title: Indolent cutaneous B-cell lymphoma: diagnosis and treatment 2012.
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.
authors.affiliation: Divisions of Pathological Anatomy, Department of Critical Care Medicine and Surgery, University of Florence Medical School, Florence, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Santucci
authors.forename: V
authors.initials: V
authors.lastname: Grandi
authors.forename: V
authors.initials: V
authors.lastname: Maio
authors.forename: C
authors.initials: C
authors.lastname: Delfino
authors.forename: R
authors.initials: R
authors.lastname: Alterini
authors.forename: N
authors.initials: N
authors.lastname: Pimpinelli
mesh_terms: D006801:Humans; D016393:Lymphoma, B-Cell / Q000175:diagnosis* / Q000188:drug therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000188:drug therapy*