The results of therapy of 57 previously untreated patients with Burkitt's tumor seen over a 2‐year period at the Lymphoma Treatment Center, Kampala, Uganda are reported. Thirty‐seven patients were randomized to either a single dose or multiple doses of intravenous cyclophosphamide (CTX) 40 mg/kg. Ten of 11 patients with localized (stage I‐II) disease have continued in complete remission regardless of drug schedule. Among stage III patients, remission duration was shorter and relapse was more common in the group receiving a single dose. Intrathecal chemotherapy using methotrexate and cytosine arabinoside was successfully employed in the management of 12 patients with malignant cells in the cerebrospinal fluid. However, more aggressive and prolonged therapy is indicated to prevent relapse. Secondary chemotherapy using vincristine, methotrexate, and cytosine arabinoside produced complete responses in 90% of patients relapsing on multiple‐dose CTX. This study showed that: 1. single doses of CTX are capable of inducing long remissions in patients with localized disease; 2. multiple doses of CTX do not appear harmful in the maintenance of remissions in patients with localized tumors; 3. multiple doses of CTX result in fewer relapses and longer remissions in patients with visceral (stage III) disease compared with a single‐dose regimen; 4. the prognosis is improved in stage III patients who undergo surgical reduction of tumor bulk prior to chemotherapy; and 5. recognition and aggressive treatment of patients in relapse, particularly those with central nervous system involvement, are important considerations in the long term management of Burkitt's tumor.
Thirty-five of 77 patients (46%) with Burkitt’s lymphoma presented or developed evidence of central nervous system involvement by tumor. Neurologic abnormalities included paraplegia, cranial neuropathy, altered levels of consciousness and malignant pleocytosis. An analysis of this series disclosed the following: Paraplegia is a common presenting feature of Burkitt’s lymphoma and is responsive to systemic chemotherapy. The association of cranial neuropathy and malignant pleocytosis with facial tumors points to direct tumor extension to intracranial structures (duraarachnoid) as the pathogenesis of these lesions. Intrathecal chemotherapy temporarily reverses malignant pleocytosis but systemic chemotherapy is required to treat cranial neuropathy. A poor prognosis follows presentation or development of malignant pleocytosis. The limitations of the current forms of therapy for CNS involvement are discussed.
A series of 66 patients with Burkitt's lymphoma or childhood lymphoma of the histiocytic or lymphoblastic type has been analysed. The six patients with histiocytic lymphomas had clinical features, response to therapy and survival rates which clearly distinguished this group from Burkitt's lymphoma. Of the six patients with lymphoblastic lymphomas, however, three presented with clinical features indistinguishable from Burkitt's lymphoma while two had facial tumors which showed atypical features. The response to chemotherapy with CTX was more favorable in the lymphoblastic than in the histiocytic group, and the survival rate in this small number of patients falls midway between those of histiocytic lymphoma and Burkitt's lymphoma. Thus, the clinical evaluation of Ugandan children with lymphoblastic lymphoma did not clearly distinguish this group from patients with Burkitt's lymphoma, and casts some doubt on the use of clinical features or response to therapy as diagnostic criteria.
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