This study analysed incidence, patient outcome, immunophenotype and prognostic factors of histological transformation (HT) from extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) to diffuse large B-cell lymphoma (DLBCL) in 467 patients (median age, 61 years). The primary sites of MALT lymphoma were the stomach (43%), ocular adnexa (25%), lung (8%), systemic (8%) and other tissues (16%). HT occurred in 8% of MALT lymphomas. Risk of HT by 15 years was 5%: 4% in limited-stage diseases (n = 385) and 16% in advanced-stage diseases (n = 56) (P = 0·02). The median time to HT was 48 months (range, 4-139). Five-year progression-free survival (PFS) and overall survival (OS) rates after HT were 80% and 94%, respectively. Immunohistochemical results of DLBCL were as follows: germinal centre B-cell (GCB)/non-GCB, 37%/63%; CD10, 9%; BCL6, 59%; MUM1, 38%; MYC, 42%; BCL2, 35%; Ki67 ≥ 90%, 23%; and CD5, 3%. The majority (75%, 9/12) of GCB-type DLBCLs exhibited CD10(-) , BCL6(+) and MUM1(-) immunophenotypes; the remainder had CD10(+) immunophenotypes. Multivariate analysis revealed that only advanced stage at HT was a significant adverse factor for PFS (P = 0·037). Thus, overall risk of HT was low and prognosis after HT was favourable; however, in advanced-stage cases, risk of HT was relatively high and prognosis was unfavourable.
We analyzed the distribution of tumors and lymphomas of the anterior mediastinum diagnosed between 1997 and 2016 at the National Cancer Center Hospital, Japan. The median age of 283 patients with anterior mediastinal tumors was 48 (range 6-84) years, and 143 (51%) were male. The incidence of tumors was as follows: thymoma, 34%; thymic carcinoma, 16%; primary mediastinal large B-cell lymphoma (PMBL), 13%; germ cell tumors, 10%; classical Hodgkin lymphoma (CHL), 9%; thymic cyst, 7%; metastatic tumors, 3%; T lymphoblastic leukemia/lymphoma (T-LBL), 2%; other lymphomas, 3%; and others, 3%. Of the newly diagnosed lymphomas in 60 patients, PMBL (46%) was the most frequent, followed by CHL (32%), T-LBL (12%), mucosa-associated lymphoid tissue lymphoma (3%), and other lymphomas (7%). These findings suggest a recent increase in PMBL in Japan. The frequency and subtype of lymphoma differ with age and sex. In female patients ≤40 years old, 58% of the anterior mediastinal tumors were PMBL (39%) or CHL (19%). Germ cell tumors were the most frequent in male patients ≤40 years old, followed by CHL (21%), PMBL (17%), and T-LBL (10%). This distribution may serve as a reference for routine histologic diagnosis of lymphomas in the anterior mediastinum in Japan.
Aims:The Lugano classification is a novel staging system for lymphomas established in 2014. In this staging system, 18 F-fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) was formally incorporated into standard staging for FDG-avid lymphomas, including Hodgkin lymphoma (HL). We retrospectively analyzed patients with HL who were newly diagnosed and treated at our institution to evaluate the utility of the Lugano classification compared to the Ann Arbor classification in Japanese patients with this type of lymphoma.
Methods:Patients newly diagnosed with HL between 2005 and 2011 were identified through our departmental database. Data from neck-chest-abdomen-pelvis CT scans, BM examinations, and FDG-PET/CT that were routinely performed for staging at our clinical practice were retrieved and analyzed.Results: Fifty-four patients with a median age of 35.5 years (range: 15-78 years) were investigated in this retrospective study. The Lugano stage matched the Ann Arbor stage in 46 patients (85%).Six patients (11%) were upstaged while two (4%) were downstaged per the Lugano classification.Four patients (7%) were converted from early stage to advanced stage, but none was converted in the reverse. Among 11 patients (20%) with PET-positive bone/bone marrow lesions, only one (2%) exhibited biopsy-proven bone marrow involvement of HL cells.
Conclusion:Our data revealed a high concordance rate between the Lugano and Ann Arbor staging system in Japanese patients with HL. Because of its low diagnostic value, routine bone marrow examination might be unnecessary for staging when FDG-PET/CT is used as a substitute.
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