Intracerebral Burkitt's Lymphoma: Pathology, Clinical Features and Treatment

Abstract

Intracerebral Burkitt's lymphoma has previously been reported as a postmortem finding. Six patients are described here in whom intracerebral Burkitt's lymphoma was diagnosed prior to death. In all cases it was a manifestation of relapsing tumour. Clinical presentations included headache and papilloedema, altered consciousness, and a variety of localizing neurological signs. In five patients who did not survive, the duration of the illness was short (four to 18 days). Post-mortems in the patients who died showed that cerebral oedema and perivascular cuffing by tumour cells were universal. The latter probably indicates the route of entry into the brain by tumour cells, which in most cases appears to be direct spread from the meninges, although blood stream spread is not excluded, and may be an alternative route. There was a tendency for tumour cells to infiltrate diffusely rather than form nodules, so that localizing signs appeared late in the time-course. In the one patient who survived, a combination of intrathecal treatment with cytosine arabinoside and methotrexate, and oral hydroxyurea had been given. The latter agent is lipid soluble and crosses the blood brain barrier. In addition, intensivesystemic treatment with cyclophosphamide, methotrexate and vincristine was administered. The diagnosis of intracerebral Burkitt's lymphoma is probably occasionally missed, since its occurrence is not widely recognized. In patients being followed-up, suspicion should arise when persistent headache, other symptoms of raised intracranial pressure, or localizing neurological signs develop. Because of its rapid course, treatment must be commenced as soon as the diagnosis is reasonably certain. It is probably wise to give both intrathecal drugs (via an Ommaya reservoir if possible) and systemic hydroxyurea. Dexamethasone should be routinely administered in an attempt to reduce raised intracranial pressure.