For citation purposes: Vasilakaki T, Myoteri D, Tsavari A, Skafida E, Arkoumani E, Koulia K, Grammatoglou X, Manoloudaki K. Localised extranodal non-Hodgkin's lymphoma of the tonsil: report of a rare case. OA Case Reports 2013 Sep 10;2(11):101.

Case Report

 
Oncology

Localised extranodal non-Hodgkin's lymphoma of the tonsil: report of a rare case

T Vasilakaki, D Myoteri, A Tsavari, E Skafida*, E Arkoumani, K Koulia, X Grammatoglou, K Manoloudaki
 

Authors affiliations

Department of Pathology, Tzaneion General Hospital, Piraeus, Greece

*Corresponding author Email: evelinaskafida@yahoo.gr

Abstract

Introduction

Non-Hodgkin's lymphoma of the Waldeyer's ring is a relatively rare entity and the palatine tonsil is the most frequently involved site. Although, the exact aetiology remains unclear, a number of predisposing factors have been identified, including human immunodeficiency virus and Epstein-Barr infection. We report a case of localised extranodal non-Hodgkin's lymphoma of the tonsil.

Case report

A 64-year-old woman presented with a sore throat. On physical examination, an approximately 2 × 1 cm smooth non-tender mass was observed in the left palatine tonsil. Serology was negative for human immunodeficiency virus and Epstein-Barr virus. Computer tomography scan revealed a non-enhancing left tonsillar mass but no signs of neck lymphadenopathy. The patient underwent bilateral tonsillectomy. Histological examination confirmed a diagnosis of non-Hodgkin's lymphoma diffuse large cell type of B phenotype. Immunohistochemically, the neoplastic cells were positive for CD19, CD20, CD10, CD79a, CD22, Bcl-2, Bcl-6 and negative for CD57, CD56, CD2, EBV, CD4, CD5, CD7, CD8, S-100p, CD3, Cyclin D1, CD1a, HMB45, CKAE1 and CKAE3. Bone marrow biopsy did not reveal lymphomatous involvement (stage I according to tumour, node, metastasis classification). The patient received chemotherapy based on CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) protocol combined to Rituximab. During follow-up, she remains disease-free 30 months after diagnosis.

Conclusion

Non-Hodgkin's lymphoma rarely involves tonsils with the diffuse large B-cell type being common at this location. A combined treatment consisting of chemotherapy and radiotherapy leads to a satisfactory outcome in patients with this uncommon neoplasm, which tends to present at an early stage and to have a favourable prognosis.

Introduction

Non-Hodgkin's lymphoma (NHL) of the oral cavity and oropharynx account for 13% of all primary extranodal NHL with approximately 70% of these occurring in the tonsils. The palatine tonsil is the most frequently involved site followed by palate, gingiva and tongue1,2-3. Most lymphomas found in the palatine tonsils are the B-cell type, and of these, diffuse large B-cell lymphoma (DLBCL) represents most of the cases, reaching as much as 80% in some of the groups studied1-53.

Although, the exact aetiology remains unclear, a number of predisposing factors have been identified, including human immunodeficiency virus and Epstein-Barr infection2. This study reports a rare case of localised extranodal NHL of the tonsil.

Case report

We report a case of a 64-year-old woman who presented with a sore throat during the last four months. On physical examination, an approximately 2 × 1 cm smooth non-tender mass was observed in the left palatine tonsil. The remainder of the physical examination was normal. Laboratory studies for tumour markers and serology tests for human immunodeficiency virus and Epstein-Barr virus were negative as well. Computer tomography scan revealed a non-enhancing left tonsillar mass but no signs of neck lymphadenopathy. Past history did not appear to be contributory regarding the aetiology. The patient underwent bilateral tonsillectomy. Histological examination confirmed a diagnosis of NHL diffuse large cell type of B phenotype. The stroma was densely infiltrated by medium- to large-sized lymphoma cells and the surface stratified squamous epithelium was ulcerated (Figures 1 and 2). Immunohistochemically, the neoplastic cells were positive for CD19, CD20, CD10, CD79a, CD22, Bcl-2, Bcl-6 and negative for CD57, CD50, CD2, EBV, CD4, CD5, CD7, CD8, S-100p, CD3, Cyclin D1, CD1a, HMB45, CKAE1 and CKAE3 (Figure 3). Bone marrow biopsy did not reveal lymphomatous involvement (stage I according to tumour, node, metastasis classification). The patient received chemotherapy with a CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisolone) combined to Rituximab. During follow-up, she remains disease-free 30 months after diagnosis.

Primary large B-cell lymphoma of the tonsil (H-E × 40).

Primary large B-cell lymphoma of the tonsil (H-E × 200).

Primary large B-cell lymphoma of the tonsil (CD79a × 200).

Discussion

NHL represents a small percentage of oral malignancies and palatine tonsil is the most frequently involved site. This lymphoma has a peak incidence in the 6th and 7th decades of life in published series‘ and the sex incidence is slightly male predominant. Clinical signs and symptoms are not specific and occur as a result of asymmetrical tonsillar enlargement. They may include a sensation of fullness in the throat, sore throat, dysphagia, odynophagia, otalgia, cervical adenopathy, tonsillar swelling or snoring. Systemic symptoms, such as fever, weight loss and night sweats are uncommon and may develop in patients with advanced disease[1].

Most tonsillar lymphomas reported in the literature are of B-cell origin and the most common histologic type, ranging from 67% to 96%, has been reported to be DLBCL. In many series‘, it is reported that the majority of these patients have localised disease (stage I or II)[1,2,3,4].

Patients with lesions that were clinically determined to be over 7 cm in size (bulky) had a significantly poorer outcome as compared with those with smaller tumours. Authors have reported 5-year survival rates of 65%-85% for patients with early stage disease and no present bulky mass[1,5,6,7,8].

Treatment includes chemotherapy alone, radiotherapy alone or a combination of both. The majority of patients received chemotherapy followed by radiotherapy[1,5,8,9,10].

Conclusion

NHL rarely involves tonsils with the diffuse large B-cell type being common at this location. A combined treatment consisting of chemotherapy and radiotherapy leads to a satisfactory outcome in patients with this uncommon neoplasm, which tends to present at an early stage and to have a favourable prognosis.

Consent

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

Authors Contribution

All authors contributed to the conception, design, and preparation of the manuscript, as well as read and approved the final manuscript.

Competing interests

None declared.

Conflict of interests

None declared.

A.M.E

All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.

References

  • 1. Laskar S, Bahl G, Muckaden MA, Nair R, Gupta S, Bakshi A. Primary diffuse large B-cell lymphoma of the tonsil: is a higher radiotherapy dose required?. Cancer 2007 Aug;110(4):816-23.
  • 2. Jacobs C, Weiss L, Hoppe RT. The management of extranodal head and neck lymphomas. Arch Otolaryngol Head Neck Surg 1986 Jun;112(6):654-8.
  • 3. Yamanaka N, Harabuchi Y, Sambe S, Shido F, Matsuda F, Kataura A. Non Hodgkin's lymphoma of Waldeyer's ring and nasal cavity. Clinical and immunological aspects. Cancer 1985 Aug;56(4):768-76.
  • 4. Hart S, Horsman JM, Radstone CR, Hancock H, Goepel JR, Hancock BW. Localized extranodal lymphoma of the head and neck: the Sheffied Lymphoma Group experience (1971-2000). Clin Oncol 2004 May;16(3):186-92.
  • 5. Mohammadianpanah M, Daneshbod Y, Ramzi M, Hamidizadeh N, Dehghani SJ, Bidouei F. Primary tonsillar lymphomas according to the new World Health Organization classification: to report 87 cases and literature review and analysis. Ann Hematol 2010 Oct;89(10):993-1001.
  • 6. Qin Y, Shi YK, He XH, Yang JL, Yang S, Yu YX. Clinical features of 89 patients with primary non Hodgkin's lymphoma of the tonsil. Ai Zheng 2006 Apr;25(4):481-5.
  • 7. Gao Y, Li Y, Yuan Z, Zhao L, Liu X, Gu D. Prognostic factors in patients with primary non Hodgkin's lymphoma of the tonsil. Zhonghua Zhong Liu Za Zhi 2002 Sep;24(5):483-5.
  • 8. Endo S, Kida A, Sawada U, Sugitani M, Furusaka T, Yamada Y. Clinical analysis of malignant lymphomas of tonsil. Acta Otolaryngol Suppl 1996;523263-6.
  • 9. Lugassy G, Hurwitz N, Shtalrid M, Varon D, Marshak G, Berrebi A. Clinical and pathological features of non-Hodgkin's lymphoma of the tonsil. Review of the literature and report of 10 cases. Isr J Med Sci 1989 May;25(5):251-5.
  • 10. Avilés A, Delgado S, Ruiz H, de la Torre A, Guzman R, Talavera A. Treatment of non-Hodgkin's lymphoma of Waldeyer's ring: radiotherapy versus chemotherapy versus combined therapy. Eur J Cancer B Oral Oncol 1996 Jan;32B(1):19-23.
Licensee to OAPL (UK) 2013. Creative Commons Attribution License (CC-BY)