Case Report

Page 1 of 2

Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY)

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

mpe

ting

inte

rest

s: n

one

decl

ared

. Con

flict

of i

nter

ests

: non

e de

clar

ed.

All a

utho

rs c

ontr

ibut

ed to

the

conc

eptio

n, d

esig

n, a

nd p

repa

ratio

n of

the

man

uscr

ipt,

as w

ell a

s rea

d an

d ap

prov

ed th

e fin

al m

anus

crip

t.Al

l aut

hors

abi

de b

y th

e As

soci

ation

for M

edic

al E

thic

s (AM

E) e

thic

al ru

les o

f disc

losu

re.

*Corresponding authorEmail: evelinaskafida@yahoo.gr

Department of Pathology, Tzaneion General Hospital, Piraeus, Greece

AbstractIntroductionNon-Hodgkin’s lymphoma of the Waldeyer’s ring is a relatively rare entity and the palatine ton-sil is the most frequently involved site. Although, the exact aetiology remains unclear, a number of predis-posing factors have been identified, including human immunodeficiency virus and Epstein–Barr infection. We report a case of localised extra-nodal non-Hodgkin’s lymphoma of the tonsil.Case reportA 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 tonsil-lectomy. Histological examination con-firmed 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, doxoru-bicin, vincristine and prednisolone) protocol combined to Rituximab. During follow-up, she remains disease- free 30 months after diagnosis. ConclusionNon-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 uncom-mon neoplasm, which tends to pre-sent at an early stage and to have a favourable prognosis.

IntroductionNon-Hodgkin’s lymphoma (NHL) of the oral cavity and oropharynx account for 13% of all primary extran-odal NHL with approximately 70% of these occurring in the tonsils. The palatine tonsil is the most frequently involved site followed by palate, gin-giva and tongue1,2. 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 studied1–3.

Although, the exact aetiology remains unclear, a number of predis-posing factors have been identified, including human immunodeficiency virus and Epstein–Barr infection. This study reports a rare case of local-ised extranodal NHL of the tonsil.

Case reportWe 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 remain-der of the physical examination was normal. Laboratory studies for tumour markers and serology tests for human immunodeficiency virus and Epstein–Barr virus were nega-tive 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 epi-thelium 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 involve-ment (stage I according to tumour,

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 ManoloudakiOn

colo

gy

Figure 1: Primary large B-cell lym-phoma of the tonsil (H–E × 40).

Case Report

Page 2 of 2

Licensee OA Publishing London 2013. Creative Commons Attribution License (CC-BY)

Com

petin

g in

tere

sts:

non

e de

clar

ed. C

onfli

ct o

f int

eres

ts: n

one

decl

ared

.Al

l aut

hors

con

trib

uted

to th

e co

ncep

tion,

des

ign,

and

pre

para

tion

of th

e m

anus

crip

t, as

wel

l as r

ead

and

appr

oved

the

final

man

uscr

ipt.

All a

utho

rs a

bide

by

the

Asso

ciati

on fo

r Med

ical

Eth

ics (

AME)

eth

ical

rule

s of d

isclo

sure

.

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

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, et al. 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, et al. Primary tonsillar lympho-mas 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, et al. 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, et al. 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, et al. Clinical analysis of malignant lymphomas of tonsil. Acta Otolaryngol Suppl. 1996;523:263–6.9. Lugassy G, Hurwitz N, Shtalrid M, Varon D, Marshak G, Berrebi. A Clini-cal 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. Treat-ment 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.

Figure 2: Primary large B-cell lym-phoma of the tonsil (H–E × 200).

Figure 3: Primary large B-cell lym-phoma of the tonsil (CD79a × 200).

node, metastasis classification). The patient received chemotherapy with a CHOP regimen (cyclophosphamide, doxorubicin, vincristine and pred-nisolone) combined to Rituximab. During follow-up, she remains dis-ease-free 30 months after diagnosis.

DiscussionNHL represents a small percentage of oral malignancies and palatine tonsil is the most frequently involved site. This lymphoma has a peak inci-dence in the 6th and 7th decades of life in published series’ and the sex incidence is slightly male predomi-nant. Clinical signs and symptoms are not specific and occur as a result of asymmetrical tonsillar enlarge-ment. They may include a sensation of fullness in the throat, sore throat, dysphagia, odynophagia, otalgia, cer-vical adenopathy, tonsillar swelling

or snoring. Systemic symptoms, such as fever, weight loss and night sweats are uncommon and may develop in patients with advanced disease1.

Most tonsillar lymphomas repor-ted in the literature are of B-cell origin and the most common histo-logic 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 local-ised disease (stage I or II)1–4.

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 mass1,5–8.

Treatment includes chemotherapy alone, radiotherapy alone or a com-bination of both. The majority of patients received chemotherapy fol-lowed by radiotherapy1,5,8–10.

ConclusionNHL rarely involves tonsils with the diffuse large B-cell type being com-mon at this location. A combined treatment consisting of chemother-apy and radiotherapy leads to a satis-factory outcome in patients with this uncommon neoplasm, which tends to present at an early stage and to have a favourable prognosis.

ConsentWritten informed consent was obtained from the patient for publi-cation of this case report and accom-panying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

References1. Laskar S, Bahl G, Muckaden MA, Nair R, Gupta S, Bakshi A, et al. Primary diffuse large B-cell lymphoma of the tonsil: is