Primary Cutaneous B-cell Lymphoma

Expert Review by

Steven M. Horwitz, M.D., Assistant Attending, Lymphoma Service, Memorial Sloan-Kettering Cancer Center

My Personal Journey with Cutaneous Lymphoma
Gary Robinson shares his experience living with cutaneous lymphoma and recommendations for those newly diagnosed.

Gary Robinson
Salt Lake City, UT

Definition
What is Primary Cutaneous B-Cell Lymphoma?

Lymphomas that grow outside the nodal system, also called extranodal lymphomas, are tumors that occur in organs or tissues outside the lymphatic system. When extranodal lymphomas originate in the skin and there is no evidence of systemic or extracutaneous disease, they are called primary cutaneous lymphomas and primary cutaneous B-cell lymphomas (CBCLs) when B-cells are involved. Skin is the second most common site of extranodal involvement after the gastrointestinal tract, making up 18 percent of all extranodal lymphomas, and 5 percent of all non-Hodgkin lymphomas. The majority of primary cutaneous lymphomas are of the T-cell type, with CBCL comprising 20 percent to 25 percent of all primary cutaneous lymphomas.

Primary cutaneous B-cell lymphomas are nearly always indolent or slow growing. They may appear on the skin as a reddish rash, lump or nodule and, because they tend to develop in the dermis, or second layer of the skin, may have a slightly raised and smooth appearance. They may recur on the skin but rarely will a primary CBCL develop into a systemic lymphoma.

Who Gets CBCL?

The disease affects men and women equally and can affect any age group. Like all lymphomas, CBCL is an acquired disease and there are no known risk factors. The disease is not inherited or contagious.

What is CBCL and what is the prognosis?

EXPERT PRESENTERS

Stuart Lessin, MD, Medical Director, KGL Skin Study Center

Lauren Pinter-Brown, MD, FACP, Clinical Professor of Medicine, Geffin School of Medicine at UCLA

Christiane Querfeld, MD, Director, Cutaneous Lymphoma Program, City of Hope Comprehensive Cancer Center

Variants
What are the different types of cutaneous B-cell lymphoma?

Recently, the World Health Organization and European Organization for Research and Treatment of Cancer Classification reached a consensus on a classification system for CBCL and determined that there are three main types of primary cutaneous B-cell lymphomas:

  • Primary cutaneous follicle center lymphoma
  • Primary cutaneous marginal zone B-cell lymphoma
  • Primary cutaneous diffuse large cell lymphoma, leg type
Cutaneous B-Cell Lymphoma: What are the variants and how are they treated?

EXPERT PRESENTERS

Christiane Querfeld, MD, Director, Cutaneous Lymphoma Program, City of Hope Comprehensive Cancer Center

Stuart Lessin, MD, Medical Director, KGL Skin Study Center

Lauren Pinter-Brown, MD, FACP, Clinical Professor of Medicine, Geffin School of Medicine at UCLA

Diagnosis
How CBCL Is Diagnosed and Staged?

A biopsy of the tumor tissue is needed to render a diagnosis and determine CBCL classification. Once a diagnosis is made, CBCL patients are either referred to an oncologist or a dermatologist specializing in CBCL for clinical staging to eliminate the possibility that the disease originated outside the skin. A comprehensive CBCL staging evaluation includes:

  • A physical examination of the entire skin and lymphatic system
  • Laboratory tests, including complete blood and serum counts and serum lactate dehydrogenase (LDH) measurements
  • Imaging tests, including a CT (computerized axial tomography) or PET (positron emission tomography) scan of the chest, abdomen and pelvis
  • A bone marrow biopsy may be recommended for more aggressive subtypes of CBCL. A bone marrow biopsy may not be necessary for patients with cutaneous follicle center lymphoma or marginal zone lymphoma if systemic staging is negative
Why are so many skin biopsies required?

EXPERT PRESENTERS:

Stuart Lessin, MD, Medical Director, KGL Skin Study Center, Broomall, PA

Pierluigi Porcu, MD, Director, Hematologic Malignancies and Hematopoietic Stem Cell Transplantation Division, Sidney Kimmel Cancer Center, Thomas Jefferson University Hospital (At time of publication: Associate Professor of Internal Medicine, Division of Hematology-Oncology, Ohio State University Comprehensive Cancer Center)  

Larisa Geskin, MD, Director, Cutaneous Oncology, Columbia University Medical Center (At time of publication: Associate Professor of Dermatology, University of Pittsburgh)

Treatment
How is Cutaneous B-cell Lymphoma Treated?
  • Primary cutaneous follicle center lymphoma and cutaneous marginal zone lymphoma are among the most slow growing or indolent lymphomas and, depending on the number of lesions present, may be treated locally with either radiation or surgery.
  • Topical steroid cream or topical chemotherapy may also be used.
  • Sometimes no treatment is needed. Most often these slow-growing lymphomas recur over time, usually only on the skin. As many as 50 percent of people with single lesions that get radiation or excision will never get another lesion.

Indolent CBCLs rarely transform into more aggressive types of lymphoma. For diseases that do transform, become widespread or in rare cases when they become systemic, a variety of chemotherapy regimens may be used. Other therapies such as the monoclonal antibody Rituxan (rituximab), either alone or in combination with chemotherapy, and interferon may also be recommended.

Learn more about treatment options.

Treating More Aggressive Types of CBCL

There is no known best treatment strategy for the more aggressive type of CBCL, primary cutaneous diffuse large-B cell lymphoma-leg type. The following treatments have had success:

Radiation

  • For single tumors, radiation is often effective as are combinations of chemotherapy or radiation and chemotherapy given sequentially.
  • Palliative 2 Gy x 2fractions EBT or brachytherapy

All of these treatments usually put the disease in remission, although relapse is common. Research is underway to test the most effective strategies for treating cutaneous B-cell lymphomas.

Learn More

Skin-Directed Therapies

Individuals with disease limited to the skin (stages I and II) can often achieve a good response with one of the readily available skin-directed therapies. Learn more about skin-directed therapies.

Systemic Therapies

Individuals who have only partial or short responses to skin-directed therapies, or more advanced stages of disease, may benefit from systemic therapy. Learn more about available systemic therapies.

Clinical Trials: A Pathway to Treatment

Physician & Patient
Clinical trials are research studies that investigate new or experimental treatments in patients living with a specific disease. Clinical trials empower patients to play an active role in their own care and open them up to access new treatments before they are widely available.
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