|Year : 2020 | Volume
| Issue : 2 | Page : 274-277
Non-hodgkin's lymphoma at the base of tongue: A rare localization
Santosh Kumar Swain, Nibi Shajahan
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University, Bhubaneswar, Odisha, India
|Date of Submission||07-Jul-2020|
|Date of Decision||13-Jul-2020|
|Date of Acceptance||14-Jul-2020|
|Date of Web Publication||23-Dec-2020|
Dr. Santosh Kumar Swain
Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha “O” Anusandhan University, K8, Kalinga Nagar, Bhubaneswar - 751 003, Odisha
Source of Support: None, Conflict of Interest: None
The extra-nodal non-Hodgkin's lymphomas (NHLs) are mainly found in the head–and-neck region after the gastrointestinal tract. In the head–and-neck region, most common sites for NHL are Waldeyer's ring, followed by the sinonasal tract. NHL at the base of the tongue is extremely rare. The evaluation of the mass at the base of the tongue is a diagnostic challenge for a clinician as a wide spectrum of the benign and malignant lesions found in this site. The diagnosis of the NHL is established by histopathological and immunohistochemical studies. Here, we reported a case of the NHL at the base of the tongue in 28-year-old female. The patient was treated with three cycles of a combination of cyclophosphamide, vincristine, adriamycin, and prednisone chemotherapy and rituximab followed by radiotherapy. There was no evidence of recurrence during the first, second, and third years of the follow up period.
Keywords: Base of the tongue, extranodal lymphoma, non-Hodgkin's lymphoma
|How to cite this article:|
Swain SK, Shajahan N. Non-hodgkin's lymphoma at the base of tongue: A rare localization. Arch Med Health Sci 2020;8:274-7
| Introduction|| |
Lymphomas are malignant tumors of the lymphocyte cell lines involving the lymph nodes, spleen, and other nonhematopoietic tissues. The lymphomas are classified into Hodgkin's and non-Hodgkin's lymphoma (NHL). In the head-and-neck region, NHL represents 60% of all the types of the lymphoma and consists of B-cell, T-cell, and natural killer-T-cell lymphoma on the basis of the histological classification. The diffuse large B-cell lymphoma accounts for the highest frequency among NHL. The most common site for extranodal lymphomas is the gastrointestinal tract, followed by the head and neck region. Lymphoma is the third most common oral cavity malignancy after squamous cell carcinoma and salivary gland malignancy and lymphoma constitute only 3%–5% of all the oral cavity malignancy. Primary oral cavity lymphoma constitutes only 1% of all the lymphoma of the body and approximately 2%–12% of the extranodal lymphomas and the malignant lymphomas of the tongue are even rarer. Isolated oral cavity and oropharyngeal lymphomas may arise from Waldeyer's ring because of its rich lymphoid tissue. The common locations for oral NHL are hard palate, vestibule, and gingival region. Primary NHL at the base of the tongue is extremely rare. NHL ranges from the relatively indolent to highly aggressive variety and even cause potentially fatal. Here, we report a case of NHL at the base of the tongue in a young female.
| Case Report|| |
A 28-year-old female attended the outpatient department of otorhinolaryngology with complaints of foreign body sensation in the throat for the past 3 months. She had no addiction to smoking, chewing tobacco, and consumes alcohol. The family history was nothing suggestive. There was no history of any systemic medical illness or any allergies. The local examination of the oral cavity and oropharynx showed a painless or nontender globular mass of approximately 2.5 cm × 3 cm situated at the right side of the base of the tongue [Figure 1]. Bilateral palatine tonsils were normal. The neck examination did not reveal any lymphadenopathy and the rest of the otorhinolaryngological examinations were within the normal limits. Complete blood cell counts were within the normal limits. Serum biochemistry was within normal ranges. human immunodeficiency virus (HIV), hepatitis B surface antigen, hepatitis C virus, and Epstein–Barr virus (EBV) were negative. Chest X-ray, ultrasound of abdomen and pelvis, echocardiography, ultrasonography of the abdomen, and pelvis, 99mTc thyroid scan showed no significant abnormalities. Computed tomography (CT) scan of the neck showed a mass at the right side of the base of the tongue with no cervical lymphadenopathy [Figure 2]. The mass was the polypoidal appearance and homogenously moderately enhancing. Magnetic resonance imaging of the tongue showed a mass at the right side of the base of the tongue. The patient underwent an excisional biopsy of the mass from the base of the tongue through the endoscopic transoral approach under general anesthesia with nasotracheal intubation. The mass of the base of the tongue was resected with electrocautery. All the margins of the resection were free of the disease. The mass was sent for histopathological examination, which showed lining of stratified squamous epithelium below which diffuse sheets of neoplastic cells having round hyperchromatic nuclei, inconspicuous nucleoli, and scanty cytoplasm along with several large cells with vesicular nuclei infiltrating the skeletal muscles [Figure 3]. A diagnosis of the NHL was made. Immunohistochemical evaluation revealed positive immunoreactivity for CD20 [Figure 4]. The patient was thoroughly evaluated for other sites involvement. However, no other location of the body was found to be affected. The bone marrow study showed no lymphomatous infiltration. The final diagnosis of NHL with a diffuse large B cell type of the base of the tongue was made. The patient was staged as Ann Arbor Stage IE. She was given three cycles of a combination of cyclophosphamide, vincristine, adriamycin, and prednisone (CHOP) chemotherapy and rituximab followed by radiotherapy. The patient received 50 Gy in 25 fractions over a period of the 6 weeks by 6 Mv photon beam on the linear accelerator. She had no evidence of the recurrence at the follow-up during the 1st year, 2nd year, and 3rd year.
|Figure 1: (a) A globular non-Hodgkin's lymphoma mass at the right side of the base of the tongue; (b) excised non-Hodgkin's lymphoma mass from the base of the tongue|
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|Figure 2: Computed tomography scan of the neck showing non-Hodgkin's lymphoma mass at the right side of the base of the tongue|
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|Figure 3: Histopathological picture showing neoplastic cells arranged uniformly with large and round nuclei with little cytoplasm and dispersed coarse to fine nuclear chromatic and inconspicuous nucleoli (H and E, ×400)|
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|Figure 4: Immunohistochemical staining of tumor cells showing positive for CD20 (×400)|
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| Discussion|| |
Lymphomas include a group of solid tumors which originate from the proliferation of the B-cell and T lymphocytes. The most common sites for these lymphomas are not only the lymph nodes but also reported from extranodal sites. Approximately 30% of the lymphomas are extranodal, majority of them being NHL. Lymphoma with small B-cells has aggressive behavior with an increased tendency for dissemination. The tongue is an extremely rare location for the lymphoma. The etiology of the lymphoma is still not well known. However, the genetic mutations and infections with EBV, human herpesvirus 8, and the HIV are important risk factors for causing lymphoma. The exact etiology of the NHL at the site of the base of the tongue is not known. Few cases of the oral cavity lymphoma such as plasmablastic lymphomas have been associated with acquired immune deficiency syndrome. However, our case was HIV negative. The incidence of the NHL with diffuse large B-cell lymphoma type is more common in Asian patients than Western patients. Males are affected more than females and occur in the age group of sixth to eight decades of life. However, patients of immunodeficiency disorders are often seen in younger age. In our case, the patient was a female and young adult without any immunodeficiency diseases. Approximately, 5%–10% of the cases of primary or secondary NHLs found in Waldeyer's ring which include adenoids, palatine tonsils, lymphoid tissue at the soft palate, the base of the tongue, and less commonly in the oral cavity. The clinical presentations of the lymphoma at the base of the tongue are not defined because of the very low incidence of such lesions.
The clinical presentations of the primary lymphoma at the base of the tongue are often not defined because of the delayed and nonspecific features. Our patient presented with foreign body sensation and mild dysphagia. The involvement of the tongue muscles by the lymphoma can cause restriction of movement of the tongue. However, this patient was not showing any evidence of the restricted tongue movement. The diagnosis of the lymphoma at the base of the tongue requires thorough endoscopic examinations of the oral cavity, oropharynx, and hypopharynx. In majority of the cases, the tumor has a smooth surface, but some reported ulcerations of the superficial mucosa which often confuse with carcinoma. The CT scan is usually done to assess the cranial nerves, neck, thorax, abdomen, and pelvis for establishing the full classification of the lymphoma. PET-CT scan is preferred for assessing the response of the lymphoma to the treatment. Limited documented cases of the lymphoma at the base of tongue hinder an understanding of the biological behavior and treatment options.
There is no absolute protocol for the treatment of the primary NHL at the base of the tongue. It has been suggested that the treatment options for oral cavity lymphomas include excisional biopsy followed by radiotherapy without systemic chemotherapy in case of the localized or solitary lesions; however, the recommendations of such treatments have not been validated because of the limited number of data. Few patients are treated by chemotherapy with or without radiotherapy. In our case, the patient was treated with chemotherapy with CHOP with rituximab followed by radiotherapy following excision of the mass from the base of the tongue. The addition of rituximab (monoclonal antibody to CD20) with CHOP has been seen to improve the complete response and increase the symptom-free and overall survival of the patient in case of large B-cell lymphoma without much toxicity. For getting accurate therapeutic options for the primary NHL at the base of the tongue, more randomized prospective studies are required and involvement of the large numbers of cases with multi-institutional trials. Proper evaluation of the mass at the base of the tongue often make diagnostic challenges because of the variety of benign and malignant lesions may be seen. However, the localization, histopathological examination, and immunohistochemical studies of the tongue mass are helpful for easy diagnosis and effective treatment.
| Conclusion|| |
The malignant lymphoma at the base of the tongue is very rare. Clinician should keep it in mind for the tumoral mass at the base of tongue localization for NHL. The diagnosis is usually established by the histopathological examination and immunohistochemistry. Although complete excision of the tumor mass is not included in the standard treatment protocol, it was done in this case for improved quality of life. Although the base of the tongue NHL has nonspecific clinical presentations, it is very important to have a high index of suspiciousness to rule out NHL in the base of the tongue as this disease is a curable lesion.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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.
Swain SK, Sahu MC. An unusual presentation of nasofacial NK/T-cell lymphoma-A case report. Egypt J Ear Nose Throat Allied Sci 2017;18:299-302.
Zapater E, Bagán JV, Carbonell F, Basterra J. Malignant lymphoma of the head and neck. Oral Dis 2010;16:119-28.
Terada T. Primary non-Hodgkin B-cell lymphoma of the tongue. Br J Oral Maxillofac Surg 2011;49:e18-9.
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.
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.
Jayakrishnan R, Thomas G, Kumar A, Nair R. Non-Hodgkin's lymphoma of the hard palate. J Oral Maxillofac Pathol 2008;12:85-7. [Full text]
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.
Lim JH, Lee MH, Lee MJ, Kim CS, Lee JS, Choi SJ, et al
. Plasmablastic lymphoma in the anal canal. Cancer Res Treat 2009;41:182-5.
Thompson LD. Diffuse large B-cell lymphoma of the nasopharynx. Ear Nose Throat J 2012;91:192, 194.
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.
Guastafierro S, Falcone U, Celentano M, Cappabianca S, Giudice A, Colella G. Primary mantle-cell non-Hodgkin's lymphoma of the tongue. Int J Hematol 2008;88:206-8.
Mohammadianpanah M, Ahmadloo N, Mozaffari MA, Mosleh-Shirazi MA, Omidvari S, Mosalaei A. Primary localized stages I and II non-Hodgkin's lymphoma of the nasopharynx: A retrospective 17-year single institutional experience. Ann Hematol 2009;88:441-7.
Coiffier B, Lepage E, Briere J, Herbrecht R, Tilly H, Bouabdallah R, et al
. CHOP chemotherapy plus rituximab compared with CHOP alone in elderly patients with diffuse large-B-cell lymphoma. N Engl J Med 2002;346:235-42.
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