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Table of Contents   
CASE REPORT  
Year : 2013  |  Volume : 24  |  Issue : 3  |  Page : 384-386
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma: Report of a case in the oral cavity


Department of Diagnostic Sciences, University of Pittsburgh, School of Dental Medicine, Pittsburgh, United States of America

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Date of Submission08-Dec-2012
Date of Decision08-Feb-2013
Date of Acceptance05-Mar-2013
Date of Web Publication12-Sep-2013
 

   Abstract 

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma (DLBCL/BL) is a new category of B-cell lymphoma according to the 4 th edition of the World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues (2008). The following report presents a case of this rare, newly described entity on the palate of a 59 year-old male.

Keywords: Burkitt lymphoma, diffuse large B-cell lymphoma, lymphoma, MYC, oral cavity

How to cite this article:
Jaradat JM, Potluri A, Bilodeau EA. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma: Report of a case in the oral cavity. Indian J Dent Res 2013;24:384-6

How to cite this URL:
Jaradat JM, Potluri A, Bilodeau EA. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma: Report of a case in the oral cavity. Indian J Dent Res [serial online] 2013 [cited 2019 Nov 18];24:384-6. Available from: http://www.ijdr.in/text.asp?2013/24/3/384/118016

   Case Presentation Top


A 59-year-old Caucasian male presented with slight swelling and sharp pain in the right maxilla. The patient's surgical history was significant for a LeFort I osteotomy and mandibular bilateral sagittal split osteotomy surgery 19 years previously. His medical history was noncontributory. The initial clinical concern was infection involving the maxillary hardware. The patient was treated with several courses of antibiotics with no significant improvement. Later, the patient developed mobility of the maxillary right dentition and progressive swelling of the right hard palate extending into the buccal vestibule.

Computed tomography (CT) without contrast revealed a destructive lesion of the right maxilla involving the buccal plate and palatal bone with perforation. Significant erosive changes of the right alveolar ridge and maxillary sinus wall were seen [Figure 1]. Soft tissue thickening was noted in the floor of the maxillary sinus. Plates and screws from the previous LeFort I osteotomy were still present but with compromised osseointegration due to the destructive nature of the lesion.
Figure 1: Computed tomography bone window with erosive bony changes and compromised osseointegration of hardware

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A biopsy and removal of hardware was performed. The tumor was composed predominantly of a diffuse proliferation of medium to large pleomorphic cells with occasional prominent nucleoli. Also, there were focal areas of extensive necrosis, crush artifact, and bony infiltration [Figure 2]. Immunohistochemical staining revealed strong Ki-67 staining, indicative of a high proliferative rate. The tumor cells were strongly positive for CD20 (a B-cell marker), Pax-5 (a B-cell marker), bcl-6 (a normal follicular center cell marker), and weakly positive for CD10 (a B-cell marker). Immunohistochemical staining for CD-3 (a T-cell marker), anti-κ (a plasma cell marker), and anti-λ (a plasma cell marker) revealed scattered positive cells. Cyclin D1 (a marker of mantle cell lymphoma), bcl-2 (lymphocyte subset), and CD21 (a follicular dendritic cell marker) were negative.
Figure 2: H & E, ×40, biopsy revealed a large pleomorphic lymphoid cell population (arrows) with crush artifact (asterisks)

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To further characterize the tumor, fluorescence in situ hybridization (FISH) studies were performed on paraffin sections. They were positive for MYC gene rearrangement and negative for BCL-2, BCL-6, and IgH gene rearrangements. A bone marrow biopsy and peripheral blood smear, performed for staging purposes, yielded no evidence of tumoral involvement.

A positron emission tomography (PET)-CT scan showed a destructive osseous lesion in the right maxilla and adjacent soft tissue with increased uptake of fludeoxyglucose (FDG). Prominent cervical lymph nodes are noted but did not demonstrate any uptake of FDG, likely representative of reactive nodes.

The clinical, radiographic, immunohistochemical, and FISH studies were supportive of a high-grade non-Hodgkin B-cell lymphoma. Although the morphologic features in our case were not characteristic for Burkitt lymphoma (BL) and were most consistent with a diffuse large B-cell lymphoma (DLBCL), the FISH findings and the immunophenotypic features were compatible with BL. Therefore, the diagnosis of B-cell lymphoma with features intermediate between DLBCL and BL was favored.

The patient is still in remission after six cycles of R-CHOP with PET/CT scan showing no evidence of active disease or nodal involvement.


   Discussion Top


Primary lymphomas in the head and neck region represents the third most common malignancies after squamous cell carcinomas and salivary gland tumors. [1] The two major categories of malignant lymphomas are non-Hodgkin's lymphoma (NHL) and Hodgkin's lymphoma (HL). In contrast to HL, NHLs have a propensity to spread to extranodal locations, [2] with approximately 40% arises at extranodal sites. [3] In the head and neck region, Waldeyer's ring is the most common site of extranodal NHL. [2],[4]

Lymphomas of the oral cavity are uncommon, accounting for less than 5% of the reported cases, with DLBCL being the most frequent type. [4],[5] The most common site of extranodal NHL in the oral region is the palate. [6] Patients with intraoral lymphoma often present with nonspecific signs such as swelling, ulcerated mass, and radiographic bone destruction. [6] Frequently, such lesions are initially mistaken for inflammatory or reactive processes. In this case, the initial clinical and radiographic impression was hardware failure and osteomyelitis.

In the 4 th edition of the World Health Organization (WHO) Classification of Tumours of Hematopoietic and Lymphoid Tissues (2008), DLBCL and BL have been defined precisely using the morphologic, immunophenotypic, and cytogenetic features to keep such entities as pure as possible. [7] However, there are cases that share many features of both BL and DLBCL. Attempts to assign such borderline cases to BL or DLBCL are nonreproducible and confusing, even when using cytogenetic and immunophenotypic studies. [8],[9] The separation between BL and DLBCL is important for treatment decisions and prognosis. [8],[9] For these reasons, the WHO 2008 classification established a new provisional category of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma. Lymphomas in this category usually demonstrate either: A BL phenotype with atypical immunophenotype and genetic features, or a BL immunophenotype with variable nuclear size intermediate between BL and DLBCL. [10] This intermediate category is not a distinct entity but rather is considered as a heterogeneous group of aggressive lymphomas. [11] Therefore, future subclassification combining morphologic, immunophenotypic, and cytogenetic features is warranted.

MYC is a proto-oncogene that plays an important role in cell growth and apoptosis. [12] Aggressive B-cell lymphomas that have been found to harbor MYC rearrangement include BL (90%), [13] DLBCL (10%), [14] and DLBCL/BL (up to 58%). [15],[16] In some cases of DLBCL/BL with MYC rearrangement, there is a concurrent rearrangement of the antiapoptotic BCL2 oncogene, which are referred to as ''double-hit'' or ''triple-hit'' lymphomas. [17] The outcome of DBCL/BL is poor irrespective of the treatment regimens. This aggressive nature is likely due to the concurrent rearrangement of both the proproliferative MYC oncogene and the antiapoptotic BCL2 oncogene. [17]

 
   References Top

1.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.  Back to cited text no. 1
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2.Kolokotronis A, Konstantinou N, Christakis I, Papadimitriou P, Matiakis A, Zaraboukas T, et al. 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.  Back to cited text no. 2
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3.Otter R, Gerrits WB, vd Sandt MM, Hermans J, Willemze R. Primary extranodal and nodal non-Hodgkin's lymphoma. A survey of a population-based registry. Eur J Cancer Clin Oncol 1989;25:1203-10.  Back to cited text no. 3
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4.Bhattacharyya I, Chehal HK, Cohen DM, Al-Quran SZ. Primary diffuse large B-cell lymphoma of the oral cavity: Germinal center classification. Head Neck Pathol 2010;4:181-91.  Back to cited text no. 4
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5.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 Oral Radiol Endod 2001;92:519-25.  Back to cited text no. 5
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6.Kemp S, Gallagher G, Kabani S, Noonan V, O'Hara C. Oral non-Hodgkin's lymphoma: Review of the literature and World Health Organization classification with reference to 40 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;105:194-201.  Back to cited text no. 6
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7.de Jong D. Novel lymphoid neoplasms-the borderland between diffuse large B-cell lymphoma and Burkitt's lymphoma. Haematologica 2009;94:894-6.  Back to cited text no. 7
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8.Haralambieva E, Boerma EJ, van Imhoff GW, Rosati S, Schuuring E, Müller-Hermelink HK, et al. Clinical, immunophenotypic, and genetic analysis of adult lymphomas with morphologic features of Burkitt lymphoma. Am J Surg Pathol 2005;29:1086-94.  Back to cited text no. 8
    
9.Carbone A, Gloghini A, Aiello A, Testi A, Cabras A. B-cell lymphomas with features intermediate between distinct pathologic entities. From pathogenesis to pathology. Hum Pathol 2010;41:621-31.  Back to cited text no. 9
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10.Salaverria I, Siebert R. The gray zone between Burkitt's lymphoma and diffuse large B-cell lymphoma from a genetics perspective. J Clin Oncol 2011;29:1835-43.  Back to cited text no. 10
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11.Kluin PH, Stein H, Leoncini L. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma. In: Swerdlow S, editor. WHO Classification of Tumours of the Haematopoietic and Lymphoid Tissues. 4 th ed. Lyon: International Agency for Research on Cancer; 2008. p. 265-6.  Back to cited text no. 11
    
12.Slack GW, Gascoyne RD. MYC and aggressive B-cell lymphomas. Adv Anat Pathol 2011;18:219-28.  Back to cited text no. 12
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13.Choi SY, Kim SJ, Kim WS, Kim K, Ko YH. Aggressive B cell lymphomas of the gastrointestinal tract: Clinicopathologic and genetic analysis. Virchows Arch 2011;459:495-502.  Back to cited text no. 13
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14.van Imhoff GW, Boerma EJ, van der Holt B, Schuuring E, Verdonck LF, Kluin-Nelemans HC, et al. Prognostic impact of germinal center-associated proteins and chromosomal breakpoints in poor-risk diffuse large B-cell lymphoma. J Clin Oncol 2006;24:4135-42.  Back to cited text no. 14
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15.Hummel M, Bentink S, Berger H, Klapper W, Wessendorf S, Barth TF, et al. A biologic definition of Burkitt's lymphoma from transcriptional and genomic profiling. N Engl J Med 2006;354:2419-30.  Back to cited text no. 15
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16.McClure RF, Remstein ED, Macon WR, Dewald GW, Habermann TM, Hoering A, et al. Adult B-cell lymphomas with burkitt-like morphology are phenotypically and genotypically heterogeneous with aggressive clinical behavior. Am J Surg Pathol 2005;29:1652-60.  Back to cited text no. 16
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17.de Leval L, Hasserjian RP. Diffuse large B-cell lymphomas and burkitt lymphoma. Hematol Oncol Clin North Am 2009;23:791-827.  Back to cited text no. 17
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Top
Correspondence Address:
Jumana M Jaradat
Department of Diagnostic Sciences, University of Pittsburgh, School of Dental Medicine, Pittsburgh
United States of America
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9290.118016

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    Figures

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