eds.: DeVita, Hellman, and Rosenberg’s Cancer : Principles & Practice of Oncology. 11th ed. Philadelphia, Pa: Wolters Kluwer, 2019, pp 884-94.
- Serour F, Dona G, Birkenfeld S, et al.: Primary neoplasms of the small bowel. J Surg Oncol 49 (1): 29-34, 1992. [PubMed: 1548877]
- 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: 8131869]
- 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: 8921448]
Cellular Classification of Small Intestine Cancer
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 tumors. (Refer to the PDQ summary on Gastrointestinal Carcinoid Tumor Treatment [Adult] for more information.)
- Gastrointestinal stromal tumors. (Refer to the PDQ summary on Gastrointestinal Stromal Tumors Treatment [Adult] 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
- Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 221–34.
Stage Information for Small Intestine Cancer
The treatment sections of this summary are organized according to histopathologic type rather than stage.
AJCC Stage Groupings and TNM Definitions
The American Joint Committee on Cancer (AJCC) has designated staging by TNM (tumor, node, metastasis) classification to define small intestine cancer. This staging classification applies only to adenocarcinomas arising in the nonampullary duodenum and small intestine. Nonadenocarcinomas arising in the small intestine should have a TNM assigned but are not assigned a stage classification.[1]
Table 1. Definitions of Primary Tumor (T)a
| T Category | T Criteria |
|---|---|
| TX | Primary tumor cannot be assessed. |
| T0 | No evidence of primary tumor. |
| Tis | High-grade dysplasia/carcinoma in situ. |
| T1 | Tumor invades the lamina propria or submucosa. |
| −T1a | Tumor invades the lamina propria. |
| −T1b | Tumor invades the submucosa. |
| T2 | Tumor invades the muscularis propria. |
| T3 | Tumor invades through the muscularis propria into the subserosa, or extends into nonperitonealized perimuscular tissue (mesentery or retroperitoneum) without serosal penetration.b |
| T4 | Tumor perforates the visceral peritoneum or directly invades other organs or structures (e.g., other loops of small intestine, mesentery of adjacent loops of bowel, and abdominal wall by way of serosa; for duodenum only, invasion of pancreas or bile duct). |
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
bFor T3 tumors, 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 interface with the pancreas.
Table 2. Definitions of Regional Lymph Node (N)a
| N Category | N Criteria |
|---|---|
| NX | Regional lymph nodes cannot be assessed. |
| N0 | No regional lymph node metastasis. |
| N1 | Metastasis in one or two regional lymph nodes. |
| N2 | Metastasis in three or more regional lymph nodes. |
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
Table 3. Definitions of Distant Metastasis (M)a
| M Category | M Criteria |
|---|---|
| M0 | No distant metastasis. |
| M1 | Distant metastasis present. |
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
Table 4. Prognostic Stage Groups for Adenocarcinomaa
| Stage | T | N | M |
|---|---|---|---|
| 0 | Tis | N0 | M0 |
| I | T1−2 | 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 |
T = primary tumor; N = regional lymph node; M = distant metastasis.
aReprinted with permission from AJCC: Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 221–34.
References
- Small Intestine. In: Amin MB, Edge SB, Greene FL, et al., eds.: AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp. 221–34.
Small Intestine Adenocarcinoma Treatment
Standard treatment options:
- 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
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
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: 8696551]
- North JH, Pack MS: Malignant tumors of the small intestine: a review of 144 cases. Am Surg 66 (1): 46-51, 2000. [PubMed: 10651347]
Small Intestine Leiomyosarcoma Treatment
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
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Recurrent Small Intestine Cancer Treatment
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 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
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Changes to This Summary (01/29/2020)
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 About Small Intestine Cancer
Updated statistics with estimated new cases and deaths for 2020 (cited American Cancer Society as reference 1).
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
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 reviewer for Small Intestine Cancer Treatment is:
- Valerie Lee, MD (Johns Hopkins University)
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. 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.
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”
The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Small Intestine Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/small-intestine/hp/small-intestine-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389423]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.
Disclaimer
Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.
- General Information About Small Intestine Cancer
- Cellular Classification of Small Intestine Cancer
- Stage Information for Small Intestine Cancer
- Small Intestine Adenocarcinoma Treatment
- Small Intestine Leiomyosarcoma Treatment
- Recurrent Small Intestine Cancer Treatment
- Changes to This Summary (01/29/2020)
- About This PDQ Summary
- PubMedLinks to PubMed
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