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| Year : 2013 | Volume
: 24
| Issue : 3 | Page : 384-386 |
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| B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma: Report of a case in the oral cavity |
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Jumana M Jaradat, Anitha Potluri, Elizabeth A Bilodeau
Department of Diagnostic Sciences, University of Pittsburgh, School of Dental Medicine, Pittsburgh, United States of America
Click here for correspondence address and email
| Date of Submission | 08-Dec-2012 |
| Date of Decision | 08-Feb-2013 |
| Date of Acceptance | 05-Mar-2013 |
| Date of Web Publication | 12-Sep-2013 |
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 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 2023 Mar 30];24:384-6. Available from: https://www.ijdr.in/text.asp?2013/24/3/384/118016 |
| Case Presentation | |  |
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 | | |
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]
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Correspondence Address:
Jumana M Jaradat
Department of Diagnostic Sciences, University of Pittsburgh, School of Dental Medicine, Pittsburgh
United States of America
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0970-9290.118016
[Figure 1], [Figure 2] |
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