Carcinoma of Unknown Primary Treatment (PDQ®)

Description

What is carcinoma of unknown primary?

Carcinoma of unknown primary (CUP) is a disease in which cancer (malignant) cells are found somewhere in the body, but the place where they first started growing (the origin or primary site) cannot be found. This occurs in about 2%-4% of cancer patients.

Actually, CUP can be described as a group of different types of cancer all of which have become known by the place or places in the body where the cancer has spread (metastasized) from another part of the body. Because all of these diseases are not alike, chance of recovery (prognosis) and choice of treatment may be different for each patient.

If CUP is suspected, a doctor will order several tests, one of which may be a biopsy. This means a small piece of tissue is cut from the tumor and looked at under a microscope. The doctor may also do a complete history and physical examination, and order chest x-rays along with blood, urine, and stool tests. A cancer can be called CUP when the doctor cannot tell from the test results where the cancer began.

The pattern of how CUP has spread may also give the doctor information to help determine where it started. For example, lung metastases are more common when cancer begins above the diaphragm (the thin muscle under the lungs that helps the breathing process). Most large studies have shown that CUP often starts in the lungs or pancreas. Less often, it may start in the colon, rectum, breast, or prostate.

An important part of trying to find out where the cancer started is to see how the cancer cells look under a microscope (histology). Other special tests may also be done that help the doctor find out where the cancer started and choose the best type of treatment.

Stage Explanation

Stages of carcinoma of unknown primary

When cancer is diagnosed, more tests are usually done to find out if cancer cells have spread to other parts of the body. This is called staging. But, when CUP is diagnosed, the number and type of tests done may be different for each patient. The treatment options in this summary are based on whether the cancer has just been found (newly diagnosed) or the cancer has come back after it has been treated (recurrent).

The treatment options are also based on where the cancer is found, where the doctor thinks the cancer started, what the cancer cells look like under a microscope, and other factors. A doctor may find that the cancer fits into one of the following groups:

Treatment Option Overview

How carcinoma of unknown primary is treated

Different types of treatment are available for patients with carcinoma of unknown primary (CUP). Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Some of the standard treatments that are used are:

Surgery is a common treatment for CUP. A doctor may remove the cancer and some of the healthy tissue around it. Different operations are used depending on where the cancer is found. If the cancer has spread to lymph nodes, the lymph nodes may be removed (lymph node dissection). If the nodes involved are in the groin, this operation is called a superficial groin dissection. If the cancer has spread to lymph nodes and also to some surrounding areas, the doctor may have to remove a larger portion of tissue around the nodes. When muscles, nerves, and other tissue in the neck are removed, this is called a radical neck dissection.

Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation may be used alone or before or after surgery.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by mouth or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body. Chemotherapy may be used alone or after surgery. Therapy given after an operation when there are no cancer cells that can be seen is called adjuvant therapy.

Hormone therapy is used to stop the hormones in the body that help cancer cells grow. This may be done by using drugs that change the way hormones work or by surgery that takes out organs that make hormones, such as the testicles (orchiectomy).

Treatment in a clinical trial

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. In the following lists of treatments for the different stages, a link to search results for current clinical trials is included for each section. These have been retrieved from NCI's clinical trials database. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Newly Diagnosed Carcinoma of Unknown Primary

If the cancer is in the neck area (cervical lymph nodes), treatment may be one of the following:

  1. Surgery to remove the tonsils (tonsillectomy).
  2. Radiation therapy.
  3. Radiation therapy followed by surgery.
  4. Neck surgery (radical neck dissection).
  5. Neck surgery followed by radiation therapy.

(Refer to the PDQ summary on Metastatic Squamous Neck Cancer With Occult Primary for more information.)

If the cancer is a poorly differentiated carcinoma (the cancer cells look very different than normal cells), the treatment will probably be chemotherapy. Surgery or radiation therapy has also been used for patients with neuroendocrine (nervous system and hormonal system) cancer.

If the cancer is peritoneal adenocarcinomatosis (the tumor is in the lining inside the abdomen), the treatment will probably be chemotherapy.

If the cancer is an isolated axillary nodal metastasis, it is likely that the cancer started in the lung or breast. If female, a mammogram (an x-ray picture of the breast) will be used to check for breast cancer. After tests to check for lung and breast cancer, the treatment may be one of the following:

  1. Surgery to remove the lymph nodes with or without surgery to remove the breast (mastectomy) or radiation therapy to the breast.
  2. Treatment as described above plus chemotherapy that is used for breast cancer.

If the cancer is in the inguinal nodes, the treatment may be one of the following:

  1. Surgery to remove the cancer.
  2. Groin surgery (superficial groin dissection).
  3. Surgery to remove some of the tumor (biopsy) with or without radiation therapy, surgery to remove the lymph nodes, or chemotherapy.

If the cancer is melanoma that has spread to a single nodal site, the treatment will probably be surgery to remove the lymph nodes.

If there is cancer in several different areas of the body and the doctor thinks that the origin of the cancer is one for which there is standard systemic therapy, then that therapy should be given. The following are examples:

  1. Hormone therapy for prostate cancer.
  2. Chemotherapy or hormone therapy for breast cancer.
  3. Chemotherapy for ovarian cancer.

If the source of the cancer cannot be found, then the best treatment may not be known. Patients may want to consider taking part in a clinical trial.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with newly diagnosed carcinoma of unknown primary.

Recurrent Carcinoma of Unknown Primary

Treatment of recurrent CUP depends on the type of cancer, what treatment was received before, the part of the body where the cancer has come back, and other factors. A patient may want to consider taking part in a clinical trial.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent carcinoma of unknown primary.

To Learn More About Carcinoma of Unknown Primary

For more information from the National Cancer Institute about carcinoma of unknown primary, see the following:

For general cancer information and other resources from the National Cancer Institute, see the following:

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp® online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

For more information from the NCI, please write to this address:

Search the NCI Web site

The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Changes to This Summary (06/13/2008)

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.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Date last modified 2008-06-13