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Primary cutaneous B-cell lymphoma: review and current concepts
T L Pandolfino1, R S Siegel, T M Kuzel
1Department of Medicine, Section of Hematology and Oncology, Northwestern University Medical School, Chicago, IL 60611, USA.
Summary
Primary cutaneous B-cell lymphoma (PCBCL) is a skin-originating lymphoma with a generally good prognosis and high survival rates. Treatment strategies for PCBCL should be tailored to the specific subtype and patient factors, with radiation therapy being a primary option.
Area of Science:
- Hematology
- Dermatology
- Oncology
Background:
- Primary cutaneous B-cell lymphoma (PCBCL) is a distinct clinical entity with increasing diagnoses due to advanced immunophenotyping.
- Confusion exists regarding PCBCL classification, treatment, and prognosis.
- This review synthesizes current data on PCBCL diagnosis, prognosis, and management.
Purpose of the Study:
- To review and analyze available data on Primary Cutaneous B-cell Lymphoma (PCBCL).
- To provide clinicians with a concise summary of PCBCL diagnosis, prognosis, and treatment.
- To clarify the classification and management of PCBCL.
Main Methods:
- A thorough review of the medical literature on PCBCL was conducted.
- Focus included classification, prognosis, and treatment trials for PCBCL.
- Literature analysis centered on diagnosis, staging, and therapeutic outcomes.
Main Results:
- PCBCL originates in the skin with no evidence of extracutaneous disease at presentation.
- The European Organization for Research and Treatment of Cancer classification is the current standard for PCBCL subtypes.
- PCBCL is typically indolent with a good prognosis (89-96% 5-year survival), except for large B-cell lymphoma of the leg (58% 5-year survival).
- Local recurrences are common (25-68%), but dissemination is rare.
Conclusions:
- PCBCL is generally an indolent lymphoma with a favorable prognosis.
- Radiation therapy is highly effective for PCBCL; chemotherapy is reserved for advanced cases.
- Treatment decisions for PCBCL should consider subtype, extent of disease, and patient condition.