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Palatine Tonsils Primary Presentation of Blastoid Variant of Mantle Cell Lymphoma: Case Report

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Abstract

Head and neck lymphomas can present with a wide range of symptoms. Timely and accurate diagnosis is often challenging. The blastoid variant of mantle cell lymphoma (MCL) accounts for less than one-third of all MCL cases. Isolated primary presentation on the palatine tonsils is rare, and prognosis and outcome are seemingly unfavorable. An 81-year-old man presented with persistent odynophagia, dysphagia, and obstructive hypertrophic palatine tonsils with purulent exudate. The signs and symptoms were non-responsive to antibiotic therapy, and the tonsils were biopsied. The cellular morphology, immunophenotype, and genotype supported a diagnosis of the blastoid variant of MCL. After staging, the patient underwent chemotherapy with Rituximab-Bendamustine (R-Benda). The patient is in clinical remission more than two years after therapy. We report an exceedingly rare case of blastoid MCL that is prone to be misdiagnosed as tonsillitis. We review the literature and discuss treatment options of this uncommon malignancy.

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References

  1. Cooper JS, Porter K, Mallin K, et al. National Cancer Database report on cancer of the head and neck: 10-year update. Head Neck. 2009;31(6):748–58.

    Article  Google Scholar 

  2. Armitage JO, Gascoyne RD, Lunning MA, Cavalli F. Non-Hodgkin lymphoma. Lancet. 2017;390(10091):298–310.

    Article  Google Scholar 

  3. Urquhart A, Berg R. Hodgkin’s and non-Hodgkin’s lymphoma of the head and neck. Laryngoscope. 2001;111(9):1565–9.

    Article  CAS  Google Scholar 

  4. Bagan JV, Carbonell F, Gomez MJ, et al. Extra-nodal B-cell non-Hodgkin’s lymphomas of the head and neck: a study of 68 cases. Am J Otolaryngol. 2015;36(1):57–62.

    Article  Google Scholar 

  5. Iguchi H, Wada T, Matsushita N, Oishi M, Yamane H. Anatomic distribution of hematolymphoid malignancies in the head and neck: 7 years of experience with 122 patients in a single institution. Acta Otolaryngol. 2012;132(11):1224–31.

    Article  Google Scholar 

  6. Weber AL, Rahemtullah A, Ferry JA. Hodgkin and non-Hodgkin lymphoma of the head and neck: clinical, pathologic, and imaging evaluation. Neuroimaging Clin N Am. 2003;13(3):371–92.

    Article  Google Scholar 

  7. Picard A, Cardinne C, Denoux Y, Wagner I, Chabolle F, Bach CA. Extranodal lymphoma of the head and neck: a 67-case series. Eur Ann Otorhinolaryngol Head Neck Dis. 2015;132(2):71–5.

    Article  CAS  Google Scholar 

  8. Mohammadianpanah M, Daneshbod Y, Ramzi M, et al. Primary tonsillar lymphomas according to the new World Health Organization classification: to report 87 cases and literature review and analysis. Ann Hematol. 2010;89(10):993–1001.

    Article  Google Scholar 

  9. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90.

    Article  CAS  Google Scholar 

  10. A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project. Blood 1997;89(11):3909–18.

  11. Vose JM. Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management. Am J Hematol. 2017;92(8):806–13.

    Article  CAS  Google Scholar 

  12. Samaha H, Dumontet C, Ketterer N, et al. Mantle cell lymphoma: a retrospective study of 121 cases. Leukemia. 1998;12(8):1281–7.

    Article  CAS  Google Scholar 

  13. Tiemann M, Schrader C, Klapper W, et al. Histopathology, cell proliferation indices and clinical outcome in 304 patients with mantle cell lymphoma (MCL): a clinicopathological study from the European MCL Network. Br J Haematol. 2005;131(1):29–38.

    Article  Google Scholar 

  14. Ferrer A, Salaverria I, Bosch F, et al. Leukemic involvement is a common feature in mantle cell lymphoma. Cancer. 2007;109(12):2473–80.

    Article  CAS  Google Scholar 

  15. Shrestha R, Bhatt VR, Guru Murthy GS, Armitage JO. Clinicopathologic features and management of blastoid variant of mantle cell lymphoma. Leuk Lymphoma. 2015;56(10):2759–67.

    Article  CAS  Google Scholar 

  16. Bhatt VR, Loberiza FR Jr, Smith LM, et al. Clinicopathologic features, management and outcomes of blastoid variant of mantle cell lymphoma: a Nebraska Lymphoma Study Group experience. Leuk Lymphoma. 2016;57(6):1327–34.

    Article  CAS  Google Scholar 

  17. Fisher RI. Mantle-cell lymphoma: classification and therapeutic implications. Ann Oncol. 1996;7(Suppl 6):S35–9.

    Article  Google Scholar 

  18. Norton AJ, Matthews J, Pappa V, et al. Mantle cell lymphoma: natural history defined in a serially biopsied population over a 20-year period. Ann Oncol. 1995;6(3):249–56.

    Article  CAS  Google Scholar 

  19. Pott C, Schrader C, Bruggemann M, et al. Blastoid variant of mantle cell lymphoma: late progression from classical mantle cell lymphoma and quantitation of minimal residual disease. Eur J Haematol. 2005;74(4):353–8.

    Article  Google Scholar 

  20. Bernard M, Gressin R, Lefrere F, et al. Blastic variant of mantle cell lymphoma: a rare but highly aggressive subtype. Leukemia. 2001;15(11):1785–91.

    Article  CAS  Google Scholar 

  21. Ott G, Kalla J, Ott MM, et al. Blastoid variants of mantle cell lymphoma: frequent bcl-1 rearrangements at the major translocation cluster region and tetraploid chromosome clones. Blood. 1997;89(4):1421–9.

    Article  CAS  Google Scholar 

  22. Viswanatha DS, Foucar K, Berry BR, Gascoyne RD, Evans HL, Leith CP. Blastic mantle cell leukemia: an unusual presentation of blastic mantle cell lymphoma. Mod Pathol. 2000;13(7):825–33.

    Article  CAS  Google Scholar 

  23. Ferrer A, Bosch F, Villamor N, et al. Central nervous system involvement in mantle cell lymphoma. Ann Oncol. 2008;19(1):135–41.

    Article  CAS  Google Scholar 

  24. Dreyling M, Klapper W, Rule S. Blastoid and pleomorphic mantle cell lymphoma: still a diagnostic and therapeutic challenge! Blood. 2018;132(26):2722–9. https://doi.org/10.1182/blood-2017-08-737502.

    Article  CAS  PubMed  Google Scholar 

  25. Dong HY, Gorczyca W, Liu Z, et al. B-cell lymphomas with coexpression of CD5 and CD10. Am J Clin Pathol. 2003;119:218–30.

    Article  Google Scholar 

  26. Rajkumar K, Rao R, Chawla N, Bandyopadhyay TK, Sinha R. Mantle cell lymphoma of the oral cavity with multiple foci: a case report and review of the literature. J Maxillofac Oral Surg. 2015;14(Suppl 1):138–44.

    Article  CAS  Google Scholar 

  27. Hart S, Horsman JM, Radstone CR, Hancock H, Goepel JR, Hancock BW. Localised extranodal lymphoma of the head and neck: the Sheffield Lymphoma Group experience (1971–2000). Clin Oncol (R Coll Radiol). 2004;16(3):186–92.

    Article  CAS  Google Scholar 

  28. Aguilera NS, Uusafr M, Wenig BM, Abbondanzo SL. The blastic variant of mantle cell lymphoma arising in Waldeyer’s tonsillar ring. J Laryngol Otol. 1998;112(10):991–4.

    Article  CAS  Google Scholar 

  29. Cohen JB, Han X, Jemal A, Ward EM, Flowers CR. Deferred therapy is associated with improved overall survival in patients with newly diagnosed mantle cell lymphoma. Cancer. 2016;122(15):2356–63.

    Article  CAS  Google Scholar 

  30. Robak T, Huang H, Jin J, et al. Bortezomib-based therapy for newly diagnosed mantle-cell lymphoma. N Engl J Med. 2015;372(10):944–53.

    Article  CAS  Google Scholar 

  31. Flinn IW, van der Jagt R, Kahl BS, et al. Randomized trial of bendamustine-rituximab or R-CHOP/R-CVP in first-line treatment of indolent NHL or MCL: the BRIGHT study. Blood. 2014;123(19):2944–52.

    Article  CAS  Google Scholar 

  32. Romaguera JE, Fayad LE, Feng L, et al. Ten-year follow-up after intense chemoimmunotherapy with Rituximab-HyperCVAD alternating with Rituximab-high dose methotrexate/cytarabine (R-MA) and without stem cell transplantation in patients with untreated aggressive mantle cell lymphoma. Br J Haematol. 2010;150(2):200–8.

    CAS  PubMed  Google Scholar 

  33. Geisler CH, Kolstad A, Laurell A, et al. Nordic MCL2 trial update: six-year follow-up after intensive immunochemotherapy for untreated mantle cell lymphoma followed by BEAM or BEAC + autologous stem-cell support: still very long survival but late relapses do occur. Br J Haematol. 2012;158(3):355–62.

    Article  CAS  Google Scholar 

  34. Ruan J, Martin P, Shah B, et al. Lenalidomide plus rituximab as initial treatment for mantle-cell lymphoma. N Engl J Med. 2015;373(19):1835–44.

    Article  CAS  Google Scholar 

  35. Maddocks K, Christian B, Jaglowski S, et al. A phase 1/1b study of rituximab, bendamustine, and ibrutinib in patients with untreated and relapsed/refractory non-Hodgkin lymphoma. Blood. 2015;125(2):242–8.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Otorhinolaryngology, Centro Hospitalar Universitário S. João, EPE, Porto, Portugal

    Joana A. S. Marques, Margarida Santos & Ricardo P. Vaz

  2. Unit of Otorhinolaryngology - Department of Surgery and Physiology, Faculty of Medicine, University of Porto, Porto, Portugal

    Joana A. S. Marques

  3. Department of Clinical Haematology, Centro Hospitalar Universitário S. João, EPE, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal

    Fátima Ferreira

  4. Department of Anatomic Pathology, Centro Hospitalar Universitário S. João, EPE, Porto, Portugal

    Daniel P. Melo

  5. Department of Pathology, Faculty of Medicine, University of Porto, Porto, Portugal

    Daniel P. Melo

  6. Unit of Anatomy - Department of Biomedicine, Faculty of Medicine, University of Porto, Porto, Portugal

    Ricardo P. Vaz

  7. Center for Health Technology and Services Research (CINTESIS), Porto, Portugal

    Ricardo P. Vaz

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  1. Joana A. S. Marques
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  2. Fátima Ferreira
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  4. Margarida Santos
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Correspondence to Joana A. S. Marques.

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Marques, J.A.S., Ferreira, F., Melo, D.P. et al. Palatine Tonsils Primary Presentation of Blastoid Variant of Mantle Cell Lymphoma: Case Report. Head and Neck Pathol 15, 588–592 (2021). https://doi.org/10.1007/s12105-020-01238-9

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  • DOI: https://doi.org/10.1007/s12105-020-01238-9

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