title: Primary cutaneous marginal zone B-cell lymphoma: a molecular and clinicopathologic study of 24 asian cases.
abstract: Cutaneous marginal zone B-cell lymphoma is a recently proposed entity and constitutes a cutaneous counterpart of extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). Borrelia burgdorferi infection has been suggested as a possible causative agent in European cutaneous cases of marginal zone B-cell lymphoma, whereas API2-MALT1 fusion and BCL10 mutation are highly associated with MALT lymphoma. Aberrant nuclear BCL10 expression may be closely correlated with API2-MALT1 fusion in gastric and pulmonary MALT lymphomas. We examined 24 Asian cases of cutaneous marginal zone B-cell lymphoma for B. burgdorferi involvement, API2-MALT1 fusion, BCL10 cellular expression, and BCL10 mutation. Neither Borrelia DNA nor API2-MALT1 fusion transcript was detected. Nuclear BCL10 expression was evident in tumor cells of 11 of 24 cases, although BCL10 mutation was found in one case only. Clinicopathologically, nuclear BCL10 was more frequently expressed in macroscopically nodular lesions than in plaques or papules (p = 0.0031). These data suggest that 1) B. burgdorferi infection may not play an important role in developing cutaneous marginal zone B-cell lymphoma in Asian cases, 2) neither API2-MALT1 fusion nor BCL10 mutation is closely associated with the pathogenesis, 3) aberrant nuclear BCL10 may frequently be expressed in the absence of these genetic abnormalities, and 4) nuclear BCL10 expression may be clinically important because it was observed in locally aggressive tumors.
authors.affiliation: Department of Pathology, Nagoya City Unviersity Medical School, Nagoya, Japan. hinagaki@med.nagoya-cu.ac.jp
authors.forename: Chunmei
authors.initials: C
authors.lastname: Li
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Inagaki
authors.forename: Tseng-Tong
authors.initials: TT
authors.lastname: Kuo
authors.forename: Sindy
authors.initials: S
authors.lastname: Hu
authors.forename: Mitsukuni
authors.initials: M
authors.lastname: Okabe
authors.forename: Tadaaki
authors.initials: T
authors.lastname: Eimoto
mesh_terms: D048868:Adaptor Proteins, Signal Transducing; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000074018:B-Cell CLL-Lymphoma 10 Protein; D025065:Borrelia burgdorferi / Q000235:genetics / Q000302:isolation & purification / Q000472:pathogenicity; D002352:Carrier Proteins / Q000032:analysis; D002467:Cell Nucleus / Q000737:chemistry / Q000473:pathology; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D016393:Lymphoma, B-Cell / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D015514:Oncogene Proteins, Fusion / Q000032:analysis / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012878:Skin Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*
chemical_list: C415943:API2-MALT1 fusion protein, human; D048868:Adaptor Proteins, Signal Transducing; D000074018:B-Cell CLL-Lymphoma 10 Protein; C118591:BCL10 protein, human; D002352:Carrier Proteins; D004273:DNA, Neoplasm; D015514:Oncogene Proteins, Fusion