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What is lymphoma of the skin?
Lymphoma is a cancer that starts in cells called lymphocytes, which are part of the body’s immune system. Lymphocytes are in the lymph nodes and other lymphoid tissues (such as the spleen, bone marrow, and some other organs, including the skin).There are 2 main types of lymphomas.
Hodgkin lymphoma (also known as Hodgkin’s lymphoma, Hodgkin disease, or Hodgkin’s disease)
Non-Hodgkin lymphoma (also known as non-Hodgkin’s lymphoma, NHL, or sometimes just lymphoma), which contains all other lymphomas, including all skin lymphomas
When a non-Hodgkin lymphoma starts only in the skin (not other organs or tissues) it is called a skin lymphoma (orcutaneous lymphoma). A lymphoma that starts in lymph nodes or another part of the body and then spreads to the skin is not considered a skin lymphoma (because it didn’t start there).
Hodgkin and non-Hodgkin lymphomas differ in how they behave, spread, and respond to treatment. Doctors can usually tell the difference between them by looking at the cancer cells under a microscope. In some cases, sensitive lab tests may be needed to tell them apart.
Hodgkin disease and other types of non-Hodgkin lymphoma are discussed in separate American Cancer Society documents. The rest of this document focuses only on lymphoma of the skin.
The lymph system and lymphoid tissue
To understand what lymphoma is, it helps to know about the body’s lymph system.
The lymph system (also known as the lymphatic system) is made up of lymphoid tissue, lymph vessels, and a clear fluid called lymph. Lymphoid tissue is found throughout the body and contains several types of immune system cells that work together to help the body fight infections.
Lymphocytes: Most of the cells in lymphoid tissue are lymphocytes, a type of white blood cell. The 2 main types of lymphocytes are B lymphocytes (B cells) and T lymphocytes (T cells).
B lymphocytes: B cells normally help protect the body against germs (bacteria or viruses) by making proteins called antibodies. The antibodies attach to the germs and attract other immune system cells that then surround and digest the antibody-coated germs. Antibodies also attract certain blood proteins that can kill bacteria.
T lymphocytes: There are several types of T cells, each with a specialized job. Some T cells help protect the body against viruses, fungi, and some bacteria. For example, they recognize virus-infected cells and destroy them. T cells can also release substances called cytokines that attract other types of white blood cells, which then digest the infected cells. Some types of T cells help boost or slow the activity of other immune system cells.
Both types of lymphocytes can develop into lymphoma cells. Overall, B-cell lymphomas are much more common than T-cell lymphomas in the United States. In the skin, though, T-cell lymphomas are more common than B-cell lymphomas. Different types of lymphoma can develop from each type of lymphocyte.
Doctors can tell B-cells and T-cells apart using lab tests that detect certain proteins on their surfaces and certain features of their DNA. There are also several stages of B-cell and T-cell development that can be recognized by these lab tests.
This information is important because for each type of lymphoma, the cancer cells tend to resemble a particular type of normal lymphocyte at a certain stage of development. Figuring out the type of lymphoma a person has helps determine treatment options.
Lymphoid tissue: Most lymphocytes are in lymph nodes, which are small, bean-sized collections of immune cells throughout the body. Lymph nodes are connected to each other by narrow tubes similar to blood vessels called lymphatics (or lymph vessels). Lymph vessels carry a colorless, watery fluid (lymph) that contains lymphocytes.
Along with the lymph nodes, collections of lymphocytes can be found in many other places in the body, including the: Spleen, Bone marrow, Thymus, Adenoids and tonsils, Digestive tract, Skin, Other organs
Lymphomas can start in any part of the body that contains lymphoid tissue.
Some other types of cancer – lung or colon cancers, for example – can spread to lymph tissue such as the lymph nodes. But cancers that start in these places and then spread to the lymph tissue are not lymphomas.
Types of lymphoma of the skin
Classifying lymphoma of the skin can be confusing (even for many doctors) because there are many types and they are not very common.
The main system of classification is from the World Health Organization (WHO) and the European Organization for Research and Treatment of Cancer (EORTC) and is called the WHO-EORTC classification. It classifies skin lymphomas based on 2 important pieces of information:
How they look under the microscope
Whether there are certain proteins on the lymphoma cells (based on lab tests)
Using this combined information, the various types of skin lymphoma can be identified.
T-cell skin lymphomas
Most skin lymphomas are T-cell lymphomas.
Mycosis fungoides: This type accounts for about half of all skin lymphomas. Mycosis fungoides (MF) can occur at any age, but most patients are in their 50s and 60s. Men are almost twice as likely as women to develop this lymphoma.
The first sign of this disease is one or more patchy, scaly, red lesions (abnormal areas) on the skin. MF lesions can be very itchy. Often these lesions are the only symptom of MF. But in some people the disease can progress to more solid, raised tumors on the skin (called plaques). Because it can be confused with other skin problems, MF may be hard to diagnose at first. Several biopsies of the lesions may be needed before the diagnosis is confirmed.
In time, the lymphoma can spread across the skin or invade lymph nodes and organs like the liver. In many patients this disease grows slowly, but it can be faster growing in older patients. Some people with MF go on to develop Sezary syndrome.
Sezary syndrome: This disease is often thought of as an advanced form of mycosis fungoides, but these are actually 2 different diseases. In Sezary syndrome (SS), most or all of the skin is involved, instead of just patches of skin. The disease causes a very itchy, red rash that can look like a sunburn. This is called generalized erythroderma. The skin is often thickened. Lymphoma cells, called Sezary cells, can be found in the bloodstream (as well as in the lymph nodes).
Patients with SS often have weakened immune systems, which increases their risk of serious infections. Whereas MF is usually slow growing, SS tends to grow and spread faster.
Primary cutaneous anaplastic large cell lymphoma (ALCL): This lymphoma usually starts as one or a few tumors on the skin. The tumors can vary in size, with some smaller than an inch across and others several inches across.
Most people with this disease are in their 50s and 60s, but it can also occur in children. It is found at least twice as often in men as in women. In most cases it does not spread beyond the skin, and the prognosis (outlook) is very good.
Lymphomatoid papulosis: This is a benign, slow-growing disease that often comes and goes on its own, even without treatment. It often begins as several large pimple-like lesions that may develop an ulcer in the middle. Under the microscope, lymphomatoid papulosis has certain features that may look like primary cutaneous ALCL.
This disorder of the skin is seen in younger people more often than the other T-cell skin lymphomas, with an average age of around 45. Men get this disease more often than women.
This disease often goes away without treatment, but it can take anywhere from a few months to many years to go away completely. Lymphomatoid papulosis doesn’t spread to internal organs and is not fatal. Rarely, some people with this skin disorder develop some other type of lymphoma that is more serious.
Subcutaneous panniculitis-like T-cell lymphoma: This rare lymphoma invades the deepest layers of the skin, where it causes nodules (lumps) to form. Most often these develop on the legs, but they can occur anywhere on the body. This lymphoma affects all ages and both sexes equally. It usually grows slowly and tends to have a good prognosis.
Primary cutaneous peripheral T-cell lymphoma, unspecified: This is a group of rare skin lymphomas that don’t fit into any special category. There are several types.
Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma develops as widespread patches, nodules and tumors. It can sometimes look like mycosis fungoides, but a biopsy will show that it is this specific tumor.
Cutaneous gamma/delta T-cell lymphoma develops as thickened plaques or actual tumors, mainly on skin of the arms and legs, but sometimes in the intestines or lining of the nose. This is a very aggressive lymphoma that spreads quickly. In older classification systems, this was considered a subtype of subcutaneous panniculitis-like T cell lymphoma.
Primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma often starts as a single area of thickening of the skin or a tumor, but later there may be multiple tumors.
Skin lymphomas that don’t fall into any of these 3 categories are called simply primary cutaneous peripheral T-cell lymphoma, unspecified. People can have either single or multiple nodules.
B-cell skin lymphomas
Primary cutaneous marginal-zone B-cell lymphoma: This is a very slow-growing lymphoma that is usually curable. In Europe (but not in the United States), it is sometimes linked to an infection with Borrelia, the germ that causes Lyme disease.
This lymphoma can occur at any age. It causes skin lesions that are red to purplish large pimples, plaques (raised or lowered, flat lesions), or nodules (bumps) on sun-exposed areas of skin, like the arms. There may be only a single lesion, but there can sometimes be a few.
Primary cutaneous follicle-center lymphoma: This is the most common B-cell lymphoma of the skin. It tends to grow slowly. The early lesions are groups of red pimples, nodules, or plaques that form on the scalp, forehead or trunk. They are seldom found on the legs. Sometimes the pimples change into nodules.
This type is typically found in middle-aged adults. It is very sensitive to radiation therapy, and most patients have an excellent outlook.
Primary cutaneous diffuse large B-cell lymphoma, leg type: This is a fast growing lymphoma that begins as large nodules, mainly on the lower legs. It occurs most often in older people, and is more common in women than men. In some patients, this lymphoma spreads to lymph nodes and internal organs, causing serious problems.
These lymphomas often require more intensive treatment. The outlook is best if there is only one lesion at the time of diagnosis.
Primary cutaneous diffuse large B-cell lymphoma, other (non-leg): This rare skin lymphoma is similar to large B-cell lymphomas that appear on the legs, except it develops on other sites in the body. It can also develop inside blood vessels under the skin. It tends to require intensive treatment, and the outlook is best if it is limited to only the skin.
What are the risk factors for lymphoma of the skin?
A risk factor is anything that increases your chance of getting a disease like cancer. While most people with lymphoma of the skin may have some factors that make them more likely to get this disease (such as their age or gender), in most people there is no clear cause of the lymphoma. Having one or more risk factors doesn’t mean that you will develop this cancer.
Age: Age is an important risk factor for this disease, with most cases occurring in people in their 50s and 60s.
Gender and race: Most (but not all) types of skin lymphoma are more common in men than in women. Most also tend to be more common in African-Americans than in whites. The reasons for this are not known.
Weakened immune system: Skin lymphomas may be more common in people with acquired immunodeficiency syndrome (AIDS), who have a weakened immune system. They may also be more common in people who have had an organ transplant such as a heart, kidney or liver transplant. These people must take drugs that suppress their immune system, which may raise the risk of skin lymphoma or lymphomas in other areas of the body.
Infections: Infection with the human immunodeficiency virus (HIV), the virus that causes AIDS, may increase a person’s risk of skin lymphoma.
In parts of Europe (but not in the United States), infection with Borrelia, the bacteria that causes Lyme disease, has also been linked with skin lymphomas. This link has only been reported in a small number of cases—most people with skin lymphoma have not had Lyme disease, and most people with Lyme disease do not develop lymphoma of the skin.
Do we know what causes lymphoma of the skin?
In most patients with non-Hodgkin lymphoma of the skin, the cause of their cancers is unknown.
Scientists now understand how certain changes in DNA can cause normal lymphocytes to become lymphoma cells. DNA is the chemical in each of our cells that makes up our genes – the instructions for how our cells function. We usually look like our parents because they are the source of our DNA. But DNA affects more than just how we look.
Some genes contain instructions for controlling when our cells grow, divide into new cells, and die at the right time. Certain genes that help cells grow, divide, or live longer are called oncogenes. Others that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes.
Some people inherit DNA mutations (changes) from a parent that increases their risk of developing some types of cancer. But non-Hodgkin lymphoma is not one of the cancer types often caused by inherited mutations.
DNA changes related to non-Hodgkin lymphoma are usually acquired after birth, rather than being inherited. Some of these acquired changes may have outside causes, but often they occur for no apparent reason. They seem to happen more often as we age, which may help explain why lymphomas usually occur in older people.
Each time a cell divides into 2 new cells, it must make a copy of its DNA. This process is not perfect, and sometimes copying errors occur. Cells have repair enzymes to help fix these errors, but some may slip past, especially if the cells are growing rapidly.
The DNA in each human cell is packaged in 23 pairs of chromosomes. Changes in chromosomes are sometimes seen in skin lymphoma cells, but there is no single chromosome change that is common to all skin lymphomas. In some cases, part of one of the chromosomes is lost (known as a chromosomal deletion). Translocations, in which DNA from one chromosome breaks off and becomes attached to a different chromosome, are another type of change seen in some skin lymphomas. When these types of chromosome changes happen, oncogenes can be turned on or tumor suppressor genes can be turned off.
Scientists are learning about the exact genes involved in this process and how they may cause lymphoma and other cancers. This information is being used to develop new tests for detecting and classifying certain types of non-Hodgkin lymphoma, as well as for developing new treatments.
Even though researchers have found some of the key DNA changes that cause lymphoma, they still do not know why these changes occur.
The immune system seems to play an important role in some cases of lymphoma. People with immune deficiencies (due to inherited conditions, drug treatment, organ transplants, or HIV infection) have a much greater chance of developing lymphoma than people without an immune deficiency.
How is lymphoma of the skin diagnosed?
Because this type of lymphoma affects the skin, it is often noticed fairly quickly. But the actual diagnosis of skin lymphoma might be delayed because the symptoms often resemble other, more common skin problems. The diagnosis of skin lymphoma can only be confirmed with tests such as biopsies (described below).
Signs and symptoms of skin lymphoma
Lymphomas of the skin can be seen and felt. They can appear as: Papules (small, pimple-like lesions), Patches (flat lesions), Plaques (thick, raised or lowered lesions), Nodules or tumors (larger lumps or bumps under the skin)
The lesions are often itchy, scaly, and red to purple in color. The lymphoma might show up as more than one type of lesion and on different parts of the skin (often in areas not exposed to the sun). Some skin lymphomas appear as a rash over some or most of the body (known as erythroderma). Sometimes larger lesions may break open (ulcerate).
Along with skin problems, in rare cases lymphoma of the skin can cause general symptoms, such as: Unexplained weight loss, Fever, Profuse sweating (enough to soak clothing), particularly at night, Severe itchiness
Most of these symptoms are more likely to be caused by other, less serious conditions. Still, if you have any of them it is important to have them checked by a doctor so that the cause can be found and treated, if needed.
Medical history and physical exam: Usually the first step is for your doctor to take your medical history. The doctor probably will ask when the changes in your skin first appeared, whether they have changed in size or appearance, and whether they are itchy or painful. You may be asked if you have any other symptoms, like fever or weight loss. Because skin lymphomas can be hard to tell apart from allergies and other causes of rashes, you might also be asked if you have recently been exposed to something that could be causing your skin problems, such as if you are taking a new medicine or are using a new laundry detergent.
During the physical exam, your doctor will note the size, shape, color, and texture of any area(s) of skin in question. The rest of your body will be checked for other areas of skin involvement.
The doctor may also feel the lymph nodes (small, bean-sized collections of immune cells) under the skin in the neck, underarm, or groin, as lymphomas can sometimes cause lymph nodes to become enlarged.
If you are being seen by your primary doctor and skin lymphoma or another type of skin cancer is suspected, you may be referred to a dermatologist (a doctor who treats skin diseases), who will look at the skin more closely.
Biopsy: A biopsy is a procedure in which a doctor removes a sample of body tissue for viewing under a microscope or other lab tests. A biopsy is needed to diagnose lymphoma of the skin.
Skin biopsies: There are several types of skin biopsies, and the doctor’s choice is based on each person’s situation. Usually a skin biopsy is done by a dermatologist.
Punch biopsy: For a punch biopsy, the doctor uses a tool that looks like a tiny round cookie cutter (usually a little more than 1/8 inch across). Once the skin is numbed with a local anesthetic, the doctor rotates the punch biopsy tool on the surface of the skin until it cuts through all the layers of the skin. Often the biopsy site is closed with a stitch.
Incisional and excisional skin biopsies: For these types of biopsies, a surgical knife is used to cut through the full thickness of skin. An incisional biopsy removes only part of the tumor, while an excisional biopsy removes the entire tumor. The piece of skin is removed for testing, and the edges of the wound are sewn together. These biopsies are usually done using a local anesthetic (numbing medicine).
Regardless of the type of skin biopsy, once the samples are removed, they are sent to a pathologist, a doctor trained in using a microscope and other lab tests to diagnose diseases.
Many of the more common forms of skin cancer (and other skin diseases) can be diagnosed just by looking at the biopsy samples under a microscope. But diagnosing and classifying lymphomas of the skin often requires one or more special lab tests to identify the lymphoma cells.
Diagnosing some forms of skin lymphoma can be very challenging, and it’s a good idea to ask your doctor how confident he or she is in the expertise of the pathologist testing your biopsy specimen. Sometimes, especially if the diagnosis is unclear, the skin samples may need to be sent to a dermatopathologist, a dermatologist or a pathologist with additional training in diagnosing skin samples. Even with this expertise, in some cases several biopsies may be needed over a period of time before the diagnosis is confirmed.
Lymph node biopsies: Skin lymphomas often spread to lymph nodes, so your doctor may recommend a lymph node biopsy to help confirm the diagnosis or to help determine how widespread the lymphoma is. This is more likely to be done if the doctor detects enlarged lymph nodes, either during a physical exam or with imaging tests (such as a CT scan).
Excisional or incisional lymph node biopsy: This is the most common type of lymph node biopsy. In this procedure, a surgeon cuts through the skin to remove either the entire lymph node (excisional biopsy) or a small part of a large tumor (incisional biopsy). If the node is near the skin surface, this is a simple operation that can often be done with local anesthesia. But if the node is inside the chest or abdomen, the patient will be asleep or deeply sedated during the biopsy.
Removing a lymph node almost always provides enough tissue to diagnose the exact type of lymphoma. This type of biopsy is preferred by most doctors, if it can be done without too much discomfort to the patient.
Fine needle aspiration (FNA) biopsy: In an FNA biopsy, the doctor uses a very thin, hollow needle attached to a syringe to withdraw (aspirate) a small amount of tissue from a tumor. If an enlarged node is near the surface of the body, the doctor can aim the needle while feeling it. If the enlarged node is deep inside the body, the doctor can guide the needle while viewing it with ultrasound or a CT scan (see “Imaging tests”).
An FNA does not require surgery, but in some cases it cannot remove enough tissue to make a definite diagnosis of lymphoma. But advances in lab tests (discussed later in this section) and the growing experience of many doctors with FNA have improved the accuracy of this procedure. Some doctors will use FNA in patients already diagnosed with lymphoma of the skin to confirm that an enlarged lymph node also contains lymphoma.
Other types of biopsies: These procedures may sometimes be done to confirm a diagnosis of lymphoma, but they are more often done to help stage (determine the extent of) a lymphoma that has already been diagnosed.
Bone marrow aspiration and biopsy: These procedures are sometimes done after lymphoma has been diagnosed to help determine if it has reached the bone marrow. The two tests are often done at the same time. The samples are usually taken from the back of the pelvic (hip) bone, but in some cases they may be taken from the sternum (breast bone) or other bones.
In bone marrow aspiration, you lie on a table (either on your side or on your belly). The doctor cleans the skin over the hip and then numbs the area and the surface of the bone with local anesthetic. This may cause a brief stinging or burning sensation. A thin, hollow needle is then inserted into the bone and a syringe is used to suck out a small amount of liquid bone marrow (about 1 teaspoon). Even with the anesthetic, most patients still have some brief pain when the marrow is removed.
A bone marrow biopsy is usually done just after the aspiration. A small piece of bone and marrow is removed with a slightly larger needle that is twisted as it is pushed down into the bone. The biopsy may also cause some brief pain. Once the biopsy is done, pressure will be applied to the site to help stop any bleeding.
Lumbar puncture (spinal tap): This test looks for lymphoma cells in the cerebrospinal fluid (CSF), which is the liquid that bathes the brain and spinal cord. Most people with skin lymphoma will not need this test. But doctors may order it if a person has symptoms that suggest the lymphoma may have reached the brain.
For this test, you may be asked to lie on your side or sit up. The doctor first numbs an area in the lower part of the back over the spine. A small, hollow needle is then placed between the bones of the spine to withdraw some of the fluid.
Lab tests of biopsy or blood samples: These tests may be done on biopsy samples or, in some cases, blood samples to help diagnose lymphoma and determine what type it is. Pathologists can sometimes tell which kind of lymphoma a patient has by just looking at the cells under a microscope, but usually these other types of tests are needed to confirm the diagnosis.
Immunohistochemistry: In this test, a part of the biopsy sample is treated with special antibodies (man-made versions of immune system proteins) that attach to cells only if specific molecules are on their surface or inside the cells. These antibodies cause color changes, which can be seen under a microscope. This test can be used to help diagnose lymphoma and tell what type it is.
Flow cytometry: Like immunohistochemistry, this test looks for certain substances on the surface of cells that help identify what types of cells they are. But this test can look at many more cells than immunohistochemistry.
For this test, a sample of cells is treated with special antibodies that stick to the cells only if certain substances are present on their surfaces. The cells are then passed in front of a laser beam. If the cells now have antibodies attached to them, the laser will cause them to give off light, which can be measured and analyzed by a computer. Groups of cells can be separated and counted by these methods.
This is the most commonly used test for immunophenotyping – classifying lymphoma cells according to the substances (antigens) on their surfaces. Different types of lymphocytes have different antigens on their surface. These antigens may also change as each cell matures.
Flow cytometry can help determine whether a biopsy sample contains lymphoma, some other cancer, or a non-cancerous disease. It has also become very useful in helping doctors determine the exact type of lymphoma so that they can select the best treatment.
Cytogenetics: This technique allows doctors to evaluate the chromosomes (long strands of DNA) in the lymphoma cells. The cells are looked at under a microscope to see if the chromosomes have any translocations (where part of one chromosome has broken off and is now attached to another chromosome), as happens in certain types of lymphoma. Some lymphoma cells may have too many chromosomes, too few chromosomes, or other chromosome abnormalities. These changes can help identify the type of lymphoma.
Cytogenetic testing usually takes about 2 to 3 weeks because the lymphoma cells must be grown in lab dishes for a couple of weeks before their chromosomes can be viewed under the microscope.
Molecular genetic tests: These tests look more closely at lymphoma cell DNA. They can detect most changes that are visible under a microscope in cytogenetic tests, as well as others that can’t be seen. Recognizing these changes can help doctors decide whether a rash or lesion is due to a benign condition or a skin lymphoma. These tests can also tell the difference between B-cell and T-cell lymphomas.
Fluorescent in situ hybridization (FISH): FISH is similar to cytogenetic testing. It uses special fluorescent dyes that only attach to specific parts of chromosomes. FISH can find most chromosome changes (such as translocations) that can be seen under a microscope in standard cytogenetic tests, as well as some changes too small to be seen with usual cytogenetic testing.
FISH can be used to look for specific changes in chromosomes. It can be used on regular blood or bone marrow samples. It is very accurate and can usually provide results within a couple of days, which is why this test is now used in many medical centers.
Polymerase chain reaction (PCR): PCR is a very sensitive DNA test that can also find some chromosome changes too small to be seen under a microscope, even if very few lymphoma cells are present in a sample.
Molecular genetic tests can also detect certain genes that have been “turned on” and are contributing to the lymphoma cells’ abnormal growth. As researchers learn more about lymphomas, these may become even more important.
Blood tests: Blood tests are used to measure the amounts of certain types of cells and chemicals in the blood. They are not used to diagnose lymphoma, but they can sometimes help determine how advanced the lymphoma is. They may also be done during certain types of treatment (such as chemotherapy) to monitor how well the bone marrow and other organs are functioning.
Complete blood count (CBC): This test measures the levels of different cells in the blood, such as the red blood cells, the white blood cells, and the platelets. The CBC is often done with a differential (or “diff”) which counts the numbers of different types of white blood cells. If a person’s blood counts are low, it might mean that the lymphoma is growing in the bone marrow and crowding out normal blood cell production.
People with Sezary syndrome will have Sezary cells in the blood, which can be found on the differential.
Blood chemistry tests: These tests look at how well the kidneys and liver are working. Results of another blood test that measures levels of lactate dehydrogenase (LDH) will often be abnormally high in the blood of patients with widespread lymphoma. The level goes up when larger amounts of lymphoma are present.
Imaging tests: Imaging tests use x-rays, sound waves, magnetic fields, or radioactive particles to produce pictures of the inside of the body. These tests may be done for a number of reasons, including:
To help find a suspicious area that might be cancerous
To learn how far a cancer may have spread
To help determine if treatment has been effective
To look for possible signs of cancer recurrence
For skin lymphomas, imaging tests are not always needed for patients with only a few skin lesions, but they are often done in patients with a lot of skin involvement or if lymphoma cells are found in the lymph nodes or blood.
Chest x-ray: An x-ray of the chest may be done to look for enlarged lymph nodes in this area.
Computed tomography (CT) scan: The CT scan is a type of x-ray test that produces detailed, cross-sectional images of your body. Instead of taking one picture, like a standard x-ray, a CT scanner takes many pictures as it rotates around you. A computer then combines these pictures into detailed images of the part of your body that is being studied.
Unlike a regular x-ray, CT scans can show the detail in soft tissues (such as internal organs). This scan can help tell if any lymph nodes or organs in your body are enlarged.
A CT scanner has been described as a large donut, with a narrow table in the middle opening. You will need to lie still on the table while the scan is being done. CT scans take longer than regular x-rays, and you might feel a bit confined by the ring while the pictures are being taken.
Before the test, you may be asked to drink 1 to 2 pints of a liquid called oral contrast. This helps outline the intestine so that certain areas are not mistaken for tumors. You may also receive an IV line through which a different kind of contrast dye (IV contrast) is injected.
The injection can cause some flushing (redness and warm feeling). A few people are allergic to the dye and get hives or, rarely, have more serious reactions like trouble breathing and low blood pressure. Be sure to tell the doctor if you have any allergies or have ever had a reaction to any contrast material used for x-rays.
CT-guided needle biopsy: CT scans can also be used to guide a biopsy needle precisely into a suspicious area. For this procedure, the patient remains on the CT scanning table while the doctor advances a biopsy needle through the skin and toward the tumor. CT scans are repeated until the needle is within the mass. A biopsy sample is then removed to be looked at under a microscope.
Magnetic resonance imaging (MRI) scan: Like CT scans, MRI scans provide detailed images of soft tissues in the body. But MRI scans use radio waves and strong magnets instead of x-rays. The energy from the radio waves is absorbed by the body and then released in a pattern formed by the type of body tissue and by certain diseases. A computer translates the pattern into a very detailed image of parts of the body.
A contrast material called gadolinium is often injected into a vein before the scan to better see details. The contrast material usually does not cause allergic reactions.
MRI scans are very helpful in looking at the brain and spinal cord, but they are not often used to evaluate skin lymphomas unless a CT scan can’t be done for some reason.
MRI scans take longer than CT scans – often up to an hour. You may have to lie inside a narrow tube, which is confining and can be distressing to some people. Newer, more open MRI machines may be another option. The MRI machine makes loud buzzing and clicking noises that you may find disturbing. Some places provide headphones or earplugs to help block this out.
Ultrasound: Ultrasound uses sound waves and their echoes to produce a picture of internal organs or masses. For this test, a small, microphone-like instrument called a transducer is placed on the skin (which is first lubricated with a gel). It gives off sound waves and picks up the echoes as they bounce off the organs. The echoes are converted by a computer into a black and white image that is displayed on a screen.
Ultrasound can be used to look at lymph nodes near the surface of the body or to look inside your abdomen for enlarged lymph nodes or organs such as the liver and spleen. (It can’t be used to look at organs or lymph nodes in the chest because the ribs block the sound waves.) It is sometimes used to help guide a biopsy needle into an enlarged lymph node.
This is an easy test to have done, and it uses no radiation. You simply lie on a table, and a technician moves the transducer over the part of your body being looked at.
Positron emission tomography (PET) scan: For a PET scan, a radioactive substance (usually a type of sugar related to glucose, known as FDG) is injected into the blood. The amount of radioactivity used is very low. Because cancer cells in the body grow quickly, they absorb large amounts of the radioactive sugar. After about an hour, you will be moved onto a table in the PET scanner. You lie on the table for about 30 minutes while a special camera creates a picture of areas of radioactivity in the body. The picture is not finely detailed like a CT or MRI scan, but it can look for possible areas of lymphoma in all areas of the body at once.
PET scans can help tell if an enlarged lymph node contains lymphoma or is benign. It can also help spot small areas that might be lymphoma, even if the area looks normal on a CT scan.
PET scans can also be used to tell if a lymphoma is responding to treatment. Some doctors will repeat the PET scan after 1 or 2 courses of chemotherapy. If the chemotherapy is working, the abnormal areas will no longer take up the radioactive sugar.
Some newer machines are able to perform both a PET and CT scan at the same time (PET/CT scan). This lets the doctor compare areas of higher radioactivity on the PET scan with the more detailed appearance of that area on the CT.
How is lymphoma of the skin staged?
Once skin lymphoma is diagnosed, tests are done to determine the stage (extent of spread) of the disease. The tests used to gather information for staging include: Physical exam, Biopsies, Imaging tests, such as CT scans, Blood tests
Knowing the stage of the lymphoma may help in deciding the best treatment and in predicting a patient’s prognosis (outlook), but staging is not as important for skin lymphomas as it is for many other types of cancer.
A staging system is a standard way to describe the extent of cancer spread. The staging systems for skin lymphomas were developed by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization for Research and Treatment of Cancer (EORTC). There are 2 different staging systems – one for mycosis fungoides and Sezary syndrome and another for the other skin lymphomas. These systems are complex and can be hard to understand. If you have questions about the stage of your lymphoma, ask your cancer care team to explain it to you in a way you understand. This can help you make informed choices about your treatment.
Staging for mycosis fungoides and Sezary syndrome
Mycosis fungoides (MF) and Sezary syndrome are staged based on 4 factors:
T describes how much of the skin is affected by the lymphoma (tumor).
N describes the extent of the lymphoma in the lymph nodes.
M is for the spread (metastasis) of the lymphoma to other organs.
B is for lymphoma cells in the blood.
T categories
T1: Skin lesions can be small patches (flat lesions), papules (small bumps), and/or plaques (raised or lowered, flat lesions), but the lesions cover less than 10% of the skin surface.
T2: The patches, papules, and/or plaques cover 10% or more of the skin surface.
T3: At least one of the skin lesions is a tumor that is at least 1 centimeter (cm) across (a cm is a little less than 1/2 inch).
T4: The skin lesions have spread, grown larger, and grown together to cover at least 80% of the skin surface.
N categories
N0: Lymph nodes are not enlarged and a lymph node biopsy is not needed.
N1: Lymph nodes are enlarged, but the patterns of cells look normal or close to normal under the microscope.
N2: Lymph nodes are enlarged, and the patterns of cells look more abnormal under the microscope.
N3: Lymph nodes are enlarged, and the patterns of cells look very abnormal under the microscope.
NX: Lymph nodes are enlarged but haven't been removed to be looked at under the microscope.
M categories
M0: The lymphoma cells have not spread outside the skin or lymph nodes.
M1: Lymphoma cells have spread to other organs or tissues, such as the liver or spleen.
B categories
B0: Less than 5% of lymphocytes in the blood are Sezary cells.
B1: Low numbers of Sezary cells in the blood (more than in B0 but less than in B2).
B2: High number of Sezary cells in the blood (at least 1,000 cells per microliter).
Stage grouping: Once the values for T, N, M, and B are known, they are combined to determine the overall stage of the lymphoma. This process is called stage grouping.
Stage IA: T1, N0, M0, B0 or B1: There are skin lesions but no tumors. Skin lesions cover less than 10% of the skin surface (T1), the lymph nodes are not enlarged (N0), lymphoma cells have not spread to other organs or tissues (M0), and the number of Sezary cells in the blood is not high (B0 or B1).
Stage IB: T2, N0, M0, B0 or B1
There are skin lesions but no tumors. Skin lesions cover at least 10% of the skin surface (T2), the lymph nodes are not enlarged (N0), lymphoma cells have not spread to other organs or tissues (M0), and the number of Sezary cells in the blood is not high (B0 or B1).
Stage IIA: T1 or T2, N1 or N2, M0, B0 or B1
There are skin lesions but no tumors. Skin lesions can cover up to 80% of the skin surface (T1 or T2). The lymph nodes are enlarged but the patterns of cells do not look very abnormal under the microscope (N1 or N2). Lymphoma cells have not spread to other organs or tissues (M0), and the number of Sezary cells in the blood is not high (B0 or B1).
Stage IIB: T3, N0 to N2, M0, B0 or B1
At least one of the skin lesions is a tumor that is 1 cm across or larger (T3). The lymph nodes are either normal (N0) or are enlarged but the patterns of cells do not look very abnormal under the microscope (N1 or N2). Lymphoma cells have not spread to other organs or tissues (M0), and the number of Sezary cells in the blood is not high (B0 or B1).
Stage IIIA: T4, N0 to N2, M0, B0
Skin lesions cover at least 80% of the skin surface (T4). The lymph nodes are either normal (N0) or are enlarged but the patterns of cells do not look very abnormal under the microscope (N1 or N2). Lymphoma cells have not spread to other organs or tissues (M0), and there are few (or no) Sezary cells in the blood (B0).
Stage IIIB: T4, N0 to N2, M0, B1
Skin lesions cover at least 80% of the skin surface (T4). The lymph nodes are either normal (N0) or are enlarged but the patterns of cells do not look very abnormal under the microscope (N1 or N2). Lymphoma cells have not spread to other organs or tissues (M0), and the number of Sezary cells in the blood is low (B1).
Stage IVA1: any T, N0 to N2, M0, B2
Skin lesions can cover any amount of the skin surface (any T). The lymph nodes are either normal (N0) or are enlarged but the patterns of cells do not look very abnormal under the microscope (N1 or N2). Lymphoma cells have not spread to other organs or tissues (M0), and the number of Sezary cells in the blood is high (B2).
Stage IVA2: any T, N3, M0, any B
Skin lesions can cover any amount of the skin surface (any T). Some lymph nodes are enlarged and the patterns of cells look very abnormal under the microscope (N3). Lymphoma cells have not spread to other organs or tissues (M0). Sezary cells may or may not be in the blood (any B).
Stage IVB: any T, any N, M1, any B
Skin lesions can cover any amount of the skin surface (any T). The lymph nodes may be normal or abnormal (any N), and Sezary cells may or may not be in the blood (any B). Lymphoma cells have spread to other organs or tissues, such as the liver or spleen (M1).
Staging for other skin lymphomas: The staging system for types of skin lymphoma other than mycosis fungoides and Sezary syndrome is still fairly new, and doctors are still trying to determine how useful it is. The system includes 3 factors: (a) T describes how much of the skin is affected by the lymphoma (tumor). (b) N describes the extent of the lymphoma in the lymph nodes. (c) M is for the spread (metastasis) of the lymphoma to other organs.
For these lymphomas, only the T category is used at the time of diagnosis. If sites besides the skin are involved at the time of diagnosis (such as lymph nodes), these lymphomas are no longer considered skin lymphomas and they are staged like regular non-Hodgkin lymphoma. The N and M categories are only used if the lymphoma progresses (continues to grow) during treatment or comes back after treatment.
T categories
T1: There is only a single skin lesion.
T1a: The skin lesion is less than 5 cm (about 2 inches) across.
T1b: The skin lesion is larger than 5 cm across.
T2: There are 2 or more places on the skin with lymphoma. These places may be in a single body region or in 2 body regions that are next to each other.
T2a: All of the skin lesions could be placed within a circle that is 15 cm (about 6 inches) across.
T2b: The circle needed to surround all of the skin lesions is larger than 15 cm across, but smaller than 30 cm across.
T2c: The circle needed to surround all of the skin lesions is larger than 30 cm across.
T3: There are skin lesions in body regions that aren’t next to each other, or in at least 3 different body regions.
T3a: There are many lesions involving 2 body regions that aren’t next to each other.
T3b: There are many lesions involving 3 or more body regions.
N categories
N0: None of the lymph nodes is enlarged or contain lymphoma cells.
N1: There are lymphoma cells in the lymph nodes that drain an area where skin contained lymphoma.
N2: One of the following is true:
At least 2 sets of lymph nodes from different areas contain lymphoma cells
There are lymphoma cells in lymph nodes that do not drain areas where the skin contained lymphoma.
N3: Lymph nodes deep inside the chest or abdomen contain lymphoma cells.
M categories
M0: No signs of lymphoma outside of the skin or lymph nodes.
M1: Lymphoma has spread to other organs or tissues.
This system does not assign an overall stage to the lymphoma, as the system for mycosis fungoides/Sezary syndrome does. Because this system is still fairly new, it’s not yet clear how well it can help predict a person’s prognosis (outlook).
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