Primary lymphoma of the central nervous system: experience at Addenbrooke's Hospital, Cambridge

A series of 22 cases of primary intercerebral lymphoma are reported. The clinical presentation and natural history were similar to high grade glioma in adults. There was a male to female ratio of 2.7:1, and a mean age at presentation of 63 years (range 47-72 years). Single masses were present in 77%, and the frontal lobe was the most frequently involved (64%). Treatment regimes included varying combinations of surgical resection and cranial irradiation. Sixteen patients have died, twelve due to recurrent or persistent intracerebral disease. The median survival was ten months. Six patients are still alive at 52, 12, 10, 9, 6 and 4 months. Failure to control the intracranial tumours was the main cause of death. Significant debulking of the tumour was the single most important prognostic factor, although a good initial response to steroids was also favourable. No patient developed spinal deposits. Even when there is a good response to radiation and prolonged survival, the quality of life may be poor: in this series only five out of 22 patients (23%) returned to a normal life. Radiation dosage and the indications for spinal treatment are discussed.