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This PDQ cancer information summary has current information about the treatment of childhood carcinoma of unknown primary. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change. The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
CONTENTS
- General Information About Carcinoma of Unknown Primary
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Carcinoma of unknown primary is a disease in which malignant (cancer) cells are found in the body but the place the cancer began is not known.
Cancer can form in any tissue in the body. The first cancer to form is called the primary cancer. The process of cancer cells spreading to other parts of the body is called metastasis. The cancer that has spread to another part of the body is called metastatic cancer.
In carcinoma of unknown primary (CUP), metastatic cancer is diagnosed, but the primary cancer has not been found.
This type of cancer is also called occult primary tumor.
In carcinoma of unknown primary, cancer cells have spread in the body but the place where the primary cancer began is not known. The signs and symptoms of disease are caused by the metastatic cancer and depend on where the cancer has spread.
The primary cancer does not cause signs and symptoms of disease.
Check with your doctor if you have any of the following general signs of cancer:
Blood and imaging tests are done to learn more about the cause of the signs and symptoms.
Tests and procedures that may be used are described below.
A biopsy is done to diagnose metastatic cancer.
A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist.
One of the following types of biopsies may be used:
The pathologist views the tissue to look for cancer cells and to find out the type of cancer. Cancer cells usually look like the cells in the type of tissue in which the cancer began. In CUP, the cancer cells do not look like the cells of the tissue they were found in. The pathologist cannot determine the type of primary cancer.
One or more of the following laboratory tests may be used to further study the tissue samples:
Because the place where the cancer started is not known, more tests and procedures are done to search for the primary cancer.
The following tests and procedures may be done:
When tests are able to find the primary cancer, the cancer is no longer a CUP and treatment is based on the type of primary cancer.
When it is not known where the cancer first formed at diagnosis, adenocarcinomas, melanomas, and embryonal tumors (such as rhabdomyosarcoma or neuroblastoma) are tumor types that are often diagnosed later in children and adolescents.
Sometimes the primary cancer is never found.
The primary cancer (the cancer that first formed) may not be found for one of the following reasons:
Because the primary cancer is unknown, it may be harder to choose the best treatment.
- Stages of Childhood Carcinoma of Unknown Primary
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The extent or spread of cancer is usually described as stages. The stage of the cancer is usually used to plan treatment. However, carcinoma of unknown primary (CUP) has already spread to other parts of the body when it is found. There is no standard staging system for CUP.
Sometimes childhood carcinoma of unknown primary recurs (comes back) after treatment.
- Treatment Option Overview
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There are different types of treatment for children with carcinoma of unknown primary.
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.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with carcinoma of unknown primary should have their treatment planned by a team of doctors who are experts in treating childhood cancer.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:
Three types of standard treatment are used:
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. External radiation therapy uses a machine outside the body to send radiation toward the area of the body with metastatic cancer.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).
Targeted therapy
Targeted therapy is a treatment that uses drugs or other substances to identify and attack cancer cells. Targeted therapies usually cause less harm to normal cells than chemotherapy and radiation therapy do.
New types of treatment are being tested in clinical trials.
Information about clinical trials is available from the NCI website.
Treatment for childhood carcinoma of unknown primary may cause side effects.
For information about side effects that begin during treatment for cancer, see our Side Effects page.
Patients may want to think about taking part 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.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
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. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
- Treatment of Childhood Carcinoma of Unknown Primary
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Treatment of newly diagnosed carcinoma of unknown primary (CUP) in children depends on the following:
For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of newly diagnosed CUP in children may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- Treatment of Recurrent Childhood Carcinoma of Unknown Primary
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For information about the treatments listed below, see the Treatment Option Overview section.
Treatment of recurrent carcinoma of unknown primary (CUP) in children may include the following:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- To Learn More About Childhood Carcinoma of Unknown Primary
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For more information from the National Cancer Institute about carcinoma of unknown primary, see the following:
For more childhood cancer information and other general cancer resources, see the following:
- About This PDQ Summary
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About PDQ
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood carcinoma of unknown primary. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
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.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
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The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Carcinoma of Unknown Primary Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/unknown-primary/patient/child-unknown-primary-treatment-pdq. Accessed <MM/DD/YYYY>.
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