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01-03-2021 | Lymphoma | Special Issue: Pediatric Pathology

Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck

Authors: A. Auerbach, J. J. Schmieg, N. S. Aguilera

Published in: Head and Neck Pathology | Issue 1/2021

Abstract

Lymphoid and histiocytic lesions of the head and neck in pediatric patients is a fascinating topic as most of these lesions are benign, but that the neoplastic cases are essential to diagnose accurately for appropriate treatment. It is thought that 90% of children will have palpable lymph nodes between the ages of 4 to 8; most, but not all, are non-malignant and some resolve spontaneously without treatment. This paper will look at many of the benign and malignant lesions of both lymphocytic and histiocytic origin that present in the head and neck of children focusing on their diagnostic criteria. There is a very pertinent discussion of nonmalignant lymphoid proliferations, as infections and other reactive conditions dominate the pathology of pediatric lymphohistiocytic head and neck lesions. Discussion of those lymphomas which arise more frequently in the head and neck focuses on those seen in children and young adults such as classic Hodgkin lymphoma and Burkitt lymphoma, as well as new more controversial entities such as pediatric-type follicular lymphoma. Histiocytic lesions, both benign and malignant, are described and may be challenging to diagnose.

Weinstock MS, Patel NA, Smith LP. Pediatric cervical lymphadenopathy. Pediatr Rev. 2018;39:433–43.PubMed

Schowinsky J. Reactive and malignant diseases of hematopoietic and lymphoid tissues. In: Greer R, Marx R, Said SPL, editors. Pediatric Head and Neck Pathology. Cambridge: Cambrige University Press; 2017. p. 505–41.

O’Malley D. General reactive conditions in lymph node and spleen. In: O’Malley D, George T, Orazi A, Abbondanzo S, editors. Benign Reaction Conditional lymph node spleen, Atlas nontumor Pathological first Series fascicle. Washinton, DC: ARP; 2009.

Chisholm KM, Fleming MD. Histologic and laboratory characteristics of symptomatic and asymptomatic castleman disease in the pediatric population. Am J Clin Pathol. 2020;153:821–32.PubMed

Haap M, Wiefels J, Horger M, Hoyer A, Müssig K. Clinical, laboratory and imaging findings in Castleman’s disease: the subtype decides. Blood Rev. 2018;32:225–34. https://doi.org/10.1016/j.blre.2017.11.005.CrossRefPubMed

Sopfe J, Endres A, Campbell K, Hayes K, Trout AT, Liang X, et al. Castleman disease in pediatrics: Insights on presentation, treatment, and outcomes from a two-site retrospective cohort study. Pediatr Blood Cancer. 2019;66:1–9.

Liu AY, Nabel CS, Finkelman BS, Ruth JR, Kurzrock R, van Rhee F, et al. Idiopathic multicentric Castleman’s disease: a systematic literature review. Lancet Haematol. 2016;3:e163–75. https://doi.org/10.1016/S2352-3026(16)00006-5.CrossRefPubMed

Luzuriaga K, Sullivan JL. Infectious mononucleosis. N Engl J Med. 2010;362:1993–2000.PubMed

Louissaint A, Ferry JA, Soupir CP, Hasserjian RP, Harris NL, Zukerberg LR. Infectious mononucleosis mimicking lymphoma: distinguishing morphological and immunophenotypic features. Mod Pathol. 2012;25:1149–59.PubMed

10.

Swerdlow S, Campo E, Harris NL, Jaffe ES, Pileri S, Stein H, et al. WHO classification of Tumours of Haematopoietic and Lymphoid Tissues. revised 4th edition. Swerdlow S, Campo E, Harris NL, Jaffe ES, Pileri S, Stein H, et al., editors. Lyon Cedex 08: International Agency for Research on Cancer (IARC); 2017.

11.

Albright JT, Pransky SM. Nontuberculous mycobacterial infections of the head and neck. Pediatr Clin N Am. 2003;50:503–14.

12.

Lin MH, Kuo TT. Specificity of the histopathological triad for the diagnosis of toxoplasmic lymphadenitis: polymerase chain reaction study. Pathol Int. 2001;51:619–23.PubMed

13.

Eapen M, Mathew CF, Aravindan KP. Evidence based criteria for the histopathological diagnosis of toxoplasmic lymphadenopathy. J Clin Pathol. 2005;58:1143–6.PubMedPubMedCentral

14.

Onciu M, Medeiros LJ. Kikuchi-Fujimoto. Lymphadenitis. 2003;10:204–11.

15.

Wang T-J, Yang Y-H, Lin Y-T, Chiang B-L. Kikuchi-Fujimoto disease in children: clinical features and disease course. J Microbiol Immunol Infect. 2004;37:219–24.PubMed

16.

Felgar RE, Furth EE, Wasik MA, Gluckman SJ, Salhany KE. Histiocytic necrotizing lymphadenitis (Kikuchi’s disease): in situ end-labeling, immunohistochemical, and serologic evidence supporting cytotoxic lymphocyte-mediated apoptotic cell death. Mod Pathol. 1997;10:231–41.PubMed

17.

Chamulak GA, Brynes RK, Nathwani BN. Kikuchi-Fujimoto disease mimicking malignant lymphoma. Am J Surg Pathol. 1990;14:514–23.PubMed

18.

Sandlund JT, Martin MG. Non-Hodgkin lymphoma across the pediatric and adolescent and young adult age spectrum. Hematology. 2016;2016:589–97.PubMed

19.

Sandlund JT. Non-Hodgkin Lymphoma in Children. Curr Hematol Malig Rep. 2015;10:237–43.PubMed

20.

Etemad-Moghadam S, Tirgary F, Keshavarz S, Alaeddini M. Head and neck non-Hodgkin’s lymphoma: A 20-year demographic study of 381 cases. Int J Oral Maxillofac Surg. 2010;39:869–72. https://doi.org/10.1016/j.ijom.2010.03.029.CrossRefPubMed

21.

Tadbir AA, Mehrabani D, Heydari ST. M 1538. 2007;2007:2002–5.

22.

Keegan THM, Glaser SL, Clarke CA, Gulley ML, Craig FE, DiGiuseppe JA, et al. Epstein-Barr virus as a marker of survival after Hodgkin’s lymphoma: a population-based study. J Clin Oncol. 2005;23:7604–13.PubMed

23.

Claviez A, Tiemann M, Lüders H, Krams M, Parwaresch R, Schellong G, et al. Impact of latent Epstein-Barr virus infection on outcome in children and adolescents with Hodgkin’s lymphoma. J Clin Oncol. 2005;23:4048–56.PubMed

24.

Urquhart A, Berg R. Hodgkin ’ s and Non-Hodgkin ’ s Lymphoma of the Head and Neck.

25.

Shamloo N, Ghannadan A, Jafari M, Ahmadi S, Mortazavi H, Baharvand M. Head and neck lymphoma in an Iranian population. Iran J Otorhinolaryngol. 2017;29:261–7.PubMedPubMedCentral

26.

del Quiñones-Avila MP, Gonzalez-Longoria AA, Admirand JH, Medeiros LJ. Hodgkin lymphoma involving Waldeyer ring: a clinicopathologic study of 22 cases. Am J Clin Pathol. 2005;123:651–6.

27.

Dunphy CH, Saravia O, Varvares MA. Hodgkin’s disease primarily involving Waldeyer’s ring. Case report and review of the literature. Arch Pathol Lab Med. 1996;120:285–7.PubMed

28.

Kapadia SB, Roman LN, Kingma DW, Jaffe ES, Frizzera G. Hodgkin’s disease of Waldeyer’s ring. Clinical and histoimmunophenotypic findings and association with Epstein-Barr virus in 16 cases. Am J Surg Pathol. 1995;19:1431–9.PubMed

29.

Cionini L, Bastiani P, Biti GP, Mungai V, Ponticelli P, Di Lollo S. Waldeyer’s ring (WR) involvement in Hodgkin’s disease. Radiother Oncol. 1985;3:299–302.PubMed

30.

Burkitt DP. The discovery of Burkitt’s lymphoma. Cancer. 1983;51:1777–86.PubMed

31.

Rohde M, Bonn BR, Zimmermann M, Lange J, Möricke A, Klapper W, et al. Relevance of ID3-TCF3-CCND3 pathway mutations in pediatric aggressive B-cell lymphoma treated according to the non-Hodgkin lymphoma Berlin-Frankfurt-münster protocols. Haematologica. 2017;102:1091–8.PubMedPubMedCentral

32.

Havelange V, Pepermans X, Ameye G, Théate I, Callet-Bauchu E, Barin C, et al. Genetic differences between paediatric and adult Burkitt lymphomas. Br J Haematol. 2016;173:137–44.PubMed

33.

Gonzalez-Farre B, Ramis-Zaldivar JE, Salmeron-Villalobos J, Balagué O, Celis V, Verdu-Amoros J, et al. Burkitt-like lymphoma with 11q aberration: a germinal center-derived lymphoma genetically unrelated to Burkitt lymphoma. Haematologica. 2019;104:1822–9.PubMedPubMedCentral

34.

Thunberg U, Amini RM, Linderoth J, Roos G, Enblad G, Berglund M. BCL2 expression in de novo diffuse large B-cell lymphoma partly reflects normal differences in age distribution. Br J Haematol. 2009;146:683–4.PubMed

35.

Szczepanowski M, Lange J, Kohler CW, Masque-Soler N, Zimmermann M, Aukema SM, et al. Cell-of-origin classification by gene expression and MYC-rearrangements in diffuse large B-cell lymphoma of children and adolescents. Br J Haematol. 2017;179:116–9.PubMed

36.

Msimang M, Ramdial P, Kuppusamy J, Nargan K, Sheik GM. AIDS-associated pdiatric high grade B-cell lymphoma with MYC and BCL2 translocations. J AIDS Clin Res. 2017;8:742.

37.

Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ. Diffuse large B-cell lymphoma variants: an update. Pathology. 2020;52:53–67.PubMed

38.

Ramis-Zaldivar JE, Gonzalez-Farré B, Balagué O, Celis V, Nadeu F, Salmerón-Villalobos J, et al. Distinct molecular profile of IRF4-rearranged large B-cell lymphoma. Blood. 2020;135:274–86.PubMedPubMedCentral

39.

Nguyen KA, Su C, Bai HX, Zhang Z, Xiao R, Karakousis G, et al. Disease site as a determinant of survival outcome in patients with systemic anaplastic lymphoma kinase positive anaplastic large cell lymphoma with extranodal involvement: an analysis of 1306 cases from the US National Cancer Database. Br J Haematol. 2018;181:196–204.PubMed

40.

Jaglowski SM, Linden E, Termuhlen AM, Flynn JM. Lymphoma in adolescents and young adults. Semin Oncol. 2009;36:381–418. https://doi.org/10.1053/j.seminoncol.2009.07.009.CrossRefPubMed

41.

Agostinelli C, Akarca AU, Ramsay A, Rizvi H, Rodriguez-Justo M, Pomplun S, et al. Novel markers in pediatric-type follicular lymphoma. Virchows Arch. 2019;475:771–9.PubMedPubMedCentral

42.

Rizzo KA, Streubel B, Pittaluga S, Chott A, Xi L, Raffeld M, et al. Marginal zone lymphomas in children and the young adult population: characterization of genetic aberrations by FISH and RT-PCR. Mod Pathol. 2010;23:866–73.PubMedPubMedCentral

43.

The Writing Group of the Histiocyte Society. Histiocytosis syndromes in children. Lancet. 1987;1:208–9.

44.

Golpanian S, Tashiro J, Gerth DJ, Thaller SR. Pediatric histiocytoses in the United States: incidence and outcomes. J Surg Res. 2014;190:221–9. https://doi.org/10.1016/j.jss.2014.03.063.CrossRefPubMed

45.

Robinson LD, Smith RJ, Rightmire J, Torpy JM, Fernbach DJ. Head and neck malignancies in children: an age-incidence study. Laryngoscope. 1988;98:11–3.PubMed

46.

Emile J-F, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, et al. Review article revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood. 2016;127:2672–82.PubMedPubMedCentral

47.

Kaatsch P. Epidemiology of childhood cancer. Cancer Treat Rev. 2010;36:277–85. https://doi.org/10.1016/j.ctrv.2010.02.003.CrossRefPubMed

48.

Grana N. Langerhans cell histiocytosis. Cancer Control. 2014;21:328–34.PubMed

49.

Leung AKC, Lam JM, Leong KF. Childhood Langerhans cell histiocytosis: a disease with many faces. World J Pediatr. 2019;15:536–45. https://doi.org/10.1007/s12519-019-00304-9.CrossRefPubMed

50.

Anonsen CK, Donaldson SS. Langerhans’ cell histiocytosis of the head and neck. Laryngoscope. 1987;97:537–42.PubMed

51.

Buchmann L, Emami A, Wei JL. Primary head and neck Langerhans cell histiocytosis in children. Otolaryngol Head Neck Surg. 2006;135:312–7.PubMed

52.

Haroche J, Abla O. Uncommon histiocytic disorders: Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease. Hematology. 2015;2015:571–8.PubMed

53.

Flaitz C, Allen C, Neville B, Hicks J. Juvenile xanthogranuloma of the oral cavity in children: a clinicopathologic study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002;94:345–52.PubMed

54.

Murphy JT, Soeken T, Megison S, Perez E. Juvenile xanthogranuloma: diverse presentations of noncutaneous disease. J Pediatr Hematol Oncol. 2014;36:641–5.PubMed

55.

Goyal G, Ravindran A, Young JR, Shah MV, Bennani NN, Patnaik MM, et al. Clinicopathological features, treatment approaches, and outcomes in Rosai-Dorfman disease. Haematologica. 2020;105:348–57.PubMedPubMedCentral

56.

Abla O, Jacobsen E, Picarsic J, Krenova Z, Jaffe R, Emile JF, et al. Consensus recommendations for the diagnosis and clinical management of Rosai-Dorfman-Destombes disease. Blood. 2018;131:2877–90.PubMedPubMedCentral

57.

Alwani MM, Elghouche AN, Schueth EA, Campiti VJ, Matt BH, Yekinni AO. Manifestations of pediatric extranodal Rosai Dorfman disease in the head and neck. Int J Pediatr Otorhinolaryngol. 2020;131:109851. https://doi.org/10.1016/j.ijporl.2019.109851.CrossRefPubMed

58.

Takahashi E, Nakamura S. Histiocytic sarcoma: an updated literature review based on the 2008 WHO classification. J Clin Exp Hematop. 2013;53:1–8.PubMed

59.

Skala SL, Lucas DR, Dewar R. Histiocytic sarcoma: review, discussion of transformation from B-cell lymphoma, and differential diagnosis. Arch Pathol Lab Med. 2018;142:1322–9.PubMed

60.

Bhalla V, Khan N, Jones M, Kumar A, Latifaj B, Colmenero I, et al. A rare case of paediatric histiocytic sarcoma of the maxilla and mandible. Dentomaxillofacial Radiol. 2016;45:20150393.

61.

Aakalu VK, Patel RM, Lim J, Setabutr P. Orbital histiocytic sarcoma in a 2-year-old child. Ophthal Plast Reconstr Surg. 2014;30:e143–5.PubMedPubMedCentral

62.

Castro ECC, Blazquez C, Boyd J, Correa H, De Chadarevian JP, Felgar RE, et al. Clinicopathologic features of histiocytic lesions following ALL, with a review of the literature. Pediatr Dev Pathol. 2010;13:225–37.PubMed

63.

Liu Q, Tomaszewicz K, Hutchinson L, Hornick JL, Woda B, Yu H. Somatic mutations in histiocytic sarcoma identified by next generation sequencing. Virchows Arch. 2016;469:233–41. https://doi.org/10.1007/s00428-016-1965-2.CrossRefPubMed

64.

Klco JM, Welch JS, Nguyen TT, Hurley MY, Kreisel FH, Hassan A, et al. State of the art in myeloid sarcoma. Int J Lab Hematol. 2011;33:555–65.PubMed

65.

Samborska M, Derwich K, Skalska-Sadowska J, Kurzawa P, Wachowiak J. Myeloid sarcoma in children: diagnostic and therapeutic difficulties. Wspolczesna Onkol. 2016;20:444–8.

66.

Gaal A, Chisholm KM, Egbert M. How rare is an oral presentation of myeloid sarcoma in the infant? J Oral Maxillofac Surg. 2018;76:561–8. https://doi.org/10.1016/j.joms.2017.08.034.CrossRefPubMed

67.

Cuthbertson DW, Punia JN, Owczarzak VL. Myeloid sarcomas of the head and neck in pediatric patients with myeloid leukemia. Ear Nose Throat J. 2016;95:405–7.PubMed

68.

Roby BB, Drehner D, Sidman JD. Granulocytic sarcoma of pediatric head and neck: an institutional experience. Int J Pediatr Otorhinolaryngol. 2013;77:1364–6. https://doi.org/10.1016/j.ijporl.2013.06.008.CrossRefPubMed

69.

Chang KTE, Tay AZE, Kuick CH, Chen H, Algar E, Taubenheim N, et al. ALK-positive histiocytosis: an expanded clinicopathologic spectrum and frequent presence of KIF5B-ALK fusion. Mod Pathol. 2019;32:598–608.PubMed

70.

Lucas CHG, Gilani A, Solomon DA, Liang X, Maher OM, Chamyan G, et al. ALK-positive histiocytosis with KIF5B-ALK fusion in the central nervous system. Acta Neuropathol. 2019;138:335–7. https://doi.org/10.1007/s00401-019-02027-7.CrossRefPubMedPubMedCentral

71.

Chan JKC, Lamant L, Algar E, Delsol G, Tsang WYW, Lee KC, et al. ALK+ histiocytosis: a novel type of systemic histiocytic proliferative disorder of early infancy. Blood. 2008;112:2965–8.PubMed

72.

Allen CE, Kelly KM, Bollard CM. Pediatric lymphomas and histiocytic disorders of childhood. Pediatr Clin N Am. 2015;62:139–65.

73.

Usmani GN, Woda BA, Newburger PE. Advances in understanding the pathogenesis of HLH. Br J Haematol. 2013;161:609–22.PubMed

74.

Henter J-I, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124–31.PubMed

75.

Prilutskiy A, Kritselis M, Shevtsov A, Yambayev I, Vadlamudi C, Zhao Q, et al. SARS-CoV-2 infection-associated hemophagocytic lymphohistiocytosis. Am J Clin Pathol. 2020;154:466–74.PubMed

76.

Morrell DS, Pepping MA, Scott JP, Esterly NB, Drolet BA. Cutaneous manifestations of hemophagocytic lymphohistiocytosis. Arch Dermatol. 2002;138:1208–12.PubMed

77.

Lindemann TL, Greene JS. Persistent cervical lymphadenopathy in an adolescent with Epstein-Barr induced hemophagocytic syndrome: manifestations of a rare but often fatal disease. Int J Pediatr Otorhinolaryngol. 2005;69:1011–4.PubMed

78.

Lang S, Kansy B. Cervical lymph node diseases in children. GMS Curr Top Otorhinolaryngol Head Neck Surg. 2014;13:08.

79.

Ghanaiee RM, Shiari R, Karimi A, Armin S, Fahimzad A, Shiva F, et al. A case series report of Iranian children: hemophagocytic lymphohistiocytosis syndrome. Arch Pediatr Infect Dis. 2013;1:31–5.

80.

Kong MS, Engel SH, Zalzal GH, Preciado D. Necrotizing epiglottitis and hemophagocytic lymphohistiocytosis. Int J Pediatr Otorhinolaryngol. 2009;73:119–25.PubMed

81.

Miettinen M, Fetsch JF. Reticulohistiocytoma (Solitary Epithelioid Histiocytoma). Am J Surg Pathol. 2006;30:521–8.PubMed

82.

Bahrani E, Fernandez-Pol S, Wang JY, Aasi SZ, Brown RA, Novoa RA. Reticulohistiocytoma (solitary epithelioid histiocytoma) with mutations in RAF1 and TSC2. J Cutan Pathol. 2020. https://doi.org/10.1111/cup.13727.CrossRefPubMed

83.

Hoyt BS, Yan S, Linos KD, Momtahen S, Sriharan A, Tran TAN, et al. BRAF V600E mutations are not an oncogenic driver of solitary xanthogranuloma and reticulohistiocytoma: testing may be useful in screening for Erdheim-Chester disease. Exp Mol Pathol. 2019;111:104320. https://doi.org/10.1016/j.yexmp.2019.104320.CrossRefPubMed

Title: Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck
Authors: A. Auerbach
J. J. Schmieg
N. S. Aguilera
Publication date: 01-03-2021
Publisher: Springer US
Keywords: Lymphoma
Hodgkin Lymphoma
Sarcoma
Anaplastic Large Cell Lymphoma
Anaplastic Large Cell Lymphoma
Published in: Head and Neck Pathology / Issue 1/2021
Electronic ISSN: 1936-0568
DOI: https://doi.org/10.1007/s12105-020-01257-6

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Pediatric Lymphoid and Histiocytic Lesions in the Head and Neck

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