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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).

Small intestine cancer


Table of Contents

GENERAL INFORMATION
CELLULAR CLASSIFICATION
STAGE INFORMATION
TREATMENT OPTION OVERVIEW
SMALL INTESTINE ADENOCARCINOMA
SMALL INTESTINE LYMPHOMA
SMALL INTESTINE LEIOMYOSARCOMA
RECURRENT SMALL INTESTINE CANCER

GENERAL INFORMATION

Depending on the histology, cancer of the small intestine is treatable and sometimes curable. Adenocarcinoma, lymphoma, sarcoma, and carcinoid tumors account for the majority of small intestine malignancies which, as a whole, account for only 1%-2% of all gastrointestinal malignancies.[1-4] 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; for information see the PDQ summary on gastrointestinal carcinoid tumor. Lymphoma of the small intestine is dealt with briefly here; for more detailed information, a separate summary containing information on non-Hodgkin's lymphoma is also available in PDQ.

References:

  1. Coit DG: Cancer of the small intestine. In: DeVita VT Jr, Hellman S, Rosenberg SA, eds.: Cancer: Principles and Practice of Oncology. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 1128-1143.

  2. Serour F, Dona G, Birkenfeld S, et al.: Primary neoplasms of the small bowel. Journal of Surgical Oncology 49(1): 29-34, 1992.

  3. Matsuo S, Eto T, Tsunoda T, et al.: Small bowel tumors: an analysis of tumor-like lesions, benign and malignant neoplasms. European Journal of Surgical Oncology 20(1): 47-51, 1994.

  4. Chow JS, Chen CC, Ahsan H, et al.: A population-based study of the incidence of malignant small bowel tumours: SEER, 1973-1990. International Journal of Epidemiology 25(4): 722-728, 1996.


CELLULAR CLASSIFICATION

Small intestine:[1]

Malignant neoplasms of the small intestine are mainly (> or = 50%) adenocarcinomas and are more common in the duodenum and jejunum than in the ileum. 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. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 77-81.


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 --

  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. Philadelphia, Pa: Lippincott-Raven Publishers, 5th ed., 1997, pp 77-81.


TREATMENT OPTION OVERVIEW

The designations in PDQ that treatments are "standard" or "under clinical evaluation" are not to be used as a basis for reimbursement determinations.


SMALL INTESTINE ADENOCARCINOMA

Treatment options:

Standard:

1. For resectable primary disease:
  • radical surgical resection[1]

2. For unresectable primary disease:
  • surgical bypass of obstructing lesion
  • palliative radiation therapy

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 II studies)

References:

  1. Rose DM, Hochwald SN, Klimstra DS, et al.: Primary duodenal adenocarcinoma: a ten-year experience with 79 patients. Journal of the American College of Surgeons 183(2): 89-96, 1996.


SMALL INTESTINE LYMPHOMA

Treatment options:

Standard:

1. For disease localized to the bowel wall (stage IE):
  • surgical resection alone may suffice if 12 or more lymph nodes are removed and prove negative, but the addition of combination chemotherapy should be considered

2. For extension of disease to the regional lymph nodes:
  • surgical resection at the time of diagnosis. Combination chemotherapy is then the treatment of choice

3. For unresectable and extensive disease:
  • combination chemotherapy is the treatment of choice radiation therapy is often used to reduce the risk of recurrence in the tumor bed


SMALL INTESTINE LEIOMYOSARCOMA

Treatment options:

Standard:

1. For resectable primary disease:
  • radical surgical resection

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

3. For unresectable metastatic disease:
  • palliative surgery
  • palliative radiation therapy
  • palliative chemotherapy

Under clinical evaluation:
For unresectable primary or metastatic disease:
  • clinical trials evaluating the value of new anticancer drugs and biologicals


RECURRENT SMALL INTESTINE CANCER

Treatment options:

1. For metastatic adenocarcinoma or leiomyosarcoma:
  • there is no standard effective chemotherapy for recurrent metastatic adenocarcinoma or leiomyosarcoma of the small intestine. All such patients should be considered candidates for clinical trials evaluating the use of new anticancer drugs or biologicals in phase I and II trials

2. For lymphoma:
  • see PDQ treatment summary for management of lymphoma

3. 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

Date Last Modified: 02/1999



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