первичные экстранодальные лимфомы

Первичная медиастинальная (тимическая) В-крупноклеточная лимфома

ОБЗОРЫ , , , , ,

Г.С. Тумян, И.З. Заводнова, М.Ю. Кичигина, Е.Г. Медведовская

ФГБУ «Российский онкологический научный центр им. Н.Н. Блохина» Минздрава России, Каширское ш., д. 24, Moсква, Российская Федерация, 115478

Для переписки: Гаяне Сергеевна Тумян, д-р мед. наук, Каширское ш., д. 24, Moсква, Российская Федерация, 115478; тел.: +7(499)324-98-29; e-mail: gaytum@mail.ru

Для цитирования: Тумян Г.С., Заводнова И.З., Кичигина М.Ю., Медведовская Е.Г. Первичная медиастинальная (тимическая) В-крупноклеточная лимфома. Клиническая онкогематология. 2017;10(1):13–24.

DOI: 10.21320/2500-2139-2017-10-1-13-24

РЕФЕРАТ

Первичная медиастинальная (тимическая) В-крупноклеточная лимфома (ПМВКЛ) относится к первичным экстранодальным опухолям и происходит из В-клеток мозгового слоя тимуса. Болезнь чаще встречается у молодых женщин и проявляется преимущественно местнораспространенным характером роста в пределах переднего верхнего средостения с частым вовлечением органов грудной клетки. ПМВКЛ имеет специфические иммуноморфологическую и генетическую характеристики, которые позволяют идентифицировать ее от остальных, сходных по проявлениям заболеваний: диффузной В-крупноклеточной лимфомы, лимфомы Ходжкина с нодулярным склерозом и медиастинальной лимфомы «серой зоны». Стандартом лечения ПМВКЛ является иммунохимиотерапия с последующим облучением остаточной опухоли в средостении. К настоящему времени преимуществ одного лекарственного режима перед другим в рамках контролируемых исследований не показано. Применение новых методов визуализации (ПЭТ/КТ) позволяют надеяться на возможность отказа от лучевой терапии у определенной части больных ПМВКЛ без ухудшения показателей отдаленной выживаемости.

Ключевые слова: первичная медиастинальная (тимическая) В-крупноклеточная лимфома, первичные экстранодальные лимфомы, диагностика, патогенез, иммуноморфологическая и генетическая характеристики, лечение.

Получено: 22 августа 2016 г.

Принято в печать: 17 декабря 2016 г.

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ЛИТЕРАТУРА

  1. Benjamin SP, McCormack LJ, Effler DB, et al. Primary lymphatic tumors of the mediastinum. Cancer. 1972;30(3):708–12. doi: 10.1002/1097-0142(197209)30:3<708::AID-CNCR2820300318>3.0.CO;2–5.
  2. Lichtenstein AK, Levine A, Taylor CR, et al. Primary mediastinal lymphoma in adults. Am J Med. 1980;68(4):509–14. doi: 10.1016/0002-343(80)90294-6.
  3. National Cancer Institute sponsored study of classifications of non-Hodgkin’s lymphomas: summary and description of a working formulation for clinical usage. The Non-Hodgkin’s Lymphoma Pathologic Classification Project. Cancer. 1982;49(10):2112–35. doi: 10.1002/1097-0142(19820515)49:10<2112::AID-CNCR2820491024>3.0.CO;2–2.
  4. Stansfeld AG, Diebold J, Noel H, et al. Updated Kiel classification for lymphomas. Lancet. 1988;1(8580):292–3. doi: 10.1016/S0140-6736(88)90367-4.
  5. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood. 1994;84(5):1361–92. doi: 10.1016/S0968-6053(00)80051-4.
  6. Swerdlow SH, Campo E, Harris NL, et al, eds. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th edition. Lyon: IARC Press; 2008. doi: 10.1002/9781118853771.ch51.
  7. Cazals-Hatem D, Lepage E, Brice P, et al. Primary mediastinal large B-cell lymphoma. A clinicopathologic study of 141 cases compared with 916 nonmediastinal large B-cell lymphomas, a GELA (“Groupe d’Etude des Lymphomes de l’Adulte”) study. Am J Surg Pathol. 1996;20(7):877–88. doi: 10.1097/00000478-199607000-00012.
  8. Harris NL. Shades of gray between large B-cell lymphomas and Hodgkin lymphomas: differential diagnosis and biological implications. Mod Pathol. 2013;26(Suppl 1):S57–70. doi: 10.1038/modpathol.2012.182.
  9. Kanavaros P, Gaulard P, Charlotte F, et al. Discordant expression of immunoglobulin and its associated molecule mb-1/CD79a is frequently found in mediastinal large B cell lymphomas. Am J Pathol. 1995;146(3):735–41.
  10. Pileri SA, Zinzani PL, Gaidano G, et al. Pathobiology of primary mediastinal B-cell lymphoma. Leuk Lymphoma. 2003;44(Suppl 3):S21–6. doi: 10.1080/10428190310001623810.
  11. Loddenkemper C, Anagnostopoulos I, Hummel M, et al. Differential Emu enhancer activity and expression of BOB.1/OBF.1, Oct2, PU.1, and immunoglobulin in reactive B-cell populations, B-cell non-Hodgkin lymphomas, and Hodgkin lymphomas. J Pathol. 2004;202(1):60–9. doi: 10.1002/path.1485.
  12. De Leval L, Ferry JA, Falini B, et al. Expression of bcl-6 and CD10 in primary Mediastinal large B-cell lymphoma: evidence for derivation from germinal center B cells? Am J Surg Pathol. 2001;25(10):1277–82. doi: 10.1097/00000478-200110000-00008.
  13. Rosenwald A, Wright G, Leroy K, et al. Molecular diagnosis of primary mediastinal B cell lymphoma identifies a clinically favorable subgroup of diffuse large B cell lymphoma related to Hodgkin lymphoma. J Exp Med 2003;198(6):851–62. doi: 10.1084/jem.20031074.
  14. CopieBergman C, Plonquet A, Alonso MA, et al. MAL expression in lymphoid cells: further evidence for MAL as a distinct molecular marker of primary mediastinal large B-cell lymphomas. Mod Pathol. 2002;15:1172–80. doi: 10.1097/01.MP.0000032534.81894.B3.
  15. Joos S, Otano-Joos MI, Ziegler S, et al. Primary mediastinal (thymic) B-cell lymphoma is characterized by gains of chromosomal material including 9p and amplification of the REL gene. Blood. 1996;87(4):1571–8.
  16. Feuerhake F, Kutok JL, Monti S, et al. NFkappaB activity, function, and target-gene signatures in primary mediastinal large B-cell lymphoma and diffuse large B-cell lymphoma subtypes. Blood. 2005;106(4):1392–9. doi: 10.1182/blood-2004-12-4901.
  17. Zhang B, Wang Z, Li T, et al. NF-kappaB2 mutation targets TRAF1 to induce lymphomagenesis. Blood. 2007;110(2):743–51. doi: 10.1182/blood-2006-11-058446.
  18. Meier C, Hoeller S, Bourgau C, et al. Recurrent numerical aberrations of JAK2 and deregulation of the JAK2-STAT cascade in lymphomas. Mod Pathol. 2009;22(3):476–87. doi: 10.1038/modpathol.2008.207.
  19. Rossi D, Cerri M, Capello D, et al. Aberrant somatic hypermutation in primary mediastinal large B-cell lymphoma. Leukemia. 2005;19(12):2363–6. doi: 10.1038/sj.leu.2403982.
  20. Steidl C, Gascoyne RD. The molecular pathogenesis of primary mediastial large B-cell lymphoma. Blood. 2011;118(10):2659–69. doi: 10.1182/blood-2011-05-326538.
  21. Martelli M, Di Rocco A, Russo E, et al. Primary mediastinal lymphoma: diagnosis and treatment options. Expert Rev Hematol. 2014;8(2):173–86. doi: 10.1586/17474086.2015.994604.
  22. Eberle FC, Salaverria I, Steidl C, et al. Gray zone lymphoma: chromosomal aberrations with immunophenotypic and clinical correlations. Mod Pathol. 2011;24(12):1586–97. doi: 10.1038/modpathol.2011.116.
  23. Eberle FC, Rodriguez-Canales J, Wei L, et al. Methylation profiling of mediastinal gray zone lymphoma reveals a distinctive signature with elements shared by classical Hodgkin’s lymphoma and primary mediastinal large B-cell lymphoma. Haematologica. 2011;96(4):558–66. doi: 10.3324/haematol.2010.033167.
  24. Moller P, Lammler B, Herrmann B, et al. The primary mediastinal clear cell lymphoma of B-cell type has variable defects in MHC antigen expression. Immunology. 1986;59(3):411–7. doi: 10.1007/bf00705408.
  25. Hamlin PA, Portlock CS, Straus DJ, et al. Primary mediastinal large B-cell lymphoma: optimal therapy and prognostic factor analysis in 141 consecutive patients treated at Memorial Sloan Kettering from 1980 to 1999. Br J Haematol. 2005;130(5):691–9. doi: 10.1111/j.1365-2141.2005.05661.x.
  26. Jacobson JO, Aisenberg AC, Lamarre L, et al. Mediastinal large cell lymphoma. An uncommon subset of adult lymphoma curable with combined modality therapy. Cancer. 1988;62(9):1893–8. doi: 10.1002/1097-0142(19881101)62:9<1893::AID-CNCR2820620904>3.0.CO;2-X.
  27. Zinzani PL, Martelli M, Magagnoli M, et al. Treatment and clinical management of primary mediastinal large B-cell lymphoma with sclerosis: MACOP-B regimen and mediastinal radiotherapy monitored by (67)Gallium scan in 50 patients. Blood. 1999;94(10):3289–93.
  28. Bishop PC, Wilson WH, Pearson D, et al. CNS involvement in primary mediastinal large B-cell lymphoma. J Clin Oncol. 1999;17(8):2479–85.
  29. Savage K, Al-Rajhi N, Voss N, et al. Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Ann Oncol. 2006;17:123–30. doi: 10.1016/s0360-3016(00)80463-0.
  30. Zinzani PL, Martelli M, Bertini M, et al. Induction chemotherapy strategies for primary mediastinal large B-cell lymphoma with sclerosis: a retrospective multinational study on 426 previously untreated patients. Haematologica. 2002;87(12):1258–6. doi: 10.3816/clm.2009.n.074.
  31. Fisher RI, Gaynor ER, Dahlberg S, et al. Comparison of a standard regimen (CHOP) with three intensive chemotherapy regimens for advanced non-Hodgkin’s lymphoma. N Engl J Med. 1993;328(14):1002–6. doi: 10.1056/NEJM199304083281404.
  32. Levitt LJ, Aisenberg AC, Harris NL, et al. Primary non-Hodgkin’s lymphoma of the mediastinum. Cancer. 1982;50(11):2486–92. doi: 10.1002/1097-0142(19821201)50:11<2486::AID-CNCR2820501138>3.0.CO;2-G.
  33. Todeschini G, Ambrosetti A, Meneghini V, et al. Mediastinal large-B-cell lymphoma with sclerosis: a clinical study of 21 patients. J Clin Oncol. 1990;8(5):804–8.
  34. Bertini M, Orsucci L, Vitolo U, et al. Stage II large B-cell lymphoma with sclerosis treated with MACOP-B. Ann Oncol. 1991;2(10):733–7.
  35. Falini B, Venturi S, Martelli M, et al. Mediastinal large B-cell lymphoma: clinical and immunohistological findings in 18 patients treated with different third-generation regimens. Br J Haematol. 1995;89(4):780–9. doi: 10.1111/j.1365-2141.1995.tb08415.x.
  36. van Besien K, Kelta M, Bahaguna P. Primary mediastinal B-cell lymphoma: a review of pathology and management. J Clin Oncol. 2001;19(6):1855–64.
  37. Zinzani PL, Martelli M, Bendandi M, et al. Primary mediastinal large B-cell lymphoma with sclerosis: a clinical study of 89 patients treated with MACOP-B chemotherapy and radiation therapy. Haematologica. 2001;86(2):187–91.
  38. Zinzani PL, Stefoni V, Finolezzi E, et al. Rituximab combined with MACOP-B or VACOP-B and radiation therapy in primary mediastinal large B-cell lymphoma: a retrospective study. Clin Lymph Myel. 2009;9(5):381–5. doi: 10.3816/CLM.2009.n.074.
  39. Dunleavy K, Pittaluga S, Maeda LS, et al. Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphoma. N Engl J Med. 2013;368(15):1408–16. doi: 10.1056/NEJMoa1214561.
  40. Moskowitz CH, Schoder H, Teruya-Feldstein J, et al. Risk-adapted dose-dense immunochemotherapy determined by interim FDG-PET in Advanced-stage diffuse large B-Cell lymphoma. J Clin Oncol. 2010;28(11):1896–903. doi: 10.1200/JCO.2009.26.5942.
  41. Savage KJ, Yenson PR, Shenkier T, et al. The outcome of primary mediastinal large B-cell lymphoma (PMBCL) in the R-CHOP treatment era. Blood. 2012;120(Suppl 1–2): Abstract 303.
  42. Martelli M, Ceriani L, Zucca E, et al. [18F]fluorodeoxyglucose positron emission tomography predicts survival after chemoimmunotherapy for primary mediastinal large B-cell lymphoma: results of the International Extranodal Lymphoma Study Group IELSG-26 Study. J Clin Oncol. 2014;32(17):1769–75. doi: 10.1200/JCO.2013.51.7524.
  43. Pinnix CC, Dabaja B, Ahmed MAet al. Single-institution experience in the treatment of primary mediastinal B cell lymphoma treated with immunochemotherapy in the setting of response assessment by 18fluorodeoxyglucose positron emission tomography. Int J Radiat Oncol Biol Phys. 2015;92(1):113–21. doi: 10.1016/j.ijrobp.2015.02.006.
  44. Sehn LH, Antin JH, Shulman LN, et al. Primary diffuse large B-cell lymphoma of the mediastinum: outcome following high-dose chemotherapy and autologous hematopoietic cell transplantation. Blood. 1998;91(2):717–23.
  45. Kuruvilla J, Pintilie M, Tsang R, et al. Salvage chemotherapy and autologous stem cell transplantation are inferior for relapsed or refractory primary mediastinal large B-cell lymphoma compared with diffuse large B-cell lymphoma. Leuk Lymphoma. 2008;49(7):1329–36. doi: 10.1080/10428190802108870.
  46. Hao Y, Chapuy B, Monti S, Sun HH. Selective JAK2 inhibition specifically decreases Hodgkin lymphoma and mediastinal large B-cell lymphoma growth in vitro and in vivo. Clin Cancer Res. 2014;20(10):2674–83. doi: 10.1158/1078-0432.CCR-13-3007.
  47. Dunleavy K, Wilson W. Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach? Blood. 2015;125(1):33–9. doi: 10.1182/blood-2014-05-575092.
  48. Berger R, Rotem-Yehudar R, Slama G, et al. Phase I safety and pharmacokinetic study of CT-011, a humanized antibody interacting with PD-1, in patients with advanced hematologic malignancies. Clin Cancer Res. 2008;14(10):3044–51. doi: 10.1158/1078-0432.CCR-07-4079.