What is Primary Cutaneous Follicle Center Lymphoma?
Different types of lymphomas, which are a kind of blood cancer, can affect the skin. Most of these are actually a type called T-cell lymphomas. However, when we talk about lymphomas that originate from B cells and affect the skin, the most common one is called primary cutaneous follicle center lymphoma (PCFCL). Primary cutaneous lymphomas are identified when the skin is involved but there’s no evidence of the disease elsewhere in the body or in lymph nodes at the time of diagnosis.
Take note, B-cell lymphomas account for 25% of skin lymphomas and they tick differently. They have a unique clinical path and prognosis, or likely outcome, when compared to other similar lymphomas that affect other areas of the body.
PCFCL, in particular, is a low-grade (less likely to spread quickly) B-cell lymphoma that comes from cells in the center of lymph follicles, which are tiny structures in the lymph nodes. It usually appears on the head or trunk and generally has a very good prognosis, meaning the chances of recovery are high.
What Causes Primary Cutaneous Follicle Center Lymphoma?
PCFCL, or Primary Cutaneous Follicle Center Lymphoma, is a type of less aggressive lymphoma where B-cells in the center of the lymph nodes become abnormal. It’s important to note that at the time of diagnosis, the disease hasn’t spread to other lymph nodes or elsewhere in the body.
Risk Factors and Frequency for Primary Cutaneous Follicle Center Lymphoma
PCFCL, a type of skin lymphoma, is the most common form of cutaneous B-cell lymphoma in Western countries, making up more than half of such cases. It generally impacts middle-aged adults more often, with a slight bias towards men. For every 1.5 men affected with PCFCL, there’s 1 woman with the same condition.
Signs and Symptoms of Primary Cutaneous Follicle Center Lymphoma
Primary Cutaneous Follicle Center Lymphoma (PCFCL) usually shows up as single or grouped, firm to purplish, painless, non-itchy bumps, plaques, or tumors. These often appear on the head, neck, and trunk. The lesions are usually smooth and don’t have any sores. While 60% of patients may have more than one lesion in a certain area, only a small number of patients (10%-20%) will have lesions in multiple spots.
- Single or grouped, firm to purplish, painless, non-itchy bumps, plaques, or tumors
- Lesions often appear on the head, neck, and trunk
- Lesions are usually smooth and not sore
- 60% of patients may have more than one lesion in a specific area
- Only 10%-20% of patients will have lesions in multiple spots
Testing for Primary Cutaneous Follicle Center Lymphoma
The diagnosis of primary cutaneous follicle center lymphoma (PCFCL), a type of skin cancer, typically happens through a skin biopsy. During a biopsy, a small piece of skin is removed and tested in a laboratory. This examination is done to look for certain cells that are common in PCFCL. These cells, called neoplastic cells, usually test positive for markers called CD19, CD20, CD79a, CD10, and BCL-6, which are proteins found on the surface of the cells.
During the lab test, the cells may also be tested for markers such as CD21/23, which can identify BCL-6 positive cells that have moved beyond their original location. Most of the time, PCFCL will not test positive or will only weakly test positive for BCL-2. The marker CD10 can be variable and is usually found in cells that are arranged in a follicular pattern, similar to the pattern found in hair follicles, and is not found in cells that are spread around in a random pattern. The markers CD5, CD43, IRF4/MUM1, and FOXP1, are typically not found in these cells.
Furthermore, a marker called Ki-67 may show less cell growth compared to normal growth rate of cells. This is important because cancer cells usually grow and multiply faster than normal cells.
Additionally, the health team would carry out other lab tests, including a complete blood count to measure the number of different types of cells in your blood, and a comprehensive metabolic panel to provide information about your body’s overall health. They would also test for lactate dehydrogenase, an enzyme that could increase when there’s tissue damage or disease present.
Imaging tests like a computed tomography (CT) scan, which uses x-rays to produce detailed pictures of the inside of the body, and possibly positron emission tomography (PET) scans, which uses a radioactive substance to look for disease in the body, might be performed to rule out any further spread of the cancer.
Treatment Options for Primary Cutaneous Follicle Center Lymphoma
If you have primary cutaneous follicle center lymphoma (PCFCL), your treatment plan will depend on the location and number of lesions, and the symptoms you’re experiencing. A lesion refers to an abnormal spot on your skin that is different from the skin around it.
One common treatment for PCFCL is local radiotherapy, which is a type of treatment where high-energy beams, such as X-rays, are used to kill abnormal cells. This treatment is recommended if you have single lesions or multiple lesions in the same area. Studies have shown that this treatment can eliminate the disease in nearly all patients (99%).
For smaller lesions, surgery may be used to remove the lesion completely. This is another very successful treatment option, curing nearly all patients (99%). However, it has been observed that patients who had surgery may have an increased risk of the disease returning compared to those who underwent radiotherapy.
If you have many lesions spread across different parts of your body, which means they can’t all be treated with radiotherapy at once, your doctor might recommend local radiotherapy on each affected area or just keeping an eye on them without initial treatment.
For those with a high number of lesions or very thick lesions that are not suitable for radiotherapy, a medication called rituximab could be suggested. Rituximab is a type of drug called a monoclonal antibody, which works by targeting and attaching to specific proteins on the surface of cancer cells, signaling the immune system to destroy them.
If your body doesn’t respond to rituximab, treatment options include chemotherapy. The regimens of R-CHOP and CHOP have been utilized in prior studies. R-CHOP consists of Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisone, while CHOP contains the same medications minus Rituximab.
Finally, it’s encouraging to know that even if PCFCL comes back on your skin, this does not necessarily mean your overall prognosis is worse. The approach to managing recurring PCFCL is similar to the initial treatment strategy.
What else can Primary Cutaneous Follicle Center Lymphoma be?
While trying to diagnose potential health conditions, doctors sometimes have to consider multiple possibilities that could cause the symptoms. In this case, the possible conditions include:
- Follicular lymphoma (a type of blood cancer)
- Primary cutaneous diffuse large B-cell lymphoma, Leg type (a rare non-Hodgkin lymphoma)
- Reactive lymphoid hyperplasia (an uncommon, benign condition)
- Primary cutaneous marginal zone lymphoma (a type of skin lymphoma)
Follicular Lymphoma, which often affects the body’s lymphatic system, might involve the skin secondarily. It can show similarities to Primary Cutaneous Follicle Centre Lymphoma (PCFCL). Certain signs like strong CD10 and BCL-2 presence, symptoms like night sweats, fever, or weight loss (referred to as B symptoms) and swollen lymph nodes can suggest it might be Follicular Lymphoma. Genetic testing can also be helpful in distinguishing between the two.
Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg type typically affects the skin of the lower legs in older women. But in some cases, it may occur in other body parts as well. It has a worse prognosis than PCFCL and needs a more aggressive treatment approach, including a combination of chemotherapy and possibly radiation therapy.
Reactive Lymphoid Hyperplasia can sometimes resemble PCFCL when examining skin biopsies of the head. It contains multiple follicles that maintain the traditional structure. The skin disease, Primary Cutaneous Marginal Zone Lymphoma, which has reactive follicles, can also look like PCFCL. It usually has a more varied appearance with smaller cells and plasma cells limited to light chains. Immunohistochemistry can also help in diagnosis.
What to expect with Primary Cutaneous Follicle Center Lymphoma
In simple terms, PCFCL – a type of cancer that affects the lymph nodes – generally has a very good prognosis, meaning patients are likely to survive and recover. In fact, the five-year survival rate is over 95%. One of the encouraging aspects of this cancer is that it doesn’t matter how it grows, whether it has BCL-2 (a protein that can contribute to cancer growth) or not, whether it starts in multiple spots, or whether certain kinds of cells called centroblasts are present. None of these factors change the patient’s overall chances of recovery.
However, there is one exception: if PCFCL starts on the legs, it tends to be more aggressive and gives a worse outlook.
Possible Complications When Diagnosed with Primary Cutaneous Follicle Center Lymphoma
Primary cutaneous follicle center lymphoma (PCFCL) typically grows very slowly if not treated, with only 10% of cases spreading over time. Because it is a slow-moving disease, milder treatments like radiation therapy have become more common. Surgery is another option but may result in cosmetic issues, particularly if the growths are large or located on the head.
After treatment, it’s important for patients to have regular check-ups to keep an eye out for any signs of the lymphoma returning or any complications related to treatment. Unfortunately, about 30% of patients experience the lymphoma coming back on the skin.
Main Points:
- PCFCL, if untreated, can grow slowly over time and disseminate in 10% of cases
- Treatments such as radiation therapy are often used due to the slow-moving nature of the disease
- Surgery can be a treatment option but might lead to cosmetic problems, especially if the lymphoma is on the head.
- Regular check-ups post-treatment are necessary to monitor for any signs of PCFCL relapse or therapy-related complications.
- 30% of cases may see a return of the lymphoma on the skin.
Preventing Primary Cutaneous Follicle Center Lymphoma
Primary cutaneous follicle-center lymphoma (PCFCL) is a rare skin disease that usually has a favorable outcome. However, it can often be mistaken for similar skin diseases related to lymphoma, which could potentially lead to a more severe health prognosis. Even though the affected areas typically develop slowly, it’s really crucial for doctors to conduct a thorough examination. This is to ensure that they can exclude the existence of more serious or aggressive conditions, like primary cutaneous diffuse large B-cell lymphomas, leg type (PCDLBCL-LT).
It should be noted that if the disease isn’t treated, it can become aggressive in some cases, specifically affecting the local area. Interestingly, it’s also been observed that the disease tends to be more aggressive when it appears on the legs, compared to when it arises on the head, neck, or trunk of the body.
