Advertisement

Pediatric-Type Follicular Lymphoma (PTFL)

  • Andishe AttarbaschiEmail author
Chapter

Abstract

Pediatric-type follicular lymphoma (PTFL), as defined in the recently updated 2016 World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues, is a rare entity of non-Hodgkin’s lymphoma (NHL) in childhood and adolescence accounting for less than 2% of all cases. PTFL is a mature B-cell neoplasm that is usually CD20+ and BCL2– and does not carry components of diffuse large B-cell lymphoma or rearrangements of BCL2, BCL6, IRF4, and IG loci. The most important histopathological differential diagnosis is large B-cell lymphoma with IRF4 rearrangement, which may be clinically indistinguishable from PTFL. PTFL is associated with male gender, adolescent age, normal serum LDH levels, lack of B-symptoms and limited stage I or II disease, mostly involving peripheral lymph nodes in the head and neck region. Treatment outcomes in PTFL seem to be excellent with either complete resection and an observational strategy only, or with risk-adapted B-cell NHL-type chemotherapy resulting in overall survival rates >95%.

Keywords

Rare non-Hodgkin’s lymphoma Pediatric-type follicular lymphoma (PTFL) Large B-cell lymphoma with IRF4 rearrangement BCL2 rearrangement Complete resection “Watch-and-wait” strategy Outcome 

References

  1. 1.
    Agrawal R, Wang J. Pediatric follicular lymphoma: a rare clinicopathologic entity. Arch Pathol Lab Med. 2009;133(1):142–6.PubMedGoogle Scholar
  2. 2.
    Anderson JR, Armitage JO, Weisenburger DD. Epidemiology of the non-Hodgkin’s lymphomas: distributions of the major subtypes differ by geographic locations. Non-Hodgkin’s Lymphoma Classification Project. Ann Oncol. 1998;9(7):717–20.CrossRefGoogle Scholar
  3. 3.
    Ferry JA, de Leval L, Louissaint A Jr, Harris NL. Follicular lymphoma. In: Jaffe ES, Arber DA, Campo E, Harris NL, Quintanilla-Martinez L, editors. Hematopathology. Philadelphia: Elsevier; 2017. p. p321–52.Google Scholar
  4. 4.
    Kansal R, Singleton TP, Ross CW, Finn WG, Padmore RF, Schnitzer B. Follicular hodgkin lymphoma: a histopathologic study. Am J Clin Pathol. 2002 Jan;117(1):29–35.CrossRefGoogle Scholar
  5. 5.
    Oschlies I, Salaverria I, Mahn F, Meinhardt A, Zimmermann M, Woessmann W, et al. Pediatric follicular lymphoma—a clinico-pathological study of a population-based series of patients treated within the Non-Hodgkin’s Lymphoma—Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials. Haematologica. 2010;95(2):253–9.CrossRefGoogle Scholar
  6. 6.
    Jaffe ES, Harris NL, Siebert R. Paediatric-type follicular lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, editors. WHO Classification of Tumours of Haemtopoietic and Lymphoid Tissues. Lyon: International Agency for Research on Cancer (IARC); 2017. p. 278–9.Google Scholar
  7. 7.
    Liu Q, Salaverria I, Pittaluga S, Jegalian AG, Xi L, Siebert R, et al. Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma. Am J Surg Pathol. 2013;37(3):333–43.CrossRefGoogle Scholar
  8. 8.
    Louissaint A Jr, Ackerman AM, Dias-Santagata D, Ferry JA, Hochberg EP, Huang MS, et al. Pediatric-type nodal follicular lymphoma: an indolent clonal proliferation in children and adults with high proliferation index and no BCL2 rearrangement. Blood. 2012;120(12):2395–404.CrossRefGoogle Scholar
  9. 9.
    Rohatiner AZ, Lister TA. The clinical course of follicular lymphoma. Best Pract Res Clin Haematol. 2005;18(1):1–10.CrossRefGoogle Scholar
  10. 10.
    Atra A, Meller ST, Stevens RS, Hobson R, Grundy R, Carter RL, et al. Conservative management of follicular non-Hodgkin’s lymphoma in childhood. Br J Haematol. 1998;103(1):220–3.CrossRefGoogle Scholar
  11. 11.
    Attarbaschi A, Beishuizen A, Mann G, Rosolen A, Mori T, Uyttebroeck A, et al. Children and adolescents with follicular lymphoma have an excellent prognosis with either limited chemotherapy or with a "Watch and wait" strategy after complete resection. Ann Hematol. 2013;92(11):1537–41.CrossRefGoogle Scholar
  12. 12.
    Finn LS, Viswanatha DS, Belasco JB, Snyder H, Huebner D, Sorbara L, et al. Primary follicular lymphoma of the testis in childhood. Cancer. 1999;85(7):1626–35.CrossRefGoogle Scholar
  13. 13.
    Kumar R, Galardy PJ, Dogan A, Rodriguez V, Khan SP. Rituximab in combination with multiagent chemotherapy for pediatric follicular lymphoma. Pediatr Blood Cancer. 2011;57(2):317–20.CrossRefGoogle Scholar
  14. 14.
    Lones MA, Raphael M, McCarthy K, Wotherspoon A, Terrier-Lacombe MJ, Ramsay AD, et al. Primary follicular lymphoma of the testis in children and adolescents. J Pediatr Hematol Oncol. 2012;34(1):68–71.CrossRefGoogle Scholar
  15. 15.
    Lorsbach RB, Shay-Seymore D, Moore J, Banks PM, Hasserjian RP, Sandlund JT, et al. Clinicopathologic analysis of follicular lymphoma occurring in children. Blood. 2002;99(6):1959–64.CrossRefGoogle Scholar
  16. 16.
    McNamara C, Davies J, Dyer M, Hoskin P, Illidge T, Lyttelton M, et al. Guidelines on the investigation and management of follicular lymphoma. Br J Haematol. 2012;156(4):446–67.CrossRefGoogle Scholar
  17. 17.
    Pinto A, Hutchison RE, Grant LH, Trevenen CL, Berard CW. Follicular lymphomas in pediatric patients. Mod Pathol. 1990;3(3):308–13.PubMedGoogle Scholar
  18. 18.
    Sorge C, Costa LJ, Taub JW, Xavier AC. Incidence and outcomes of rare paediatric non-hodgkin lymphomas. Br J Haematol. 2018.Google Scholar
  19. 19.
    Pittaluga S, Harris NL, Siebert R, Salaverria I. Large B-cell lymphoma with IRF4 rearrangment. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, editors. WHO Classification of Tumours of Haemtopoietic and Lymphoid Tissues. Lyon: International Agency for Research on Cancer (IARC); 2017. p. 280–1.Google Scholar
  20. 20.
    Louissaint A Jr, Schafernak KT, Geyer JT, Kovach AE, Ghandi M, Gratzinger D, et al. Pediatric-type nodal follicular lymphoma: a biologically distinct lymphoma with frequent MAPK pathway mutations. Blood. 2016;128(8):1093–100.CrossRefGoogle Scholar
  21. 21.
    Martin-Guerrero I, Salaverria I, Burkhardt B, Szczepanowski M, Baudis M, Bens S, et al. Recurrent loss of heterozygosity in 1p36 associated with TNFRSF14 mutations in IRF4 translocation negative pediatric follicular lymphomas. Haematologica. 2013;98(8):1237–41.CrossRefGoogle Scholar
  22. 22.
    Kussick SJ, Kalnoski M, Braziel RM, Wood BL. Prominent clonal B-cell populations identified by flow cytometry in histologically reactive lymphoid proliferations. Am J Clin Pathol. 2004;121(4):464–72.CrossRefGoogle Scholar
  23. 23.
    Salaverria I, Philipp C, Oschlies I, Kohler CW, Kreuz M, Szczepanowski M, et al. Translocations activating IRF4 identify a subtype of germinal center-derived B-cell lymphoma affecting predominantly children and young adults. Blood. 2011;118(1):139–47.CrossRefGoogle Scholar
  24. 24.
    Quintanilla-Martinez L, Sander B, Chan JK, Xerri L, Ott G, Campo E, et al. Indolent lymphomas in the pediatric population: follicular lymphoma, IRF4/MUM1+ lymphoma, nodal marginal zone lymphoma and chronic lymphocytic leukemia. Virchows Arch. 2016;468(2):141–57.CrossRefGoogle Scholar
  25. 25.
    Mellgren K, Attarbaschi A, Abla O, Alexander S, Bomken S, Bubanska E, et al. Non-anaplastic peripheral T cell lymphoma in children and adolescents-an international review of 143 cases. Ann Hematol. 2016;95(8):1295–305.CrossRefGoogle Scholar
  26. 26.
    Ronceray L, Abla O, Barzilai-Birenboim S, Bomken S, Chiang AK, Jazbec J, et al. Children and adolescents with marginal zone lymphoma have an excellent prognosis with limited chemotherapy or a watch-and-wait strategy after complete resection. Pediatr Blood Cancer. 2018;65(4).CrossRefGoogle Scholar
  27. 27.
    Thorer H, Zimmermann M, Makarova O, Oschlies I, Klapper W, Lang P, et al. Primary central nervous system lymphoma in children and adolescents: low relapse rate after treatment according to Non-Hodgkin-Lymphoma Berlin-Frankfurt-Munster protocols for systemic lymphoma. Haematologica. 2014;99(11):e238–41.CrossRefGoogle Scholar
  28. 28.
    Murphy SB, Fairclough DL, Hutchison RE, Berard CW. Non-Hodgkin’s lymphomas of childhood: an analysis of the histology, staging, and response to treatment of 338 cases at a single institution. J Clin Oncol. 1989;7(2):186–93.CrossRefGoogle Scholar
  29. 29.
    Rosolen A, Perkins SL, Pinkerton CR, Guillerman RP, Sandlund JT, Patte C, et al. Revised International Pediatric Non-Hodgkin Lymphoma Staging System. J Clin Oncol. 2015;33(18):2112–8.CrossRefGoogle Scholar
  30. 30.
    Kridel R, Sehn LH, Gascoyne RD. Pathogenesis of follicular lymphoma. J Clin Invest. 2012;122(10):3424–31.CrossRefGoogle Scholar
  31. 31.
    Perkins SL, Gross TG. Pediatric indolent lymphoma--would less be better? Pediatr Blood Cancer. 2011;57(2):189–90.CrossRefGoogle Scholar
  32. 32.
    Gerrard M, Cairo MS, Weston C, Auperin A, Pinkerton R, Lambilliote A, et al. Excellent survival following two courses of COPAD chemotherapy in children and adolescents with resected localized B-cell non-Hodgkin’s lymphoma: results of the FAB/LMB 96 international study. Br J Haematol. 2008;141(6):840–7.CrossRefGoogle Scholar
  33. 33.
    Goldman S, Smith L, Anderson JR, Perkins S, Harrison L, Geyer MB, et al. Rituximab and FAB/LMB 96 chemotherapy in children with Stage III/IV B-cell non-Hodgkin lymphoma: a Children’s Oncology Group report. Leukemia. 2013;27(5):1174–7.CrossRefGoogle Scholar
  34. 34.
    Pillon M, Di Tullio MT, Garaventa A, Cesaro S, Putti MC, Favre C, et al. Long-term results of the first Italian Association of Pediatric Hematology and Oncology protocol for the treatment of pediatric B-cell non-Hodgkin lymphoma (AIEOP LNH92). Cancer. 2004;101(2):385–94.CrossRefGoogle Scholar
  35. 35.
    Woessmann W, Seidemann K, Mann G, Zimmermann M, Burkhardt B, Oschlies I, et al. The impact of the methotrexate administration schedule and dose in the treatment of children and adolescents with B-cell neoplasms: a report of the BFM Group Study NHL-BFM95. Blood. 2005;105(3):948–58.CrossRefGoogle Scholar
  36. 36.
    Shankar A, Hall GW, Gorde-Grosjean S, Hasenclever D, Leblanc T, Hayward J, et al. Treatment outcome after low intensity chemotherapy [CVP] in children and adolescents with early stage nodular lymphocyte predominant Hodgkin’s lymphoma - an Anglo-French collaborative report. Eur J Cancer. 2012;48(11):1700–6.CrossRefGoogle Scholar
  37. 37.
    Marcus R, Imrie K, Belch A, Cunningham D, Flores E, Catalano J, et al. CVP chemotherapy plus rituximab compared with CVP as first-line treatment for advanced follicular lymphoma. Blood. 2005;105(4):1417–23.CrossRefGoogle Scholar
  38. 38.
    Marcus R, Imrie K, Solal-Celigny P, Catalano JV, Dmoszynska A, Raposo JC, et al. Phase III study of R-CVP compared with cyclophosphamide, vincristine, and prednisone alone in patients with previously untreated advanced follicular lymphoma. J Clin Oncol. 2008;26(28):4579–86.CrossRefGoogle Scholar
  39. 39.
    Meinhardt A, Burkhardt B, Zimmermann M, Borkhardt A, Kontny U, Klingebiel T, et al. Phase II window study on rituximab in newly diagnosed pediatric mature B-cell non-Hodgkin’s lymphoma and Burkitt leukemia. J Clin Oncol. 2010;28(19):3115–21.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Pediatric Hematology and OncologySt. Anna Children’s HospitalViennaAustria
  2. 2.Department of Pediatrics and Adolescent MedicineMedical University of ViennaViennaAustria

Personalised recommendations