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Stage IV intramucosal gastric marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type

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Abstract

A 45-year-old woman with no symptoms underwent upper gastrointestinal endoscopy. A discolored area was noted at the greater curvature of the gastric upper body. Endoscopic ultrasonography demonstrated thickening of the second sonographic layer indicating that the depth of invasion was confined to the mucosa. A urea breath test and anti-Helicobacter pylori antibody test were negative. A computed tomography scan showed a consolidation at the right lung. Gastric biopsy and transbronchial lung biopsy (TBLB) demonstrated a monotonous proliferation of atypical small lymphocytes. A diagnosis of gastric marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma) was made. The clinical stage was stage IV. A genetic analysis showed rearrangement of the joining region of the immunoglobulin heavy chain gene and identical clones in both lesions. An API2MALT1 fusion gene was detected in the gastric lesion. After H. pylori eradication treatment, combination treatment with rituximab plus CHOP (R-CHOP) was performed; 6 months later an endoscopy revealed complete disappearance of the lesion. Multiple gastric biopsies showed no infiltrating atypical lymphocytes. Similarly, the lesion in the lung showed complete remission (CR) on CT and TBLB. This report shows that a gastric MALT lymphoma located in the mucosa and disseminated to the lung maintained CR by R-CHOP.

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References

  1. Wotherspoon AC, Doglioni C, Diss TC, Pan L, Moschini A, de Boni M, et al. Regression of primary low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type after eradication of Helicobacter pylori. Lancet. 1993;342:575–7.

    Article  PubMed  CAS  Google Scholar 

  2. Ruskoné-Fourmestraux A, Fischbach W, Aleman BM, Boot H, Du MQ, Megraud F, et al. EGILS consensus report. Gastric extranodal marginal zone B-cell lymphoma of MALT. Gut. 2011;60:747–58.

    Google Scholar 

  3. Raderer M, Vorbeck F, Formanek M, Österreicher C, Valencak J, Penz M, et al. Importance of extensive staging in patients with mucosa-associated lymphoid tissue (MALT)-type lymphoma. Br J Cancer. 2000;83:454–7.

    Article  PubMed  CAS  Google Scholar 

  4. Thieblemont C, Berger F, Dumontet C, Moullet I, Bouafia F, Felman P, et al. Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood. 2000;95:802–6.

    PubMed  CAS  Google Scholar 

  5. Raderer M, Wöhrer S, Streubel B, Troch M, Turetschek K, Jager U, et al. Assessment of disease dissemination in gastric compared with extragastric mucosa-associated lymphoid tissue lymphoma using extensive staging: a single-center experience. J Clin Oncol. 2006;24:3136–41.

    Article  PubMed  Google Scholar 

  6. Krol AD, Hermans J, Kramer MH, Kluin PM, Kluin-Nelemans HC, Blok P, et al. Gastric lymphomas compared with lymph node lymphomas in a population-based registry differ in stage distribution and dissemination patterns but not in patient survival. Cancer. 1997;79:390–7.

    Article  PubMed  CAS  Google Scholar 

  7. Nakamura T, Seto M, Tajika M, Kawai H, Yokoi T, Yatabe Y, et al. Clinical features and prognosis of gastric MALT lymphoma with special reference to responsiveness to H. pylori eradication and API2 MALT1 status. Am J Gastroenterol. 2008;103:62–70.

    Article  PubMed  CAS  Google Scholar 

  8. Du MQ, Peng HZ, Dogan A, Diss TC, Liu H, Pan LX, et al. Preferential dissemination of B-cell gastric mucosa-associated lymphoid tissue (MALT) lymphoma to the splenic marginal zone. Blood. 1997;90:4071–7.

    PubMed  CAS  Google Scholar 

  9. Kuo SH, Cheng AL, Lin CW, Hsu CH, Wu MS, Yeh KH, et al. t(11;18)(q21;q21) translocation as predictive marker for non-responsiveness to salvage thalidomide therapy in patients with marginal zone B-cell lymphoma with gastric involvement. Cancer Chemother Pharmacol. 2011;68:1387–95.

    Article  PubMed  CAS  Google Scholar 

  10. Ye H, Liu H, Raderer M, Chott A, Ruskone-Fourmestraux A, Wotherspoon A, et al. High incidence of t(11;18)(q21;q21) in Helicobacter pylori-negative gastric MALT lymphoma. Blood. 2003;101:2547–50.

    Article  PubMed  CAS  Google Scholar 

  11. Troch M, Jonak C, Müllauer L, Püspök A, Formanek M, Hauff W, et al. A phase II study of bortezomib in patients with MALT lymphoma. Haematologica. 2009;94:738–42.

    Article  PubMed  CAS  Google Scholar 

  12. Nakamura S, Ye H, Bacon CM, Goatly A, Liu H, Banham AH, et al. Clinical impact of genetic aberrations in gastric MALT lymphoma: a comprehensive analysis using interphase fluorescence in situ hybridisation. Gut. 2007;56:1358–63.

    Article  PubMed  Google Scholar 

  13. Dogan A, Du M, Koulis A, Briskin MJ, Isaacson PG. Expression of lymphocyte homing receptors and vascular addressing in low-grade gastric B-cell lymphomas of mucosa-associated lymphoid tissue. Am J Pathol. 1997;151:1361–9.

    PubMed  CAS  Google Scholar 

  14. Graziadei G, Pruneri G, Carboni N, Luminari S, La Targia ML, Neri A, et al. Low-grade MALT lymphoma involving multiple mucosal sites and bone marrow. Ann Hematol. 1998;76:81–3.

    Article  PubMed  CAS  Google Scholar 

  15. Iwano M, Okazaki K, Uchida K, Nakase H, Ohana M, Matsushima Y, et al. Characteristics of gastric B-cell lymphoma of mucosa-associated lymphoid tissue type involving multiple organs. J Gastroenterol. 2004;39:739–46.

    Article  PubMed  Google Scholar 

  16. Remstein ED, Kurtin PJ, James CD, Wang XY, Meyer RG, Dewald GW. Mucosa-associated lymphoid tissue lymphomas with t(11;18)(q21;q21) and mucosa-associated lymphoid tissue lymphomas with aneuploidy develop along different pathogenetic pathways. Am J Pathol. 2002;161:63–7.

    Article  PubMed  Google Scholar 

  17. Streubel B, Ye H, Du MQ, Isaacson PG, Chott A, Raderer M. Translocation t(11;18)(q21;q21) is not predictive of response to chemotherapy with 2CdA in patients with gastric MALT lymphoma. Oncology. 2004;66:476–80.

    Article  PubMed  CAS  Google Scholar 

  18. Raderer M, Streubel B, Woehrer S, Puespoek A, Jaeger U, Formanek M, et al. High relapse rate in patients with MALT lymphoma warrants lifelong follow-up. Clin Cancer Res. 2005;11:3349–52.

    Article  PubMed  Google Scholar 

  19. Blazquez M, Haioun C, Chaumette MT, Gaulard P, Reyes F, Soulé JC, et al. Low grade B cell mucosa associated lymphoid tissue lymphoma of the stomach: clinical and endoscopic features, treatment, and outcome. Gut. 1992;33:1621–5.

    Article  PubMed  CAS  Google Scholar 

  20. Konoplev S, Lin P, Qiu X, Medeiros LJ, Yin CC. Clonal relationship of extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue involving different sites. Am J Clin Pathol. 2010;134:112–8.

    Article  PubMed  CAS  Google Scholar 

  21. Ichihara E, Tabata M, Takigawa N, Sato Y, Kondo E, Aoe M, et al. Synchronous pulmonary MALT lymphoma and pulmonary adenocarcinoma after metachronous gastric MALT lymphoma and gastric adenocarcinoma. J Thorac Oncol. 2008;3:1362–3.

    Article  PubMed  Google Scholar 

  22. Kawamata N, Miki T, Fukuda T, Suzuki K, Sumi Y, Ohdama S, et al. Determination of a common clonal origin of gastric and pulmonary mucosa-associated lymphoid tissue lymphomas presenting five years apart. Intern Med. 1995;34:220–3.

    Article  PubMed  CAS  Google Scholar 

  23. Ye H, Liu H, Attygalle A, Wotherspoon AC, Nicholson AG, Charlotte F, et al. Variable frequencies of t(11;18)(q21;q21) in MALT lymphomas of different sites: significant association with CagA strains of H pylori in gastric MALT lymphoma. Blood. 2003;102:1012–8.

    Article  PubMed  CAS  Google Scholar 

  24. Streubel B, Simonitsch-Klupp I, Müllauer L, Lamprecht A, Huber D, Siebert R, et al. Variable frequencies of MALT lymphoma-associated genetic aberrations in MALT lymphomas of different sites. Leukemia. 2004;18:1722–6.

    Article  PubMed  CAS  Google Scholar 

  25. Nakamura S, Sugiyama T, Matsumoto T, Iijima K, Ono S, Tajika M, et al. Long-term clinical outcome of gastric MALT lymphoma after eradication of Helicobacter pylori: a multicentre cohort follow-up study of 420 patients in Japan. Gut. 2012;61:507–13.

    Article  PubMed  Google Scholar 

  26. Remstein ED, Dogan A, Einerson RR, Paternoster SF, Fink SR, Law M, et al. The incidence and anatomic site specificity of chromosomal translocations in primary extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) in North America. Am J Surg Pathol. 2006;30:1546–53.

    Article  PubMed  Google Scholar 

  27. Inagaki H, Nakamura T, Li C, Sugiyama T, Asaka M, Kodaira J, et al. Gastric MALT lymphomas are divided into three groups based on responsiveness to Helicobacter Pylori eradication and detection of API2 MALT1 fusion. Am J Surg Pathol. 2004;28:1560–7.

    Article  PubMed  Google Scholar 

  28. Starostik P, Patzner J, Greiner A, Schwarz S, Kalla J, Ott G, et al. Gastric marginal zone B-cell lymphomas of MALT type develop along 2 distinct pathogenetic pathways. Blood. 2002;99:3–9.

    Article  PubMed  CAS  Google Scholar 

  29. Ennishi D, Yokoyama M, Mishima Y, Watanabe C, Terui Y, Takahashi S, et al. Rituximab plus CHOP as an initial chemotherapy for patients with disseminated MALT lymphoma. Leuk Lymphoma. 2007;48:2241–3.

    Article  PubMed  CAS  Google Scholar 

  30. Raderer M, Wohrer S, Streubel B, Drach J, Jager U, Turetschek K, et al. Activity of rituximab plus cyclophosphamide, doxorubicin/mitoxantrone, vincristine and prednisone in patients with relapsed MALT lymphoma. Oncology. 2006;70:411–7.

    Article  PubMed  CAS  Google Scholar 

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The authors declare that they have no conflict of interest.

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Correspondence to Masahiko Ohtaka.

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Ohtaka, M., Sato, T., Kobayashi, S. et al. Stage IV intramucosal gastric marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type. Clin J Gastroenterol 6, 127–133 (2013). https://doi.org/10.1007/s12328-013-0374-1

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  • DOI: https://doi.org/10.1007/s12328-013-0374-1

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