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Indolent cutaneous B-cell lymphoma: diagnosis and treatment 2012
1Divisions of Pathological Anatomy, Department of Critical Care Medicine and Surgery, University of Florence Medical School, Florence, Italy.
Insights
Primary cutaneous B-cell lymphomas (CBCL), including marginal zone lymphoma (MZL) and follicle center lymphoma (FCL), exhibit indolent behavior and excellent prognoses. Differentiating these CBCL subtypes relies on distinct clinical and histological features.
Area of Science:
- Dermatology
- Hematology
- Oncology
Background:
- Primary cutaneous B-cell lymphomas (CBCL) are a heterogeneous group of lymphoid neoplasms.
- Two principal subtypes, marginal zone lymphoma (MZL) and follicle center lymphoma (FCL), are recognized.
- These lymphomas typically follow an indolent course with a favorable prognosis.
Purpose of the Study:
- To delineate the clinico-pathologic characteristics of primary cutaneous B-cell lymphomas (CBCL).
- To highlight the diagnostic distinctions between marginal zone lymphoma (MZL) and follicle center lymphoma (FCL).
- To emphasize the indolent nature and excellent prognosis associated with these CBCL subtypes.
Main Methods:
- Comparative clinico-pathologic analysis of MZL and FCL.
- Histopathological examination focusing on cellular phenotype and lesion morphology.
- Evaluation of clinical presentation, including site predilection and lesion distribution.
Main Results:
- MZL and FCL demonstrate indolent behavior with a low risk of extracutaneous spread and high survival rates.
- Clinical differences exist in site predilection and frequency of multiple lesions.
- Histological features, including immunophenotype (e.g., CD5, CD10, bcl2, bcl6) and lesion architecture, aid in differential diagnosis.
Conclusions:
- Primary cutaneous B-cell lymphomas (CBCL), specifically MZL and FCL, are generally indolent with excellent prognoses.
- Accurate differentiation between MZL and FCL is achievable through careful assessment of clinical and histological parameters.
- Radiotherapy is a highly effective treatment modality for these conditions.
Abstract:
Among primary cutaneous B-cell lymphomas (CBCL), two main clinico-pathologic entities are recognized, i.e. marginal zone lymphoma (MZL), otherwise defined as extranodal MZL, MALT (Mucosa-Associated Lymphoid Tissue) type, and follicle center lymphoma (FCL). They are mostly characterized by indolent course (very limited risk of extracutaneous spread), very good response to non-aggressive treatment (radiotherapy is the gold standard), and excellent prognosis (>90% 5-year survival overall). The clinical presentation of MZL and FCL slightly differ concerning site predilection (trunk and upper limbs in the former, head&neck and trunk in the latter) and frequency of cases with multiple, non-contiguous lesions (higher in MZL). Histologically, MZL and FCL share the multiphasic evolution of lesions, while some distinctive features are clues to diagnosis and differential diagnosis: CD5-/CD10-/bcl2+ phenotype of neoplastic cells, "colonization" of reactive lymphoid follicles by neoplastic cells, lymphoplasmacytoid and plasma cells at the periphery of nodular infiltrates in MZL; CD5-, CD10 +/-, bcl6+, MUM-1 neg, FOX-P1 neg, IRF4 neg, IgM neg phenotype of neoplastic cells (centrocytes), and neoplastic follicles (in early lesions) in FCL.
