International Journal of Pediatric Otorhinolaryngology, 4 (1982) 349-35 1 Elsevier Biomedical Press

349

Burkitt’s lymphoma in the tonsil

Peter Poulsen

Department of Otorhinolaryngology, Veje (Denmark)

(Received April 26th, 1982)

(Accepted May 13th, 1982)

Summary

A case of Burkitt’s lymphoma is reported in which both its location in Waldeyr’s ring and the age of the patient, 2 years, are rare. The disease is found in an endemic

and a sporadic form. The endemic form especially is thought to be related to Epstein

Barr virus infection. The treatment is chemotherapy combined with cytostatica,

always containing cyclophosphamid; it is one of the few human tumours which can

be completely cured with cytostatica.

Introduction

Burkitt’s lymphoma (BL) is known in a relatively frequent endemic form from equatorial Africa and New Guinea, and also in a rare sporadic form from the rest of

the world [3]. BL was first described by Burkitt in 1959 [5] and is mainly a children’s tumour

with an average first appearance for the endemic form of 7 years against 11 years for

the sporadic [3]. BL is rare before the age of 2 and the location is most frequently

abdominal, retroperiotoneal, in facial bones, thyroid glands, salivary glands, bone marrow and central nervous system (CNS), while it is seldom seen in the lymphatic system, especially Waldeyr’s ring.

Case Report

A 2-year-old boy was admitted after a 24-hour-period of slight difficulties in swallowing. Objectively, a right-sided 2 X 2 X 3 cm large tumour was found, corre-

sponding to the tonsil. The patient had tonsillectomy and histologic and cytologic examinations showed Burkitt’s lymphoma, among other things with a typical, but not pathognomonic, ‘starry-sky’ pattern [ 1,4] (Fig. 1). He was then transferred to the

0165-5876/82/0000-00/,$02.75 0 1982 Elsevier Biomedical Press

Fig. 1. Shows the typical, but not pathognomonic, ‘starry-sky’ pattern.

regional oncologic centre where radiation therapy was applied to the tumour area. It

was further decided to give radiation to the CNS prophylactically because of the

rapid tumour development, the unusual location, the patient’s age and because the

sporadic form of Burkitt’s lymphoma has a high frequency of CNS involvement [3].

Furthermore, the patient was given chemotherapy in the form of cyclophosphamid, vincristine and prednisone, as well as methotrexate intraspinally. There were no

signs of metastases and normal conditions were found by sternal and lumbar punctures. After 6 months, the patient was still in complete remission.

Discussion

Together with low differentiated nasopharyngeal carcinoma, BL occupies a spe- cial place among human malignant tumours, as both are probably due to Epstein Barr virus (EBV), which is supposed to work oncogenically and induce specific chromosomal abnormalities [7]. The endemic form has EBV genome in the cells in almost 100% of the cases, whereas the sporadic form only has it in 8-17% [7]. Owing to the great interest in this type of tumour, the WHO has defined the histologic and cytopathologic criteria in a memorandum [4].

BL is a very lively proliferating tumour and se-LDH is an important indicator for spreading and effect of treatment. The treatment is chemotherapy in various combinations, but always containing cyclophosphamid. Further, intrathecal

351

methotrexate is recommended [2,6], and perhaps radiation therapy against CNS and

tumour [8].

Owing to the massive tumour regression from especially big abdominal tumours,

great metabolic disturbances may occur at the start of the treatment, sometimes with a lethal result [2,6]. BL is one of the few human tumours which can be completely

cured by chemotherapy [6], and relapse is seldom seen after one year of complete

remission. The 5-years’ survival can be stated at approximately 408, best for the

youngest patients [2,8].

Acknowledgements

Jan Rasmussen, M.D., Department of Pathology, Hospital of Vejle, has kindly lent me a specimen for the microscope, for which I thank him.

References

1 Anderson, W.A.D. and Scotti, T.M., Pathology, C.V. Mosby Company, St. Louis, 1972, p. 644.

2 Arseneau, J.C., Canelloz, G.P., Banks, P.M., Berard, C.W., Gralnick, H.R. and DeVita, V.T., American

Burkitt’s lymphoma: a clinicopathologic study of 30 cases, Amer. J. Med., 58 (1975) 314-321.

3 Banks, P.M., Arseneau, J.C., Gralmck, H.R., Canelloz, G.P., DeVita, V.T. and Costan, W.B., American

Burkitt’s lymphoma: a clinicopathologic study of 30 cases, Amer. J. Med., 58 (1975) 322-329.

4 Berard, C., O’Conor, G.T., Thomas, L.D. and Torloni, H., Bull. WHO, 40 (1969) 60 l-607.

5 Burkitt, D., A Sarcoma involving the jaws in African children, Brit. J. Surg., 46 (1958) 218-223.

6 Nkrumah, F.K., Perkins, I.V. and Biggar, R.J., Combination chemotherapy in abdominal Burkitt’s

lymphoma, Cancer, 40 (1977) 1410-1416.

7 Philip, P., Jensen, M.K. and Pallesen, G., Marker chromosome 14q+ in non-endemic Burkitt’s

lymphoma, Cancer, 39 (1977) 1495-1499.

8 UICC, Clinical Oncology, 2nd ed, Springer, Berlin, 1978.