Primary Mediastinal B-Cell Lymphoma — An Overview | MyLymphomaTeam

Connect with others who understand.

sign up Log in
Resources
About MyLymphomaTeam
Powered By

Primary Mediastinal B-Cell Lymphoma — An Overview

Medically reviewed by Mark Levin, M.D.
Written by Aminah Wali, Ph.D.
Posted on July 21, 2021

Primary mediastinal large B-cell lymphoma (PMBCL) — sometimes called primary thymic mediastinal lymphoma — is a cancer that forms from cells of the immune system called B lymphocytes, or B cells. It is a type of non-Hodgkin lymphoma (NHL), one of the major categories of lymphoma. PMBCL primarily affects young adults, mostly women, but it can also affect children. It is a relatively rare cancer and makes up only 2 percent to 4 percent of all non-Hodgkin lymphomas.

What Is Primary Mediastinal B-Cell Lymphoma?

PMBCL causes a tumor to form in a space between the lungs known as the mediastinum. The majority of people with PMBCL have a tumor greater than 10 centimeters in diameter — known as bulky disease — and are diagnosed in the early stages of disease, when it is still localized. Although PMBCL is a form of NHL, the condition’s cancer cells resemble those found in classical Hodgkin lymphoma when analyzed under a microscope. PMBCL also shares features with diffuse large B-cell lymphoma (DLBCL). PMBCL may be considered a subtype of DLBCL.

Signs and Symptoms of Primary Mediastinal Large B-Cell Lymphoma

Most signs and symptoms of PMBCL are caused by the mediastinal mass compressing nearby organs. They may include:

  • Difficulty swallowing (dysphagia)
  • Rapid breathing
  • Blood clots in the veins
  • Coughing

Many people also experience vena cava syndrome, which causes signs and symptoms such as difficulty breathing, swelling of the face and abdomen, and large veins in the chest.

Enlarged lymph nodes, a common feature of non-Hodgkin lymphoma, are typically seen only within the mediastinum.

How Is Primary Mediastinal Large B-Cell Lymphoma Diagnosed?

Diagnosing PMBCL involves many of the same tests used for diffuse large B-cell lymphoma.

Lymph Node Biopsy

A lymph node biopsy involves your doctor removing lymph node tissue from the mediastinum and examining it for the presence of cancer cells. The biopsy can be a surgical biopsy, which entails removing an entire lymph node from the body, or a needle biopsy, the removal of a portion of a lymph node.

Bone Marrow Testing

A doctor may obtain a bone marrow sample via bone marrow biopsy or aspiration. A bone marrow biopsy entails removing a small tissue sample from the bone marrow. A bone marrow aspiration involves taking a sample of liquid from the bone marrow.

Imaging Tests

CT scans, as well as scans combining positron emission tomography (PET) with CT — called PET-CT — are used to image the tissues within the body. CT or PET-CT can be used to image the characteristic mediastinal mass and pleural effusion (the accumulation of fluid around the lungs) that is common in PMBCL.

Blood Tests

Blood tests can be used to measure blood cell counts and levels of the lactate dehydrogenase protein, which may be higher than normal in a person with PMBCL.

Spinal Tap

Also called a lumbar puncture, a spinal tap involves using a needle to collect fluid from the spinal canal. This test may be performed to determine if PMBCL has spread to the nervous system.

Treatments for Primary Mediastinal Large B-Cell Lymphoma

Chemotherapy is the standard initial treatment approach for PMBCL. Combinations of chemotherapy drugs are often used for certain treatment regimens, generally with a drug called Rituxan (rituximab). Rituximab is an antibody that can target PMBCL cancer cells because it recognizes a specific molecule called CD20 on their surface.

Chemotherapy regimens commonly used to treat PMBCL include:

  • R-CHOP — Rituximab plus cyclophosphamide (sold as Cytoxan), doxorubicin (also known as hydroxydaunorubicin‌ and sold as Adriamycin), vincristine (formerly sold as Oncovin), and prednisone
  • Dose-adjusted EPOCH-R — Etoposide (also known as VP-16), prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab
  • MACOP‐B — Methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone, and Blenoxane (bleomycin)
  • VACOP‐B — Etoposide, doxorubicin, cyclophosphamide, vincristine, prednisone, and bleomycin

Chemotherapy — with or without the addition of rituximab — is often effective at treating PMBCL. However, some people have cancer that is refractory (doesn’t respond well to treatment) or that has relapsed (come back after a period of time). In such cases, a stem cell transplant may be recommended. A stem cell transplant allows a person undergoing treatment to tolerate high-dose chemotherapy by replacing the damaged cells of the bone marrow with healthy cells. A transplant can be done using stem cells from a healthy donor, in an allogeneic stem cell transplantation, or using stem cells from the person’s own body, in an autologous stem cell transplantation.

In some cases, radiotherapy (radiation therapy) may be used to shrink the mediastinal mass.

Treatments Under Investigation

People diagnosed with PMBCL may participate in clinical trials to identify potential new therapies. One new type of treatment that is currently being investigated is called chimeric antigen receptor (CAR) T-cell therapy. CAR T-cell therapy involves removing white blood cells called T cells from the person with cancer and then altering the cells to contain CARs on the surface. The modified cells are then put back into the person, where the cells can recognize molecules on the cancer cells and target the cancer cells for destruction.

Outlook for Primary Mediastinal Large B-Cell Lymphoma

The prognosis, or outlook, for PMBCL is generally favorable. The majority of people respond well to treatment and many are cured. The five-year survival rate is 80 percent to 90 percent. However, some people experience refractory or relapsed disease that is much more difficult to treat, leading to poor outcomes. Ongoing research to identify more effective treatments is important for improving the outcomes of people living with PMBCL.

Talk With Others Who Understand

MyLymphomaTeam is the social network for people with lymphoma and their loved ones. More than 8,500 members come together to ask questions, give advice, and share their experience of life with lymphoma with others who understand.

Do you or a loved one have primary mediastinal large B-cell lymphoma? Share your experience in the comments below, or start a conversation by posting on MyLymphomaTeam.

Posted on July 21, 2021
All updates must be accompanied by text or a picture.

We'd love to hear from you! Please share your name and email to post and read comments.

You'll also get the latest articles directly to your inbox.

This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Mark Levin, M.D. is a hematology and oncology specialist with over 37 years of experience in internal medicine. Review provided by VeriMed Healthcare Network. Learn more about him here.
Aminah Wali, Ph.D. received her doctorate in genetics and molecular biology from the University of North Carolina at Chapel Hill. Learn more about her here.

Related Articles

You are the key opinion leader of your disease — this was the sentiment shared by speakers in a p...

6 Ways To Advocate for Your Lymphoma Care According to Health Care Professionals

You are the key opinion leader of your disease — this was the sentiment shared by speakers in a p...
There are many risk factors, both inherited and environmental, believed to increase one’s risk of...

Is Diffuse Large B-Cell Lymphoma Inherited? Genetics and 8 Other Risk Factors

There are many risk factors, both inherited and environmental, believed to increase one’s risk of...
Double-hit lymphoma (DHL) is an aggressive cancer that develops from white blood cells called B l...

Double-Hit Lymphoma in DLBCL: Symptoms, Survival Rate, and More

Double-hit lymphoma (DHL) is an aggressive cancer that develops from white blood cells called B l...
B-cell lymphoma, also called B-cell non-Hodgkin lymphoma, is a type of blood cancer that develops...

12 Types of B-Cell Lymphoma and Subtypes of DLBCL

B-cell lymphoma, also called B-cell non-Hodgkin lymphoma, is a type of blood cancer that develops...
The majority of people with diffuse large B-cell lymphoma (DLBCL) will live with their condition ...

DLBCL Prognosis: Fear, Hope, and Understanding Survival Rates

The majority of people with diffuse large B-cell lymphoma (DLBCL) will live with their condition ...
Shouldn’t “cured” mean you’ll stay healthy? Although Hodgkin lymphoma (formerly called Hodgkin’s ...

7 Complications of Hodgkin Lymphoma

Shouldn’t “cured” mean you’ll stay healthy? Although Hodgkin lymphoma (formerly called Hodgkin’s ...

Recent Articles

MyHealthTeam does not provide health services, and if you need help, we’d strongly encourage you ...

Crisis Resources

MyHealthTeam does not provide health services, and if you need help, we’d strongly encourage you ...
No one knows what it’s like to have cancer unless they’ve actually been through it. Lou Lanza is ...

5 Suggestions To Ease Your Cancer Journey From a Lymphoma Advocate

No one knows what it’s like to have cancer unless they’ve actually been through it. Lou Lanza is ...
Roughly 50 percent to 60 percent of people relapse within the first two years of achieving remiss...

DLBCL Relapse Chances and Treatment Options

Roughly 50 percent to 60 percent of people relapse within the first two years of achieving remiss...
Diffuse large B-cell lymphoma (DLBCL) is staged based on which lymph nodes and organs are involve...

Early vs. Advanced DLBCL: How Are They Treated Differently?

Diffuse large B-cell lymphoma (DLBCL) is staged based on which lymph nodes and organs are involve...
After successful treatment for diffuse large B-cell lymphoma (DLBCL), you’ll enter complete remis...

DLBCL Relapse: 4 Symptoms To Watch For

After successful treatment for diffuse large B-cell lymphoma (DLBCL), you’ll enter complete remis...
When treatment for diffuse large B-cell lymphoma (DLBCL) successfully kills all of your detectabl...

Remission and Complete Response in DLBCL: How Long It Lasts and More

When treatment for diffuse large B-cell lymphoma (DLBCL) successfully kills all of your detectabl...
MyLymphomaTeam My lymphoma Team

Thank you for subscribing!

Become a member to get even more:

sign up for free

close