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Characterization of molecular genetics and clinicopathology in thymic MALT lymphoma

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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 Sjögren 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, CD79α, 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-κB pathway are frequent in thymic MALT lymphoma, suggesting that dysregulation of the NF-κB pathway is an important mechanism underlying the pathogenesis of thymic MALT lymphoma.

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  1. Swerdlow SH, Campo E, Harris NL, et al (2017) WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. International Agency for Research on Cancer (IARC), Lyon, France

  2. Raderer M, Wohrer S, Streubel B et al (2006) Assessment of disease dissemination in gastric compared with extragastric mucosa-associated lymphoid tissue lymphoma using extensive staging: a single-center experience [J]. J Clin Oncol 24(19):3136–3141

    Article  Google Scholar 

  3. Raderer M, Kiesewetter B, Ferreri AJ (2016) Clinicopathologic characteristics and treatment of marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)[J]. CA Cancer J Clin 66(2):153–171

    Article  Google Scholar 

  4. Kominato S, Nakayama T, Sato F et al (2012) Characterization of chromosomal aberrations in thymic MALT lymphoma [J]. Pathol Int 62(2):93–98

    Article  CAS  Google Scholar 

  5. Jung H, Yoo HY, Lee SH et al (2017) The mutational landscape of ocular marginal zone lymphoma identifies frequent alterations in TNFAIP3 followed by mutations in TBL1XR1 and CREBBP[J]. Oncotarget 8(10):17038–17049

    Article  Google Scholar 

  6. Hyeon J, Lee B, Shin SH et al (2018) Targeted deep sequencing of gastric marginal zone lymphoma identified alterations of TRAF3 and TNFAIP3 that were mutually exclusive for MALT1 rearrangement[J]. Mod Pathol 31(9):1418–1428

    Article  CAS  Google Scholar 

  7. Go H, Cho HJ, Paik JH et al (2011) Thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue: a clinicopathological and genetic analysis of six cases[J]. Leuk Lymphoma 52(12):2276–2283

    Article  CAS  Google Scholar 

  8. Wang L, Ying J, Xue L et al (2013) Clonality analysis in Castleman’s disease and its application in differential diagnosis. J Diag Pathol 20(7):397–400

    Google Scholar 

  9. Dubois S, Viailly PJ, Mareschal S et al (2016) Next-generation sequencing in diffuse large b-cell lymphoma highlights molecular divergence and therapeutic opportunities: a LYSA study[J]. Clin Cancer Res 22(12):2919–2928

    Article  CAS  Google Scholar 

  10. Talwalkar SS, Valbuena JR, Abruzzo LV et al (2006) MALT1 gene rearrangements and NF-kappaB activation involving p65 and p50 are absent or rare in primary MALT lymphomas of the breast[J]. Mod Pathol 19(11):1402–1408

    Article  CAS  Google Scholar 

  11. Inagaki H, Chan JK, Ng JW et al (2002) Primary thymic extranodal marginal-zone B-cell lymphoma of mucosa-associated lymphoid tissue type exhibits distinctive clinicopathological and molecular features[J]. Am J Pathol 160(4):1435–1443

    Article  Google Scholar 

  12. Zaharie F, Pop LA, Petrushev B et al (2019) Next-generation sequencing-based characterization of the invasion by anatomical contiguity in a primary osseous diffuse large B-cell lymphoma. Correlation between the genetic profile of the malignancy and the clinical outcome of the patient[J]. Histol Histopathol 34(6):663–670

    PubMed  CAS  Google Scholar 

  13. Karvounis E, Kappas I, Angelousi A et al (2020) Mucosa-associated lymphoid tissue lymphoma of the thyroid gland: a systematic review of the literature[J]. Eur Thyroid J 9(1):11–18

    Article  Google Scholar 

  14. Du MQ (2016) MALT lymphoma: a paradigm of NF-kappaB dysregulation[J]. Semin Cancer Biol 39:49–60

    Article  CAS  Google Scholar 

  15. Du MQ (2017) MALT lymphoma: genetic abnormalities, immunological stimulation and molecular mechanism[J]. Best Pract Res Clin Haematol 30(1–2):13–23

    Article  Google Scholar 

  16. Vela V, Juskevicius D, Gerlach MM et al (2020) High throughput sequencing reveals high specificity of TNFAIP3 mutations in ocular adnexal marginal zone B-cell lymphomas[J]. Hematol Oncol 38(3):284–292

    Article  CAS  Google Scholar 

  17. Moody S, Thompson JS, Chuang SS et al (2018) Novel GPR34 and CCR6 mutation and distinct genetic profiles in MALT lymphomas of different sites[J]. Haematologica 103(8):1329–1336

    Article  CAS  Google Scholar 

  18. Nocturne G, Boudaoud S, Miceli-Richard C et al (2013) Germline and somatic genetic variations of TNFAIP3 in lymphoma complicating primary Sjogren’s syndrome[J]. Blood 122(25):4068–4076

    Article  CAS  Google Scholar 

  19. Paul J, Soujon M, Wengner AM et al (2017) Simultaneous inhibition of PI3Kdelta and PI3Kalpha induces ABC-DLBCL regression by blocking BCR-dependent and -independent activation of NF-kappaB and AKT[J]. Cancer Cell 31(1):64–78

    Article  CAS  Google Scholar 

  20. Momoi A, Nagai K, Isahai N et al (2016) Thymic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue with 8q24 abnormality[J]. Intern Med 55(7):799–803

    Article  CAS  Google Scholar 

  21. Xing W, Guanjun Z, Hongling W, Huan Z et al (2019) Clinicopathological features of primary thymic extranodal marginal zone B cell lymphomaof mucosa associated lymphoid tissue [J]. Chin Clin Oncol 48(4):315–317

    Google Scholar 

  22. Sugimoto KJ, Asahina M, Shimada A et al (2014) IgG3 subclass-positive primary thymic MALT lymphoma without trisomy 3 and trisomy 18: report of a case and review of literature[J]. Int J Clin Exp Pathol 7(12):8980–8987

    PubMed  PubMed Central  Google Scholar 

  23. You S, Sun JS, Park KJ et al (2020) Amyloid deposition in thymic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue in a patient with myasthenia gravis: a case report[J]. Thoracic Cancer 11(3):781–784

    Article  Google Scholar 

  24. Song W, Wang W, Zhou N (2011) Clinicopathological analysis of thymic mucosa-associated lymphoid tissue lymphoma[J]. Lin Chuang Zhong Liu Xue Za Zhi 16(9):829–832

  25. Sun L, Shi H, Wei L (2012) Clinicopathologic features of primary thymic extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue type. Zhonghua Bing Li Xue Za Zhi 41(4):234–238

  26. Wang Z, Li H, Cao Z et al (2016) Clinicopathologic study of primary thymic extranodal marginal zone lymphoma of mucosa associated lymphoid tissue and lymphoepithelial sialadenitis-like thymic hyperplasia[J]. J Clin Exp Pathol 32(12):1338–42

    CAS  Google Scholar 

  27. Shuai H, Li Z, Lang Z (2017) Two cases: mucosa-associated lymphoid tissue lymphoma of the thymus[J]. Shi Yong Fang She Xue Za Zhi 33(6):676–677

  28. Chang X, Chen J, Jiang Y et al (2012) Mucosa-associated lymphoid tissue lymphoma of the thymus: a study of 2 cases[J]. Xie He Yi Xue Za Zhi 3(01):41–46

  29. Xuejiao S, Wenwen S, Na Z et al (2018) Two eases of primary Sjogren’s syndrome complicated with thymus mucosa-associated lymphoid tissue lymphoma[J]. Chin J Gen Pract 17(11):936–937

    Google Scholar 

  30. Xu DM, Wang L, Zhu HY et al (2020) Primary thymic mucosa-associated lymphoid tissue lymphoma: 7 clinical cases report and a review of the literature[J]. Zhonghua Xue Ye Xue Za Zhi 41(1):54–8

    PubMed  CAS  Google Scholar 

  31. Sunada K, Hasegawa Y, Kodama T et al (2007) Thymic and pulmonary mucosa-associated lymphoid tissue lymphomas in a patient with Sjogren’s syndrome and literature review[J]. Respirology 12(1):144–147

    Article  Google Scholar 

  32. Kurabayashi A, Iguchi M, Matsumoto M et al (2010) Thymic mucosa-associated lymphoid tissue lymphoma with immunoglobulin-storing histiocytosis in Sjogren’s syndrome[J]. Pathol Int 60(2):125–130

    Article  Google Scholar 

  33. Royer B, Cazals-Hatem D, Sibilia J et al (1997) Lymphomas in patients with Sjogren’s syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses[J]. Blood 90(2):766–775

    Article  CAS  Google Scholar 

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Correspondence to Xiaoli Feng.

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This study was approved by the institutional review boards of the National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College (NCC3092).

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Wang, X., Miao, Y., Cao, Z. et al. Characterization of molecular genetics and clinicopathology in thymic MALT lymphoma. Ann Hematol 101, 91–97 (2022).

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