PMID- 34605949 OWN - NLM STAT- MEDLINE DCOM- 20220113 LR - 20220113 IS - 1432-0584 (Electronic) IS - 0939-5555 (Linking) VI - 101 IP - 1 DP - 2022 Jan TI - Characterization of molecular genetics and clinicopathology in thymic MALT lymphoma. PG - 91-97 LID - 10.1007/s00277-021-04671-0 [doi] AB - Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma is a type of low-grade malignant B-cell lymphoma. The aim of this study was to investigate the clinicopathological characteristics of thymic MALT lymphoma. We analyzed the clinical, morphological, immunophenotypical, cytogenetic, and molecular characteristics of 11 cases of thymic MALT lymphoma. The relevant literature was also reviewed. The median age of the 11 patients was 50 (range: 33-60). There was a female predominance with a female-to-male ratio of 10:1. Three patients presented with Sjogren syndrome, autoimmune thrombocytopenia purpura, and type B1 thymoma, respectively. Microscopically, thymic MALT lymphoma was characterized by epithelium-lined cysts that were surrounded by small lymphocytes, centrocyte-like cells, and monocytoid B-cells. Plasmacytic differentiation was observed in two cases. The tumor cells expressed CD20, CD79alpha, and BCL2. Clonal immunoglobulin genes were detected in all 8 examined cases. Fluorescence in situ hybridization (FISH) for 18q21 was performed in 7 cases, and no translocations involving 18q21 were found. Targeted gene sequencing was performed in five cases with available DNA samples, and TNFAIP3, CARD11, IGLL5, and CCND3 mutations were identified. Thymic MALT lymphoma is a rare type of B cell malignancy with a female predominance and excellent clinical outcomes. Molecular aberrations involving the NF-kappaB pathway are frequent in thymic MALT lymphoma, suggesting that dysregulation of the NF-kappaB pathway is an important mechanism underlying the pathogenesis of thymic MALT lymphoma. CI - (c) 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. FAU - Wang, Xiaojun AU - Wang X AD - Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 Panjiayuan Nan Li, Chaoyang District, Beijing, 100021, China. FAU - Miao, Yi AU - Miao Y AD - Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, Nanjing, China. FAU - Cao, Zheng AU - Cao Z AD - Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 Panjiayuan Nan Li, Chaoyang District, Beijing, 100021, China. FAU - Zheng, Shan AU - Zheng S AD - Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 Panjiayuan Nan Li, Chaoyang District, Beijing, 100021, China. FAU - Xue, Xuemin AU - Xue X AD - Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 Panjiayuan Nan Li, Chaoyang District, Beijing, 100021, China. FAU - Feng, Xiaoli AU - Feng X AD - Department of Pathology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 Panjiayuan Nan Li, Chaoyang District, Beijing, 100021, China. fengxl@hotmail.com. LA - eng PT - Journal Article DEP - 20211004 PL - Germany TA - Ann Hematol JT - Annals of hematology JID - 9107334 SB - IM MH - Adult MH - Female MH - Gene Rearrangement MH - High-Throughput Nucleotide Sequencing MH - Humans MH - In Situ Hybridization, Fluorescence MH - Lymphoma, B-Cell, Marginal Zone/*genetics/pathology MH - Male MH - Middle Aged MH - Mutation MH - Thymus Neoplasms/*genetics/pathology MH - Translocation, Genetic OTO - NOTNLM OT - Autoimmune disease OT - Clonal gene rearrangement OT - Mucosa-associated lymphoid tissue OT - Mucosa-associated lymphoid tissue lymphoma OT - Thymic EDAT- 2021/10/05 06:00 MHDA- 2022/01/14 06:00 CRDT- 2021/10/04 12:25 PHST- 2021/06/09 00:00 [received] PHST- 2021/09/17 00:00 [accepted] PHST- 2021/10/05 06:00 [pubmed] PHST- 2022/01/14 06:00 [medline] PHST- 2021/10/04 12:25 [entrez] AID - 10.1007/s00277-021-04671-0 [pii] AID - 10.1007/s00277-021-04671-0 [doi] PST - ppublish SO - Ann Hematol. 2022 Jan;101(1):91-97. doi: 10.1007/s00277-021-04671-0. Epub 2021 Oct 4.