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:

• Having a parent who was exposed to certain chemicals.
• Having a parent who was exposed to metal, granite, or wood dust in the workplace.
• A family history of cancer, including LCH.
• Having a personal history or family history of thyroid disease.
• Having infections as a newborn.
• Smoking, especially in young adults.
• Being Hispanic.
• Not being vaccinated as a child.

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:

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:

• Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Neurological exam: A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
• Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:

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  The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.

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  The portion of the blood sample made up of red blood cells.

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  The number and type of white blood cells.

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  The number of red blood cells and platelets.

• Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the body by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Liver function test: A blood test to measure the blood levels of certain substances released by the liver. A high or low level of these substances can be a sign of disease in the liver.
• BRAF gene testing: A laboratory test in which a sample of blood or tissue is tested for certain changes in the BRAF gene.
• Urinalysis: A test to check the color of urine and its contents, such as sugar, protein, red blood cells, and white blood cells.
• Water deprivation test: A test to check how much urine is made and whether it becomes concentrated when little or no water is given. This test is used to diagnose diabetes insipidus, which may be caused by LCH.
• Bone marrow aspiration and biopsy: The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone. A pathologist views the bone marrow and bone under a microscope to look for signs of LCH.

  

  Bone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.

  The following tests may be done on the tissue that was removed:

  • Immunohistochemistry: A laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test is used to help diagnose cancer and to help tell one type of cancer from another type of cancer.
  • Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of the cells, such as size, shape, and the presence of tumor (or other) markers on the cell surface. The cells from a sample of a patient’s blood, bone marrow, or other tissue are stained with a fluorescent dye, placed in a fluid, and then passed one at a time through a beam of light. The test results are based on how the cells that were stained with the fluorescent dye react to the beam of light.
• Bone scan: A procedure to check if there are rapidly dividing cells in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.

  

  Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.

• X-ray: An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. Sometimes a skeletal survey is done. This is a procedure to x-ray all the bones in the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

  

  Computed tomography (CT) scan of the abdomen. The patient lies on a table that slides through the CT machine, which takes x-ray pictures of the inside of the body.

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. A substance called gadolinium may be injected into a vein. The gadolinium collects around the LCH cells so that they show up brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  

  Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.

• PET scan (positron emission tomography scan): A procedure to find tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

  

  Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.

• Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.

  

  Abdominal ultrasound. An ultrasound transducer connected to a computer is passed over the surface of the abdomen. The ultrasound transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).

• Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called lung function test.
• Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
• Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas in the gastrointestinal tract or lungs. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for LCH cells. To diagnose LCH, a biopsy of bone, skin, lymph nodes, liver, or other sites of disease may be done.

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:

• How old the patient is when diagnosed with LCH.
• Which organs or body systems are affected by LCH.
• How many organs or body systems the cancer affects.
• Whether the cancer is found in the liver, spleen, bone marrow, or certain bones in the skull.
• How quickly the cancer responds to initial treatment.
• Whether there are certain changes in the BRAF gene.
• Whether the cancer has just been diagnosed or has come back (recurred).

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

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:

• Single-system LCH: LCH is found in one part of an organ or body system or in more than one part of that organ or body system. Bone is the most common single place for LCH to be found.
• Multisystem LCH: LCH occurs in two or more organs or body systems or may be spread throughout the body. Multisystem LCH is less common than single-system LCH.

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

• Low-risk organs include the skin, bone, lungs, lymph nodes, gastrointestinal tract, pituitary gland, thyroid gland, thymus, and central nervous system (CNS).
• High-risk organs include the liver, spleen, and bone marrow.

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.

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:

• Pediatrician.
• Pediatric surgeon.
• Pediatric hematologist.
• Radiation oncologist.
• Neurologist.
• Endocrinologist.
• Pediatric nurse specialist.
• Rehabilitation specialist.
• Psychologist.
• Social worker.

Nine types of standard treatment are used:

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:

• Slow growth and development.
• Hearing loss.
• Bone, tooth, liver, and lung problems.
• Changes in mood, feeling, learning, thinking, or memory.
• Second cancers, such as leukemia, retinoblastoma, Ewing sarcoma, brain or liver cancer.

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:

• Physical exam.
• Neurological exam.
• Ultrasound exam.
• MRI.
• CT scan.
• PET scan.

Other tests that may be needed include:

• Brain stem auditory evoked response (BAER) test: A test that measures the brain's response to clicking sounds or certain tones.
• Pulmonary function test (PFT): A test to see how well the lungs are working. It measures how much air the lungs can hold and how quickly air moves into and out of the lungs. It also measures how much oxygen is used and how much carbon dioxide is given off during breathing. This is also called a lung function test.
• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.

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.

Skin Lesions

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

• Observation.

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

• Steroid therapy.
• Chemotherapy given by mouth or vein.
• Chemotherapy applied to the skin.
• Photodynamic therapy with psoralen and ultraviolet A (PUVA) therapy.
• UVB radiation therapy.

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:

• Surgery (curettage) with or without steroid therapy.
• Low-dose radiation therapy for lesions that affect nearby organs.

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:

• Chemotherapy and steroid therapy.
• Surgery (curettage).

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

• Observation.
• Low-dose radiation therapy.
• Chemotherapy, for lesions that spread from the spine into nearby tissue.
• Surgery to strengthen the weakened bone by bracing or fusing the bones together.

Treatment of two or more bone lesions may include:

• Chemotherapy and steroid therapy.

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

• Chemotherapy with or without steroid therapy.
• Bisphosphonate therapy.

CNS Lesions

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

• Chemotherapy with or without steroid therapy.

Treatment of newly diagnosed LCH CNS neurodegenerative syndrome may include:

• Targeted therapy.
• Chemotherapy.
• Retinoid therapy.
• Immunotherapy (IVIG) with or without chemotherapy.

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 LCH of the Lung in Adults

Treatment for LCH of the lung in adults may include:

• Quitting smoking for all patients who smoke. Lung damage will get worse over time in patients who do not quit smoking. In patients who quit smoking, lung damage may get better or it may get worse over time.
• Chemotherapy.
• Lung transplant for patients with severe lung damage.

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:

• Surgery with or without steroid therapy.
• Chemotherapy with or without low-dose radiation therapy.
• Radiation therapy.
• Bisphosphonate therapy, for severe bone pain.
• Anti-inflammatory drugs with chemotherapy.

Treatment of LCH of the Skin in Adults

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

• Surgery.
• Steroid or other drug therapy applied or injected into the skin.
• Photodynamic therapy with psoralen and ultraviolet A (PUVA) radiation.
• UVB radiation therapy.
• Chemotherapy or immunotherapy given by mouth, such as methotrexate, thalidomide, hydroxyurea, or interferon.
• Retinoid therapy may be used if the skin lesions do not get better with other treatment.

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

• Chemotherapy.

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:

• Chemotherapy.
• Targeted therapy (imatinib, or vemurafenib).

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.

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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.

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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.

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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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