Abstract
Mantle cell lymphoma (MCL) is a well-defined, non-Hodgkin lymphoma of B-cell origin displaying diverse morphological phenotypes and variable disease course. The World Health Organization recognizes two aggressive histopathologic variants of this type of lymphoma: pleomorphic and blastoid MCL. To date, only few cases of MCL affecting the oral cavity have been reported. Additionally, the involvement of the oral and maxillofacial area by aggressive MCL subsets is considered extremely rare with only two patients reported in the English language literature to the best of our knowledge. Herein, we describe a 69 year-old male with a prior history of MCL of the right lateral pharyngeal wall develo** a recurrent lesion extending to the palatal mucosa as diffuse ulceration and exhibiting histomorphological features of blastoid MCL. We also review the pertinent literature with emphasis on the diagnostic challenges and distinction between the different MCL variants.
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12105-021-01391-9/MediaObjects/12105_2021_1391_Fig1_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12105-021-01391-9/MediaObjects/12105_2021_1391_Fig2_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12105-021-01391-9/MediaObjects/12105_2021_1391_Fig3_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12105-021-01391-9/MediaObjects/12105_2021_1391_Fig4_HTML.jpg)
![](http://media.springernature.com/m312/springer-static/image/art%3A10.1007%2Fs12105-021-01391-9/MediaObjects/12105_2021_1391_Fig5_HTML.jpg)
Similar content being viewed by others
Data Availability
Department of Oral Medicine & Pathology and Hospital Dentistry, School of Dentistry, National and Kapodistrian University of Athens, Greece.
References
Swerdlow SH, Berger F, Isaacson PI, Muller-Hermelink HK. Mantle cell lymphoma. In: Harris NL, Jaffe ES, Stein H, Vardiman JW, editors. World Health Organization classification of tumours. pathology and genetics of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2001. p. 168. (Current pathologic criteria for diagnosis of MCL with discussion of histologic variants, biology, and clinical features).
Epstein JB, Epstein JD, Le ND, Gorsky M. Characteristics of oral and paraoral malignant lymphoma: a population-based review of 361 cases. Oral Surg Oral Med Oral Pathol. 2001;92:519–25.
Banks PM, Chan J, Cleary ML, Delsol G, De Wolf-Peeters C, Gatter K, Grogan TM, Harris NL, Isaacson PG, Jaffe ES, et al. Mantle cell lymphoma. A proposal for unification of morphologic, immunologic, and molecular data. Am J Surg Pathol. 1992;16:637–40.
Swerdlow SH, Campo E, Seto M, Muller-Hermelink HK. Mantle cell lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, editors. WHO classification of tumours of haematopoietic and lymphoid tissues. IARC; 2008. p. 229–232.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, editors. WHO classification of tumours of haematopoietic and lymphoid tissues (Revised 4th edition). IARC; 2017.
Zhou Y, Wang H, Fang W, Romaguer JE, Zhang Y, Delasalle KB, Kwak L, Yi Q, Du XL, Wang M. Incidence trends of mantle cell lymphoma in the United States between 1992 and 2004. Cancer. 2008;113:91–8.
Fu S, Wang M, Lairson DR, Li R, Zhao B, Du XL. Trends and variations in mantle cell lymphoma incidence from 1995 to 2013: a comparative study between Texas and National SEER areas. Oncotarget. 2017;8:112516–29.
Vose JM. Mantle cell lymphoma: 2017 update on diagnosis, risk-stratification, and clinical management. Am J Hematol. 2017;92:806–13.
Mozos A, Royo C, Hartmann E, De Jong D, Baró C, Valera A, Fu K, Weisenburger DD, Delabie J, Chuang SS, Jaffe ES, Ruiz-Marcellan C, Dave S, Rimsza L, Braziel R, Gascoyne RD, Solé F, López-Guillermo A, Colomer D, Staudt LM, Rosenwald A, Ott G, Jares P, Campo E. SOX11 expression is highly specific for mantle cell lymphoma and identifies the cyclin D1-negative subtype. Haematologica. 2009;94(11):1555–62. [published correction appears in Haematologica. 2010;95(9):1620].
Lukach L, Lehman H, Livoff A, Nahlieli O, Michaeli E, Allon I. Mantle cell lymphoma of the oral cavity: an uncommon site for an uncommon lesion, two new cases and literature review. Oral Oncol. 2020;103:1045–78.
Fitzpatrick SG, Al-Quran SZ, Fetner AE, Kuklan RM, Cohen DM, Bhattacharyya I. Mantle cell lymphoma of the hard palate: a series of 4 cases. Oral Surg. 2012;5:76–83.
Klapper W. Histopathology of mantle cell lymphoma. Semin Hematol. 2011;48:148–54.
Young KH, Chan WC, Fu K, Iqbal J, Sanger WG, Ratashak A, Greiner TC, Weisenburger DD. Mantle cell lymphoma with plasma cell differentiation. Am J Surg Pathol. 2006;30:954–61.
Jain P, Zhang S, Kanagal-Shamanna R, Ok CY, Nomie K, Gonzalez GN, Gonzalez-Pagan O, Hill HA, Lee HJ, Fayad L, Westin J, Nastoupil L, Hagemeister F, Chen W, Oriabure O, Badillo M, Jiang C, Yixin Y, Li S, Tang G, Yin CC, Patel KP, Medeiros LJ, Nair R, Ahmed S, Iyer SP, Thirumurthi S, Champlin R, Xu G, Tinsu P, Santos D, Wang R, Han G, Zhang J, Song X, Neelapu S, Romaguera J, Futreal A, Flowers C, Fowler N, Wang L, Wang ML. Genomic profiles and clinical outcomes of de novo blastoid/pleomorphic MCL are distinct from those of transformed MCL. Blood Adv. 2020;4:1038–50.
Chuang WY, Chang H, Chang GJ, Wang TH, Chang YS, Wang TH, Yeh CJ, Ueng SH, Chien HP, Chang CY, Wan YL, Hsueh C. Pleomorphic mantle cell lymphoma morphologically mimicking diffuse large B cell lymphoma: common cyclin D1 negativity and a simple immunohistochemical algorithm to avoid the diagnostic pitfall. Histopathology. 2017;70:986–99.
Kolokotronis A, Konstantinou N, Christakis I, Papadimitriou P, Matiakis A, Zaraboukas T, Antoniades D. Localized B-cell non-Hodgkin’s lymphoma of oral cavity and maxillofacial region: a clinical study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2005;99:303–10.
Tan K, Tan L, Soo R, Putti T, Chong S. Involvement of the appendix and palate by pleomorphic variant mantle cell lymphoma. Leuk Lymphoma. 2006;47:1704–7.
Rockacy J, Viozzi C, Zent C. Mantle cell lymphoma presenting as a slowly enlarging lesion of the floor of mouth in a healthy 72-year-old female: report of a case. J Oral Maxillofac Surg. 2007;65:333–7.
Guastafierro S, Falcone U, Celentano M, Cappabianca S, Giudice A, Colella G. Primary mantle cell lymphoma of the tongue. Int J Hematol. 2008;88:206–8.
Guggisberg K, Jordan R. Mantle cell lymphoma of the oral cavity: case series and comprehensive review of the literature. Oral Surg Oral Med Oral Pathol. 2010;109:98–104.
Kyo C, Kawaoka Y, Kinoshita K, Ohno H. Mantle cell lymphoma presenting with a tumor of the hard palate. Intern Med. 2010;49:1663–6.
Scheller K, Becker S, Scheller C. Symmetric palatal swelling as the first clinical manifestation of a mantle cell non-Hodgkin’s lymphoma: a case report and review of literature. J Oral Maxillofac Pathol. 2011;15:311–5.
Milgrom SA, Yahalom J. Indolent non-Hodgkin lymphoma primarily involving the hard palate: outcome following radiotherapy. Leuk Lymphoma. 2013;54:1208–11.
Dereci O, Ay S, Açıkalın MF, Karagülle M. III-fitting dentures as primary presentation of mantle cell lymphoma: a case report and literature review of the primary mantle cell lymphomas of the hard palate. J Oral Maxillofac Pathol. 2015;19:255–9.
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.
Mundada MC, Ahmed F, Murthy SS, Mohan MK. Mantle cell lymphoma of tongue masquerading as squamous cell carcinoma. Int J Oral Health Sci. 2015;5:121–4.
Ainscough S, Power AM, Brown AN. Mantle cell lymphoma: primary oral presentation. Ann R Coll Surg Engl. 2016;23:e1–2.
Marcucci M, Marcucci G, Cordeiro MS, León JE, Araujo JP, de Oliveira JX, Santos KCP. Mantle cell lymphoma: involvement of nodal and extranodal sites in the head and neck, with multifocal oral lesions. Autops Case Rep. 2017;7:26–31.
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.
Zhou Y, Wang H, Fang W, Romaguer JE, Zhang Y, Delasalle KB, Kwak L, Yi Q, Du XL, Wang M. Incidence trends of mantle cell lymphoma in the United States between 1992 and 2004. Cancer. 2008;113(4):791–8.
Dreyling M, Klapper W, Rule S. Blastoid and pleomorphic mantle cell lymphoma: still a diagnostic and therapeutic challenge. Blood. 2018;132(26):2722–9.
Liu Z, Dong HY, Gorczyca W, Tsang P, Cohen P, Stephenson CF, Berger CS, Wu CD, Weisberger J. CD5- mantle cell lymphoma. Am J Clin Pathol. 2002;118(2):216–24.
Miao Y, Lin P, Saksena A, Xu J, Wang M, Romaguera J, Yin CC, Medeiros LJ, Li S. CD5-negative mantle cell lymphoma: clinicopathologic correlations and outcome in 58 patients. Am J Surg Pathol. 2019;43(8):1052–60.
Soleimani A, Tanios G, Safah H, Saba NS. A systemic review of CD5-negative mantle cell lymphoma identifies potential clinical and biological implications. Blood. 2016;128:3048.
Hoster E, Klapper W, Hermine O, Kluin-Nelemans HC, Walewski J, van Hoof A, Trneny M, Geisler CH, Di Raimondo F, Szymczyk M, Stilgenbauer S, Thieblemont C, Hallek M, Forstpointner R, Pott C, Ribrag V, Doorduijn J, Hiddemann W, Dreyling MH, Unterhalt M. Confirmation of the mantle-cell lymphoma International Prognostic Index in randomized trials of the European Mantle-Cell Lymphoma Network. J Clin Oncol. 2014;32(13):1338–46.
Ladha A, Zhao J, Epner EM, Pu JJ. Mantle cell lymphoma and its management: where are we now? Exp Hematol Oncol. 2019;8:2.
Hoster E, Dreyling M, Klapper W, Gisselbrecht C, van Hoof A, Kluin-Nelemans HC, Pfreundschuh M, Reiser M, Metzner B, Einsele H, Peter N, Jung W, Wörmann B, Ludwig WD, Dührsen U, Eimermacher H, Wandt H, Hasford J, Hiddemann W, Unterhalt M. German Low Grade Lymphoma Study Group (GLSG); European Mantle Cell Lymphoma Network. A new prognostic index (MIPI) for patients with advanced-stage mantle cell lymphoma. Blood. 2008;111(2):558–65.
Fu S, Wang M, Li R, Lairson DR, Zhao B, Du XL. Increase in survival for patients with mantle cell lymphoma in the era of novel agents in 1995–2013: findings from Texas and national SEER areas. Cancer Epidemiol. 2019;58:89–97.
Wu H, Wang J, Zhang X, Yang H, Wang Y, Sun P, Cai Q, **a Y, Liu P. Survival trends in patients under age 65 years with mantle cell lymphoma, 1995–2016: a SEER-based analysis. Front Oncol. 2020;10:588314.
Ambinder AJ, Shenoy PJ, Nastoupil LJ, Flowers CR. Using primary site as a predictor of survival in mantle cell lymphoma. Cancer. 2013;119(8):1570–7.
Acknowledgements
We would like to thank Dr. Savvas Titsinides for his contribution to patient’s diagnosis and management.
Funding
The authors did not receive support from any organization for the submitted work. No funds, grants, or other support was received.
Author information
Authors and Affiliations
Contributions
All authors contributed to the study conception and design. All authors read and approved the final manuscript. Conceptualization: MG, VIT, NGN; Methodology: MG, VIT, NGN; Formal analysis and investigation: MG, VIT, NGN; Writing—original draft preparation: MG, VIT, EP; Writing—review and editing: EP, PP, NGN; Supervision: NGN.
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflicts of interest to declare that are relevant to the content of this article.
Ethical Approval
Ethics approval was waived by the local Ethics Committee of School of Dentistry, National and Kapodistrian University of Athens, Greece in view of the retrospective nature of the study and the fact that all procedures were part of the routine care, based on institutional regulations.
Informed Consent
No informed consent was obtained due to retrospective nature of the case and since only intra-oral images and pathology slides were used. No consent was obtained from the participant due to retrospective nature of the case and since only intra-oral images and pathology slides were used.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Georgaki, M., Theofilou, V.I., Pettas, E. et al. Blastoid Mantle Cell Lymphoma of the Palate: Report of a Rare Aggressive Entity and Review of the Literature. Head and Neck Pathol 16, 631–642 (2022). https://doi.org/10.1007/s12105-021-01391-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12105-021-01391-9