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 Table of Contents  
Year : 2017  |  Volume : 4  |  Issue : 1  |  Page : 51-54

Non-Hodgkin's lymphoma of the maxilla: A rare case report and review

1 Department of Oral and Maxillofacial Surgery, Haldia Institute of Dental Sciences and Research, Haldia, West Bengal, India
2 Department of Oral Pathology and Microbiology, Haldia Institute of Dental Sciences and Research, Haldia, West Bengal, India
3 Department of Oral Pathology and Microbiology, Index Institute of Dental Sciences, Indore, Madhya Pradesh, India

Date of Web Publication14-Feb-2017

Correspondence Address:
Arpita Kabiraj
Department of Oral Pathology and Microbiology, Index Institute of Dental Sciences, Indore, Madhya Pradesh - 452 016
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sjos.SJOralSci_41_16

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Non-Hodgkin's lymphomas (NHLs) embody a diverse group of malignancies that originate from the lymphoid system. NHL often exhibit in an extranodal pattern, pertaining to the head and neck region. Intraoral sites are much less frequent, accounting for approximately 3.5% of all oral malignancies. Although the exact cause of NHL still remains inconspicuous, however, research has focused on some factors that may contribute to the development of lymphoma, including genetic factors, impaired immune system and viruses, such as HIV or EBV. Clinically, the bony lesion may present as localized or diffuse swelling, with low-grade pain, sweating, unexplained weight loss, fever, etc. Radiographically, these lesions resemble osteomyelitis or other malignancies creating a diagnostic dilemma. Microscopically, diffused lymphomas consist of large tumor cells with large nuclei that are more than twice the size of lymphocytes which may either exhibit centroblastic or immunoblastic features. Here, we report a rare case of NHL affecting the jaws of a 60-year-old male patient.

Keywords: Centroblast, extranodal, lymphoreticular, malignancy

How to cite this article:
Banerjee R, Ghosh AK, Misra M, Kabiraj A. Non-Hodgkin's lymphoma of the maxilla: A rare case report and review. Saudi J Oral Sci 2017;4:51-4

How to cite this URL:
Banerjee R, Ghosh AK, Misra M, Kabiraj A. Non-Hodgkin's lymphoma of the maxilla: A rare case report and review. Saudi J Oral Sci [serial online] 2017 [cited 2022 Sep 28];4:51-4. Available from: https://www.saudijos.org/text.asp?2017/4/1/51/200142

  Introduction Top

Malignant lymphomas represent a heterogeneous and diverse group of neoplasms of the lymphoid tissue chiefly involving the lymphocyte cell lines; with vivid clinical outcomes and presentation depending on the treatment protocol and prognosis.[1],[2] Lymphomas can be broadly categorized into Hodgkin's and non-Hodgkin's type. Among these, Hodgkin's disease is typified histologically by the presence of multinucleated giant cells known as Reed–Sternberg cells. All other neoplasms of lymphoreticular system are referred to as non-Hodgkin's lymphoma (NHL) and are derived predominantly from the cells of B-lymphocyte series.[3] Usually, the oral manifestations of NHL are secondary to a more widespread involvement throughout the body, however, it can rarely present as a primary lesion in the oral cavity, having 0.1–0.2% prevalence.[2]

NHL arises primarily within the lymph nodes, but approximately 24% affect extranodal locations. The most frequent extranodal sites include the gastrointestinal tract, skin, bones, and Waldeyer's ring. NHL of bone is rare, representing only 5% of all extranodal lymphomas with the mandible accounting for only 0.6%. This uncommon localization can pose significant diagnostic problems and is frequently misdiagnosed.[4] Here, we present an unusual case of primary NHL of the mandible.

  Case Report Top

A 60-year-old male patient reported with swelling on the right cheek region since 1 year. The patient gave a history of swelling which was initially small and gradually increased to attain the present size. The swelling was associated with mild intermittent pain, pus discharge, and paresthesia. The patient reported to be diabetic and hypertensive but claimed to be under control. He had undergone multiple extractions which were uneventful. The vital signs were within normal limits.

On extraoral examination, diffuse swelling was seen in the right middle-third of face measuring approximately 4 cm × 3 cm in size. The swelling extended from the right lateral surface of the nose to 2 cm in front of the tragus of ear anteroposteriorly and from infraorbital plane to angle of mouth region superioinferiorly obliterating the right nasolabial fold. The surface of the swelling appeared erythematous on the nasal side and smooth [Figure 1]. On palpation, there was local rise of temperature; the swelling was mild tender and firm to hard in consistency. Right submandibular and upper cervical lymph nodes were palpable, tender, hard in consistency and fixed to underlying structures. Intraorally, ulceroproliferative growth was seen in the right maxillary alveolar region extending from premolars to maxillary tuberosity which was tender, hard in consistency with purulent discharge, everted margins, and indurated base. Based on history and clinical findings, a provisional diagnosis of malignant ulcer of the right maxillary posterior alveolar region was thought of.
Figure 1: Extraoral phototgraph of the patient showing diffused swelling in the right middle third of face

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The patient was subjected to various investigations. Axial section of computed tomography revealed hypodense areas extending distal to right maxillary canine to tuberosity region [Figure 2]. Therefore, incisional biopsy was planned, and the specimen was sent for histopathological analysis. The photomicrograph showed the presence of connective tissue matrix with numerous diffused tumor cells infiltrating soft tissue matrix [Figure 3]. These cells were chiefly composed of small lymphocytes with hyperchromatic nuclei, slightly irregular, with extensively isolated figures of mitosis. The nuclei of the tumor cells were vesiculosus and of the irregular morphology, with atypical nucleoli and figures [Figure 4]. Based on the histopathological features, a final diagnosis of NHL was considered.
Figure 2: Axial section of computed tomography showing hypodense areas extending distal to right maxillary canine to tuberosity region

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Figure 3: H and E stained section showing presence of connective tissue matrix with numerous diffused tumor cells infiltrating soft tissue matrix

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Figure 4: Tumor cells composed of small lymphocytes with hyperchromatic irregular nuclei and extensively isolated figures of mitosis

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  Discussion Top

NHL embodies a diverse group of malignancies originating from the lymphoreticular system.[5] They principally engross lymph nodes, spleen, and other nonhemopoietic tissues of the body. It is proposed that approximately 24%–40% of NHLs prevail in the extranodal sites (primary or occult); among which maximum number of cases is seen in the head and neck region accounting for 2%–3% of them in the oral cavity and jaws. This is the most significant differentiating feature between NHL and Hodgkin's disease.[2] Waldeyer's ring is considered to be the most common site of NHL in orofacial region whereas the association of the jaws by the neoplasm is rare; maxilla being more frequently involved than the mandible.[3] It was Parker and Jackson who described primary lymphoma of bone as primary reticular cell sarcoma. Due to the low frequency, nonspecific presentations and resemblance to other common dental diseases; primary NHLs have often been misdiagnosed.[4],[6],[7]

There are no typical clinical presenting features when jaw bones are concerned. These lesions can present as swelling of the jaw, pain, numbness, tooth mobility, or cervical lymphadenopathy.[3] Few lesions have been associated with painless enlargement of lymph nodes attributing to be the most common presenting symptom. These features were found in concordance to our present reported case. The clinical presentation epitomizes similar to an odontogenic process or localized osteomyelitis. Lesions are sometimes thought to be inflammatory or infectious, therefore delaying diagnosis as was the case seen with the patient presented here. The clinical presentation of mandibular NHL usually involves localized bone swelling, teeth mobility, mass in an extraction socket, pain, pathologic fracture and often anesthesia or paresthesia along the distribution of the inferior alveolar nerve.[8],[9] In our case report, the lesion was seen to be affecting the right maxillary region.

Guevara-Canales et al. conducted a systematic review of 15 studies and a total 714 patients and proposed that the most frequent intraoral site for NHL was gingiva, followed by palate. It has been postulated in many studies and reports that primary oral lymphomas present as a soft to firm tissue mass which is usually nontender and may or may not be ulcerated. This lesion can imitate periodontal or apical abscesses at the time. Bony lesions, however, often present with low-grade pain which can mimic a toothache making the diagnosis difficult. Radiographically, these bony lesions appear very similar to osteomyelitis or other malignancies creating, even more, difficulty in diagnosis.[10] Microscopically, diffused lymphomas consist of large tumor cells with large nuclei that are more than twice the size of lymphocytes. These tumor cells show either centroblastic or immunoblastic features. The centroblasts have round or irregular nuclei and membrane-bound nucleoli, while the immunoblasts consist of round nuclei and centrally located prominent nucleoli.[11] Histopathologic evaluation along with immunophenotypic and cytogenetic studies, elucidate the histologic type. Immunohistochemistry plays a pertinent role in distinguishing cell types and differential diagnosis. Although a panel of monoclonal antibodies are thought to be used that includes CD20 (selective marker for subpopulation of B-cells), CD10 (marker for follicular center B-cells), CD3 (marker for T-cells and natural killer cells), CD30 (marker for activated T- and B-cells), ALK-1 (marker for anaplastic large cells), and Ki-67 (proliferation marker). However, in our case, immunohistochemistry was not performed due to restricted resources.[12]

The diagnosis of oral lymphomas may be exigent due to a frequent low index of clinical acumen, sometimes leading to misdiagnosis and/or delayed treatment. Thus, biopsies should be vigilantly performed to obtain adequate specimens. There are different recent trends in diagnosis of lymphomas, although standard hematoxylin and eosin stained sections still prove to be the root for all lymphoma diagnosis. NHL can involve facial bones although it is rare with only mild and nonspecific symptoms. Therefore, it is imperative that health-care providers be more attentive about this malignancy and conduct a complete physical examination, along with proper radiological and histopathological investigations to identify the disease at an earlier stage.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Vicente-Barrero M, Garcia-Castro I, Knezevic M, Castellano-Reyes JJ, Garcia-Jimenez F, Camacho-Garcia MC, et al. Non-Hodgkin lymphomas of oral cavity. EPOJ 2002;59:669-73.  Back to cited text no. 1
Alirezaei S, Baharvand M, Tavakoli B, Sarikhani S, Mafi AR. Advanced primary lymphoma of oral cavity: Report of a case. Open J Stomatol 2014;4:109-14.  Back to cited text no. 2
Kini R, Saha A, Naik V. Diffuse large B-cell lymphoma of mandible: A case report. Med Oral Patol Oral Cir Bucal 2009;14:e421-4.  Back to cited text no. 3
Adouani A, Bouguila J, Jeblaoui Y, Ben Aicha M, Abdelali MA, Hellali M, et al. B-cell lymphoma of the mandible: A case report. Clin Med Oncol 2008;2:445-50.  Back to cited text no. 4
Nogai H, Dörken B, Lenz G. Pathogenesis of non-Hodgkin's lymphoma. J Clin Oncol 2011;29:1803-11.  Back to cited text no. 5
Longo F, De Maria G, Esposito P, Califano L. Primary non-Hodgkin's lymphoma of the mandible. Report of a case. Int J Oral Maxillofac Surg 2004;33:801-3.  Back to cited text no. 6
Cox DP, Treseler P, Dong R, Jordan RC. Rare oral cavity presentation of a B-cell lymphoblastic lymphoma. A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;103:814-9.  Back to cited text no. 7
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. 8
Leong IT, Fernandes BJ, Mock D. Epstein-Barr virus detection in non-Hodgkin's lymphoma of the oral cavity: An immunocytochemical and in situ hybridization study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2001;92:184-93.  Back to cited text no. 9
Guevara-Canales JO, Morales-Vadillo R, de Faria PE, Sacsaquispe-Contreras SJ, Leite FP, Chaves MG. Systematic review of lymphoma in oral cavity and maxillofacial region. Acta Odontol Latinoam 2011;24:245-50.  Back to cited text no. 10
Bugshan A, Kassolis J, Basile J. Primary diffuse large B-Cell lymphoma of the mandible: Case report and review of the literature. Case Rep Oncol 2015;8:451-5.  Back to cited text no. 11
Steinbacher DM, Dolan RW. Isolated non-Hodgkin's lymphoma of the mandible. Oral Oncol 2006;42:187-9.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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