Portuguese Version

Year:  2004  Vol. 70   Ed. 2 - (20º)

Relato de Caso

Pages: 273 to 276

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Involvement of the palatine tonsil by non-Hodgkin's lymphoma: report of 3 cases

Author(s): Patrícia Ciminelli Linhares Pinto 1,
Cecília Pache de Faria 2,
Geraldo Augusto Gomes 3,
Augusto Paulo Pinto 4

Keywords: lymphoma, extranodal, palatine tonsil.

Abstract:
The objective of our study is to comment on three cases of non-Hodgkin's lymphoma (NHL) of the palatine tonsil treated at Rio de Janeiro Federal University's Hospital. The NHL occur in extranodal sites in 20-30% of the cases, of these, 10-30% are seen in the head and neck. Sixty to seventy percent of the NHL of the head and neck are present in the Waldeyer's ring, and we see aproximately 80% of the Waldeyer's ring NHL in the tonsil. We conclude that NHL of the tonsil is a rather uncommon condition that should be promptly diagnosed for a correct treatment and follow-up.

INTRODUCTION

Lymphomas are a set of varied lymphoproliferative diseases traditionally divided into Hodgkin (HL) and non-Hodgkin disease or lymphoma (NHL). HL is rarely manifested as extranodal disease (1% of the cases). In turn, NHL can affect extranodal sites in 25-30% of the cases, being that the most frequent region is gastrointestinal tract. The head and neck region is involved in only 10-30% of the cases with extra-nodal affection. Waldeyer's ring is involved in 60-70% of the cases of head and neck NHL 1-7. As to extranodal sites, Waldeyer's ring is affected in only 10-15% of the cases. Out of the NHL that affects Waldeyer's ring, 80% are located in the palatine tonsil.8

The purpose of the present study was to describe three cases of NHL that affected palatine tonsil and to discuss the topic.

CASE REPORT

Case 1

A.P.S., female Caucasian subject, born on 17/03/1900. She was seen in February 1980 with complaint of tumor on the right neck region for 1 year, and onset of tumor on the left few months later. She had experienced odynophagia and dysphagia for 3 months. No reference to fever, weight loss, night sweating or other associated symptoms.

Physical examination: head and neck - presence of lymph nodes larger than 5cm on the right jugular-carotid region, inferior and superior. Lymph nodes larger than 4cm in the medial portion of the left jugular-carotid region, hardened and fixed.

Anterior rhinoscopy: no significant findings, no visible lesions.
Oropharyngoscopy: voluminous tumor mass on the right palatine tonsil, extending approximately 1cm upwards, towards the cavum, and inferiorly, to the epiglottis level, through the right lateral wall of the pharynx. Paralyzed palate on the right side.

We conducted biopsy of palatine tonsil lesion and diagnosed well-differentiated diffuse lymphocytarian lymphoma in the palatine tonsil.

No other references to sites with affected lymph nodes. Started radiotherapy with cobalt. After 8 months the patient presented absence of neck lymph nodes and regression of right palatine tonsil lesion. In May 1981, the patient was hospitalized complaining of dyspnea. Chest x-ray showed upper mediastinum widening by marginal opacity to the right paratracheal region, with left lateral deviation of the trachea; presence of pleural effusion. Increase of soft parts of neck and supraclavicular regions on the right.

The patient was submitted to polychemotherapy with cyclophosphamide, vincristine and prednisone, after confirming pleural lymphomatous infiltration.

The patient was hospitalized a number of other times with dyspnea; submitted to congestive heart failure treatment with no positive result. Patient died in July 1981.

Case 2

L.V.S., female Caucasian subject, born on 12/12/1957 (44 years of age). The patient was seen for the first time in November 2001, reporting that she had had a neck mass on the right for two months, associated with dysphagia, local pain and nonspecific weight loss. No other associated complaints. Smoked 2 packs/cigarettes a day for 20 years.

Physical examination: head and neck - bilateral neck lymphadenomegalia, approximately 8 cm on the right and 3 cm on the left, hardened, mobile and a bit painful upon palpation. Oropharyngoscopy: palatine tonsil hypertrophy, more marked on the right, with presence of ulcerated lesion. No other significant findings were identified. Abdominal ultrasound and chest x-ray without changes. Computed tomography of the neck region showed bilateral multiple cervical lymphadenomegalia, with neoplastic aspect. Biopsy of the right palatine tonsil and neck lymph node evidenced diffuse NHL of large B-cells. Serology for HIV and hepatitis B and C and bone marrow biopsy were normal. Submitted to chemotherapy with cyclophosphamide, adriamycin, oncovin and prednisone (CHOP), being that the last cycle was administered in April 2002. Still followed up, presenting complete emission of lymphoma.

Case 3

M.R.S., male Caucasian subject, born on 19/06/1973. Patient was seen for the first time in December 2001, referred to assessment of right palatine tonsil lesion. History of odynophagia and vocal affections (nasal voice) for 23 months. No dysphonia, fever, weight loss or other associated symptoms.

Physical examination: presented right palatine tonsil hypertrophy, with presence of ulcerated lesion. Biopsy revealed diagnosis of diffuse lymphoma of large B-cells.

Chest CT scan showed mediastinal lymphadenomegalia.

Abdominal CT scan and bone marrow biopsy were normal.

HIV and hepatitis B and C serology were not reactive.

Currently, the patient is under treatment with chemotherapy regimen CHOP.

DISCUSSION AND COMMENTS

In the present study, we report three cases of NHL affecting palatine tonsil. In one case, lymphoma presented as primary palatine tonsil tumor, without involvement of other organs or ganglionar chains. In the two other cases, we found affection of other lymph nodes, especially in the neck, even though diagnosis of the systemic disease has been possible thanks to local symptomatology of oropharynx. Hanna et al. described affection of associated lymph nodes in 20% of the cases 1. In their study about NHL of the tonsil, Alvie et al. described neck adenopathy as the most common initial signs in patients with NHL and only 10% of these patients have extranodal head and neck disease 9.

In the cases described, we found that odynophagia and/or dysphagia were important symptoms that motivated the medical visit, showing relevance as local symptoms, even in cases with neck adenomegalia. Similarly, we can see that in the literature most of the patients with palatine tonsil lymphoma present dysphagia and odynophagia, in addition to some cases of dyspnea, owing to tumor size 9-11. Hanna et al.1 described tonsil lymphoma presenting unilateral tonsil increase, ulceration, dysphagia or odynophagia, as detected in our cases.

Most of the patients with NHL and extra-nodal affection, such as the case of palatine tonsil, do not present exuberant systemic symptoms or associated B symptoms (fever, weight loss, and night sweating), as observed in our cases and in literature reports 1, 11. Hanna et al. described only 20% of patients with extra-nodal NHL of the head and neck as having B symptoms or systemic symptoms 1. Ezzat et al. found B symptoms in only 15% of their patients, and most of them presented symptoms related to primary site involvement 11. Only in two described patients we presented nonspecific weight loss, without other systemic or B symptoms described in the other cases.

In case 2, we presented a patient with history of smoking. Alcohol abuse and smoking are relevant in initial diagnosis of tumors that affect palatine tonsils, since there is high incidence of squamous cell carcinoma in these subjects 10. Pain and ulceration in the tonsils are more common in patients with carcinoma than with lymphoma, but diagnosis can only be confirmed with careful histopathology examination 1. Upon reviewing the literature, it was clear that the main malignancy affecting the palatine tonsil in adults is squamous cell carcinoma. In children, tonsilar malignancy is very rare, but in its presence, lymphomas are the most likely diagnosis 12-14.

Treatment of NHL can be conducted with isolated therapy in cases in which there is localized disease (stage I)8 but standard treatment in other cases is polychemotherapy, associated or not with radiotherapy 8, 11. Surgery is normally only used to define the diagnosis 1. It is extremely important to carefully follow up the patient together with the hematologist and oncologist.

Ezzat et al., in addition to other authors, described in their series predominance of diffuse histology type of large B-cells in NHL affecting palatine tonsil, similarly to what was observed in our cases 9, 11, 15.

Waldeyer's ring lymphoma represent 60-70% of all extranodal lymphomas of the head and neck, and 10-15% of all extranodal lymphomas, second only to gastrointestinal lymphomas. In the Waldeyer's ring, palatine tonsil is the most affected site by NHL, reaching 80% in some series 8-10, 13. Despite these statistics, it is still an uncommon disease, representing a small percentage of head and neck and palatine tonsil malignant neoplasms 9. We should be careful to make the correct diagnosis and provide treatment and follow-up with the hematologist/oncologist.

REFERENCES

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1 Resident Physician in Otorhinolaryngology, Hospital Universitário Clementino Fraga Filho (HUCFF), Federal University of Rio de Janeiro (UFRJ).
2 Resident Physician in Otorhinolaryngology, HUCFF, UFRJ.
3 Resident Physician in Otorhinolaryngology, HUCFF, UFRJ.
4 Ph.D., Professor and Head of the Service Otorhinolaryngology, HUCFF, UFRJ.
Affiliation: Service of Otorhinolaryngology, HUCFF, UFRJ.
Address correspondence to: Patrícia Ciminelli Linhares Pinto - Rua Dias da Rocha, 35/ 1002 Copacabana Rio de Janeiro RJ 22051-020
Tel (55 21) 2236-5190/ 9963-8747 - E-mail: patricia@linhares.com.br
Study presented at 36o Congresso Brasileiro de Otorrinolaringologia, Florianopolis, November 19 - 23, 2002.

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