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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 5  |  Issue : 1  |  Page : 50-52

Primary Non-Hodgkin's lymphoma of the tongue: A rare presentation


Department of ENT and Head and Neck Surgery, Mahatma Gandhi Medical College and Research Institute, Puducherry, India

Date of Web Publication1-Jul-2016

Correspondence Address:
Satvinder Singh Bakshi
House Number B2, Shree Pushpa Complex, 15th Bharathi Street, Ananda Nagar, Puducherry - 605 009
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2277-4696.185184

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  Abstract 

The head and neck is the second most common region for extranodal lymphomas. The most common site is the Waldeyer's ring, and involvement of the base of tongue is extremely rare. We present a rare case of a young female with primary non-Hodgkin's lymphoma (NHL) of the base of tongue. A 23-year-old female presented with a history of foreign body sensation in her throat for a month. Oral examination revealed a lobulated smooth mass at the base of tongue. Contrast-enhanced computed tomography neck shows polypoidal homogeneously enhancing soft tissue lesion in the base of tongue extending till the lateral pharyngeal wall. The biopsy of the lesion was reported as NHL. Hodgkin's lymphoma should be kept in the differential diagnosis of swelling arising from the base of tongue. We report a rare and varied presentation of extranodal lymphoma. A careful clinical evaluation supported by histopathological and radiologic investigations will help in identifying the disease at an early stage, resulting in a better prognosis.

Keywords: Base of tongue, extranodal lymphoma, non-Hodgkin's lymphoma


How to cite this article:
Karanam L, Bakshi SS, Priya M, Coumare V N, Vijayasundaram S. Primary Non-Hodgkin's lymphoma of the tongue: A rare presentation. J Dent Allied Sci 2016;5:50-2

How to cite this URL:
Karanam L, Bakshi SS, Priya M, Coumare V N, Vijayasundaram S. Primary Non-Hodgkin's lymphoma of the tongue: A rare presentation. J Dent Allied Sci [serial online] 2016 [cited 2019 Sep 18];5:50-2. Available from: http://www.jdas.in/text.asp?2016/5/1/50/185184


  Introduction Top


Lymphomas are malignant neoplasms of the lymphocyte cell lines affecting the lymph nodes, spleen, and other nonhemopoietic tissues. They are classified as either Hodgkin's or non-Hodgkin's lymphoma (NHL), either B-lymphocyte or T-lymphocyte origin. The most common site for extranodal lymphomas is the gastrointestinal tract followed by the head and neck region.[1] NHL can range from being relatively indolent to highly aggressive and potentially fatal.

Approximately, 2.5% of malignant lymphomas arise in the oral and paraoral region, mainly from Waldeyer's ring.[1] The involvement of the base of tongue is extremely rare. The incidence is more in males with a median age of 59 years.[2] We report a rare case of NHL of the base of tongue in a young immunocompetent female.


  Case Report Top


A 23-year-old female presented with 1-month history of foreign body sensation in her throat associated with mild difficulty in swallowing. There was no history change in voice or difficulty in breathing. There were no other medical comorbidities.

On examination, a lobulated, smooth, nonulcerated, pinkish mass approximately 2 cm × 2 cm was seen at the base of tongue [Figure 1]. Digital palpation revealed a firm and large swelling involving the pharyngeal tongue, especially on the left side. The mobility of the tongue was unaffected. Videolaryngoscopic examination revealed that the mass was confined to the base of tongue extending laterally with no visible extension of the mass into hypopharynx. Neck examination revealed no palpable neck nodes. Her systemic examination was normal. Investigations such as hemogram, urine analysis, and chest X-ray were normal.
Figure 1: Smooth lobulated pinkish mass arising from the base of tongue

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Contrast-enhanced computed tomography (CT) neck showed a polypoidal homogeneous moderately enhancing soft tissue lesion measuring 23 mm × 24 mm × 18 mm arising from base of tongue on the left side, extending to the lateral pharyngeal wall to just above the epiglottis [Figure 2].
Figure 2: Homogeneous lesion seen arising from the base of tongue extending to the lateral pharyngeal wall on the left side

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Biopsy of the oropharyngeal mass showed highly cellular smear composed predominantly of monotonous lymphoid population of medium-sized cells with vesicular nuclei and predominant nucleoli resembling lymphoma cells on the background of scanty mature lymphocytes and tangible body macrophages, Features suggestive of NHL. Immunohistochemical (IHC) analysis was positive for leukocyte common antigen (LCA) and CD20, and final report was confirmatory of NHL.

The CT scan of thorax and abdomen was normal, and the patient was staged as Ann Arbor Stage IE.

The patient was treated with four cycles of cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP) regimen chemotherapy and is asymptomatic at 5 months of follow-up.


  Discussion Top


Lymphomas are heterogeneous group of clonal malignant diseases, arising as a result of somatic mutation in lymphocyte progenitor cells either B-cell or T-cell or both and can range from relatively indolent to aggressive forms.[2]

Lymphomas can be classified as Hodgkin's lymphoma (HL) or NHL.[3] Primary extranodal origin implies that the disease does not originate from lymph nodes or the tissues containing lymphoid follicles. HL rarely shows extranodal disease (1% cases) in contrast to NHL (23–30% cases).[4] It is difficult to diagnose the extranodal lymphomas as they do not have the typical presenting features of NHL.

Typical location of extranodal NHL includes gastrointestinal tract, Waldeyer's ring, and skin. The most common malignancy in the oral cavity is squamous cell carcinomas and lymphomas constitute only 2% of the total tumors.[5]

According to van der Waal et al.,[2] median age of patients with oral NHL was 59 years (3–88 years), and males were more commonly affected than females. In the study of Shah et al., the median age of patients with primary extranodal NHL of oral cavity was 46.2 years with M: F ratio of 3:2.[6] In our case, the patient is a 23-year-old female.

van der Waal et al. have mentioned palate and gingiva as the most common site for oral cavity lymphomas.[2] In the study of Shah et al., gingivobuccal complex was the site in 12 out of the 15 patients.[6] Our patient presented with swelling arising from the base of tongue over the left side.

Chromosomal translocation plays an important role in the genomic malfunctioning leading to lymphoid malignancies.[7] Other risk factors such as viruses commonly associated with NHL include Epstein–Barr virus, human T-cell lymphotropic virus-1, human herpesvirus-8, human type C retrovirus, and HIV.[7],[8]

Lymphomas are staged using the Ann Arbor staging system [9] which has four stages. Most of the extranodal head and neck NHL fall into Stage IE if localized. An additional suffix A or B is added if systemic signs such as unexplained weight loss >10%, fever, or night sweats are absent or present.

IHC is almost compulsory for the correct diagnosis and characterization of immunophenotype in most of the lymphomas.[10] The panel of markers is decided based on morphologic differential diagnosis which includes LCA, B-cell markers CD20 and CD79a, and T-cell markers CD3 and CD5.

Staging of oropharyngeal lymphoma as well as other nodal lymphomas requires CT of the head and neck, thorax, abdomen, and pelvis. Concurrent positron emission tomography (PET) with 18F-fluorodeoxyglucose and PET/CT is a useful method for image-guided biopsy, staging, and assessment of therapeutic response.[11] Our patient CT showed a moderately enhancing mass arising in the left lateral pharyngeal wall extending from the base of tongue to just above the level of epiglottis.

According to the international prognostic index, patient of more than 60 years, advanced stage diseases such as Stage III/IV, elevated lactate dehydrogenase, poor performance status, and more than one extranodal site have a poor prognosis. However, our case was a young female with Stage IE, with good performance status and will fall into the better prognosis category.

In general, the standard treatment for patients with early stage oropharyngeal lymphoma is chemotherapy followed by involved field radiotherapy (IFRT).[12] Combined modality therapy should be considered for most patients with Stage I to IIE disease including three to six cycles of CHOP followed by IFRT.[13] Our patient was treated with four cycles of CHOP regimen chemotherapy.


  Conclusion Top


Even though squamous cell carcinomas of the oropharynx are more common, lymphomas should still be kept in consideration as a differential diagnosis in lesions arising from this place. This is important as the management and prognosis of lymphomas is different, and early detection can result in a complete cure and better long-term survival. We present a rare case of extranodal lymphoma of the base of tongue in a young immunocompetent female. We highlight the need for a careful clinical evaluation supported by histopathological and radiologic investigations which will help in identifying the disease at an early stage.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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. 1
    
2.
van der Waal RI, Huijgens PC, van der Valk P, van der Waal I. Characteristics of 40 primary extranodal non-Hodgkin lymphomas of the oral cavity in perspective of the new WHO classification and the International Prognostic Index. Int J Oral Maxillofac Surg 2005;34:391-5.  Back to cited text no. 2
    
3.
Chan JK. The new World Health Organization classification of lymphomas: The past, the present and the future. Hematol Oncol 2001;19:129-50.  Back to cited text no. 3
    
4.
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;13:371-92.  Back to cited text no. 4
    
5.
Kobler P, Borcic J, Filipovic ZI, Nola M, Sertic D. Primary non-Hodgkin's lymphoma of the oral cavity. Oral Oncol Extra 2005;41:12-4.  Back to cited text no. 5
    
6.
Shah GH, Panwar SK, Chaturvedi PP, Kane SN. Isolated primary extranodal lymphoma of the oral cavity: A series of 15 cases and review of literature from a tertiary care cancer centre in India. Indian J Med Paediatr Oncol 2011;32:76-81.  Back to cited text no. 6
[PUBMED]  Medknow Journal  
7.
Mawardi H, Cutler C, Treister N. Medical management update: Non-Hodgkin lymphoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;107:e19-33.  Back to cited text no. 7
    
8.
Hokett SD, Cuenin MF, Peacock ME, Thompson SH, Van Dyke TE. Non-Hodgkin's lymphoma and periodontitis. A case report. J Periodontol 2000;71:504-9.  Back to cited text no. 8
    
9.
Edge S, Byrd DR, Compton CC, Fritz AG, Greene FL, Trotti A, editors. American Joint Committee on Cancer: AJCC Cancer Staging Manual. 6th ed. New York: Springer; 2002.  Back to cited text no. 9
    
10.
Rao IS. Role of immunohistochemistry in lymphoma. Indian J Med Paediatr Oncol 2010;31:145-7.  Back to cited text no. 10
[PUBMED]  Medknow Journal  
11.
Elstrom RL, Leonard JP, Coleman M, Brown RK. Combined PET and low-dose, noncontrast CT scanning obviates the need for additional diagnostic contrast-enhanced CT scans in patients undergoing staging or restaging for lymphoma. Ann Oncol 2008;19:1770-3.  Back to cited text no. 11
    
12.
Maghfoor I, Akhtar S, Bakshi N. Current concepts in lymphoma biology: Effect on management and outcome. J Appl Hematol 2012;3:153-8.  Back to cited text no. 12
  Medknow Journal  
13.
Persky DO, Unger JM, Spier CM, Stea B, LeBlanc M, McCarty MJ, et al. Phase II study of rituximab plus three cycles of CHOP and involved-field radiotherapy for patients with limited-stage aggressive B-cell lymphoma: Southwest Oncology Group study 0014. J Clin Oncol 2008;26:2258-63.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2]


This article has been cited by
1 Extranodal lymphoma of the tongue, a very rare entity-report of two cases with literature review
J. Clarke,S. Medford,S. Islam,C. Ramsingh,M. Christopher
International Journal of Surgery Case Reports. 2019; 54: 70
[Pubmed] | [DOI]



 

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