患者さん向け Langerhans Cell Histiocytosis Treatment (PDQ®)

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This PDQ cancer information summary has current information about the treatment of childhood and adult Langerhans cell histiocytosis. 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.

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General Information About Langerhans Cell Histiocytosis (LCH)

Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body.

Langerhans cell histiocytosis (LCH) is a rare cancer that begins in LCH cells. LCH cells are a type of dendritic cell which fights infection. Sometimes there are mutations (changes) in LCH cells as they form. These include mutations of the BRAF, MAP2K1, RAS and ARAF genes. These changes may make the LCH cells grow and multiply quickly. This causes LCH cells to build up in certain parts of the body, where they can damage tissue or form lesions.

LCH is not a disease of the Langerhans cells that normally occur in the skin.

LCH may occur at any age, but is most common in young children. Treatment of LCH in children is different from treatment of LCH in adults. The treatment of LCH in children and the treatment of LCH in adults is described in separate sections of this summary.

Family history of cancer or having a parent who was exposed to certain chemicals may increase the risk of LCH.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk.

Risk factors for LCH include the following:

The signs and symptoms of LCH depend on where it is in the body.

These and other signs and symptoms may be caused by LCH or by other conditions. Check with your doctor if you or your child have any of the following:

Skin and nails

LCH in infants may affect the skin only. In some cases, skin-only LCH may get worse over weeks or months and become a form called high-risk multisystem LCH.

In infants, signs or symptoms of LCH that affects the skin may include:

In children and adults, signs or symptoms of LCH that affects the skin and nails may include:

Mouth

Signs or symptoms of LCH that affects the mouth may include:

Bone

Signs or symptoms of LCH that affects the bone may include:

Children with LCH lesions in bones around the ears or eyes have a high risk for diabetes insipidus and other central nervous system diseases.

Lymph nodes and thymus

Signs or symptoms of LCH that affects the lymph nodes or thymus may include:

Endocrine system

Signs or symptoms of LCH that affects the pituitary gland may include:

Signs or symptoms of LCH that affects the thyroid may include:

Eye

Signs or symptoms of LCH that affects the eye may include:

Central nervous system (CNS)

Signs or symptoms of LCH that affects the CNS (brain and spinal cord) may include:

These signs and symptoms may be caused by lesions in the CNS or by CNS neurodegenerative syndrome.

Liver and spleen

Signs or symptoms of LCH that affects the liver or spleen may include:

Lung

Signs or symptoms of LCH that affects the lung may include:

Bone marrow

Signs or symptoms of LCH that affects the bone marrow may include:

Tests that examine the organs and body systems where LCH may occur are used to diagnose LCH.

The following tests and procedures may be used to detect (find) and diagnose LCH or conditions caused by LCH:

Certain factors affect prognosis (chance of recovery) and treatment options.

LCH in organs such as the skin, bones, lymph nodes, or pituitary gland usually gets better with treatment and is called "low-risk". LCH in the spleen, liver, or bone marrow is harder to treat and is called "high-risk".

The prognosis and treatment options depend on the following:

In infants up to one year of age, LCH may go away without treatment.

Stages of LCH

There is no staging system for Langerhans cell histiocytosis (LCH).

The extent or spread of cancer is usually described as stages. There is no staging system for LCH.

Treatment of LCH is based on where LCH cells are found in the body and whether the LCH is low risk or high risk.

LCH is described as single-system disease or multisystem disease, depending on how many body systems are affected:

LCH may affect low-risk organs or high-risk organs:

Recurrent LCH

Recurrent LCH is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body. It often recurs in the bone, ears, skin, or pituitary gland. LCH often recurs the year after stopping treatment. When LCH recurs, it may also be called reactivation.

Treatment Option Overview for LCH

There are different types of treatment for patients with Langerhans cell histiocytosis (LCH).

Different types of treatments are available for patients with LCH. 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. Whenever possible, patients should take part in a clinical trial in order to receive new types of treatment for LCH. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI website. Choosing the most appropriate treatment is a decision that ideally involves the patient, family, and health care team.

Children with LCH should have their treatment planned by a team of health care providers 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 healthcare providers who are experts in treating children with LCH and who specialize in certain areas of medicine. These may include the following specialists:

Nine types of standard treatment are used:

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). When chemotherapy is placed directly onto the skin or into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).

Chemotherapy may be given by injection or by mouth or applied to the skin to treat LCH.

Surgery

Surgery may be used to remove LCH lesions and a small amount of nearby healthy tissue. Curettage is a type of surgery that uses a curette (a sharp, spoon-shaped tool) to scrape LCH cells from bone.

When there is severe liver or lung damage, the entire organ may be removed and replaced with a healthy liver or lung from a donor.

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 cancer. Ultraviolet B (UVB) radiation therapy may be given using a special lamp that directs radiation toward LCH skin lesions.

Photodynamic therapy

Photodynamic therapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For LCH, laser light is aimed at the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients who have photodynamic therapy should not spend too much time in the sun.

In one type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet A radiation is directed to the skin.

Immunotherapy

Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or biologic therapy. There are different types of immunotherapy:

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to attack cancer cells. Targeted therapies may cause less harm to normal cells than chemotherapy or radiation therapy do. There are different types of targeted therapy:

Other drug therapy

Other drugs used to treat LCH include the following:

Stem cell transplant

Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the LCH treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

Observation

Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for Langerhans cell histiocytosis may cause side effects.

For information about side effects that begin during treatment for cancer, see our Side Effects page.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

Many patients with multisystem LCH have late effects caused by treatment or by the disease itself. These patients often have long-term health problems that affect their quality of life.

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 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.

When treatment of LCH stops, new lesions may appear or old lesions may come back.

Many patients with LCH get better with treatment. However, when treatment stops, new lesions may appear or old lesions may come back. This is called reactivation (recurrence) and may occur within one year after stopping treatment. Patients with multisystem disease are more likely to have a reactivation. Common sites of reactivation are bone, ears, or skin. Diabetes insipidus also may develop. Less common sites of reactivation include lymph nodes, bone marrow, spleen, liver, or lung. Some patients may have more than one reactivation over a number of years.

Follow-up tests may be needed.

Because of the risk of reactivation, LCH patients should be monitored for many years. Some of the tests that were done to diagnose LCH may be repeated. This is to see how well the treatment is working and if there are any new lesions. These tests may include:

Other tests that may be needed include:

The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Treatment of Low-Risk LCH in Children

For information about the treatments listed below, see the Treatment Option Overview section.

Skin Lesions

Treatment of newly diagnosed childhood Langerhans cell histiocytosis (LCH) skin lesions may include:

When severe rashes, pain, ulceration, or bleeding occur, treatment may include the following:

Lesions in Bones or Other Low-Risk Organs

Treatment of newly diagnosed childhood LCH bone lesions in the front, sides, or back of the skull, or in any other single bone may include:

Treatment of newly diagnosed childhood LCH lesions in bones around the ears or eyes is done to lower the risk of diabetes insipidus and other long-term problems. Treatment may include:

Treatment of newly diagnosed childhood LCH lesions of the spine or thigh bone may include:

Treatment of two or more bone lesions may include:

Treatment of two or more bone lesions combined with skin lesions, lymph node lesions, or diabetes insipidus may include:

CNS Lesions

Treatment of newly diagnosed childhood LCH central nervous system (CNS) lesions may include:

Treatment of newly diagnosed LCH CNS neurodegenerative syndrome may include:

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 High-Risk LCH in Children

For information about the treatments listed below, see the Treatment Option Overview section.

Treatment of newly diagnosed childhood LCH multisystem disease lesions in the spleen, liver, or bone marrow and another organ or site may include:

Treatment of Recurrent, Refractory, and Progressive Childhood LCH in Children

For information about the treatments listed below, see the Treatment Option Overview section.

Recurrent LCH is cancer that cannot be detected for some time after treatment and then comes back. Refractory LCH is cancer that does not get better with treatment. Progressive LCH is cancer that continues to grow during treatment.

Treatment of recurrent, refractory, or progressive low-risk LCH may include:

Treatment of recurrent, refractory, or progressive high-risk multisystem LCH may include:

Treatments being studied for recurrent, refractory, or progressive childhood LCH include the following:

Treatment of LCH in Adults

For information about the treatments listed below, see the Treatment Option Overview section

Langerhans cell histiocytosis (LCH) in adults is a lot like LCH in children and can form in the same organs and systems as it does in children. These include the endocrine and central nervous systems, liver, spleen, bone marrow, and gastrointestinal tract. In adults, LCH is most commonly found in the lung as single-system disease. LCH in the lung occurs more often in young adults who smoke. Adult LCH is also commonly found in bone or skin.

As in children, the signs and symptoms of LCH depend on where it is found in the body. See the General Information section for the signs and symptoms of LCH.

Tests that examine the organs and body systems where LCH may occur are used to detect (find) and diagnose LCH. See the General Information section for tests and procedures used to diagnose LCH.

In adults, there is not a lot of information about what treatment works best. Sometimes, information comes only from reports of the diagnosis, treatment, and follow-up of one adult or a small group of adults who were given the same type of treatment.

Treatment of LCH of the Lung in Adults

Treatment for LCH of the lung in adults may include:

Sometimes LCH of the lung will go away or not get worse even if it's not treated.

Treatment of LCH of the Bone in Adults

Treatment for LCH that affects only the bone in adults may include:

Treatment of LCH of the Skin in Adults

Treatment for LCH that affects only the skin in adults may include:

Treatment for LCH that affects the skin and other body systems in adults may include:

Treatment of Single-System and Multisystem LCH in Adults

Treatment of single-system and multisystem disease in adults that does not affect the lung, bone, or skin may include:

For more information about LCH trials for adults, see the Histiocyte Society website.

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 Langerhans Cell Histiocytosis

For more information from the National Cancer Institute about Langerhans cell histiocytosis treatment, see the following:

For more childhood cancer information and other general cancer resources, see the following:

About This PDQ Summary

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 and adult Langerhans cell histiocytosis. 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).

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The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Langerhans Cell Histiocytosis Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/langerhans/patient/langerhans-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389196]

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