Nonendemic Burkitt's lymphoma with jaw involvement: case report.

Toothache of a lower primary molar followed by bilateral swelling of the lower molar regions were the first symptoms of a 6-year-old Finnish male with Burkitt-type malignant lymphoma. Radiographic examination revealed osteolytic changes in the right mandibular and left maxillary quadrant. The cortical bone around the affected teeth was absent and some teeth were grossly displaced. When the therapy was started the teeth returned to their normal positions within a week. Descriptions are given of the jaw lesions, and the clinical, histological, and radiographic diagnosis as well as the clinical course and treatment of the tumor. Burkitt’s lymphoma is a distinct pathologic entity characterized as a diffuse undifferentiated malignant lymphoma of B-lymphocyte origin (Mann et al. 1976). The tumor was first described by Dr. Dennis Burkitt in children from regions of equatorial Africa (Burkitt 1958). Following the original reports of endemic African lymphoma, similar cases began to be identified in other parts of the world, including the United States where this lymphoma is now known as nonendemic or American Burkitt’s lymphoma (Cohen et al. 1969). Endemic and nonendemic Burkitt’s lymphomas are histologically indistinguishable, but differences exist with respect to presenting tumor sites, age of onset, patterns of relapse, and immunological findings. In endemic areas of Africa, Burkitt’s lymphoma is the most common early childhood malignancy. Its peak age of occurrence is between the 4th and 7th years (Hupp et al. 1982). In nonendemic areas, the mean peak age has been given as around 12 years (Hupp et al. 1982; Terrill et al. 1977). The predilection for the jaw is typical for endemic Burkitt’s lymphoma, but the jaws are involved in only 15-18% of nonendemic cases (Ziegler and Magrath 1974, Adatia 1978, Sariban et al. 1984). The most i Hupp et al. 1982; Terrill et al. 1977; Eisenbud et al. 1985. frequently involved sites in nonendemic cases are the terminal ileum, cervical lymph nodes, and bone marrow (Ziegler et al. 1979). When involving the jaws, the tumor can cause jaw tenderness and loosening and displacement of teeth, coupled with the radiographic signs of generalized destruction of tooth crypts and diffuse disruption of jaw trabeculation. Its rapid expanding growth also causes intraand extraoral swelling and gross distortion of the face (Burkitt 1958; Sariban et al. 1984). Both endemic and nonendemic cases of Burkitt’s lymphoma are very sensitive to chemotherapy (Ziegler et al. 1979). However, in the nonendemic cases two patterns of relapse may be seen. There may be an early relapse after a short remission. If this occurs, the disease is resistant to further treatment and the prognosis is poor. A second pattern of relapse after a long remission also may be seen. In this situation, the response to treatment is comparable to previously untreated patients (Terrill et al. 1977). In nonendemic cases of the disease, only the early relapse pattern has been noted (Terrill et aL 1977). In the endemic form an association of Epstein-Barr virus and malaria has been linked to the pathogenetic mechanism for Burkitt’s lymphoma. Epstein-Barr virus-related antigens have been found in about 95% of all cases of endemic Burkitt’s lymphomas, but only in about 25% of the cases of nonendemic Burkitt’s lymphomas (Pageno et al. 1973). In Finland, 4-5 cases of Burkitt’s lymphoma or Burkitt’s lymphoma-type tumor are registered annually. This report deals with facial and jaw involvement of a young Finnish boy, who had Burkitt-type malignant lymphoma.