Rapid and sustained response of an intra- and extracranial large cell lymphoma mass to liposomal intrathecal Ara-C and R-MegaCEOP systemic chemotherapy.

Prognosis of patients suffering from secondary central nervous system (CNS) lymphoma is dismal. Intracranial spread of a lymphoma arising in adjacent extranodal tissues is a rare event. A 32-year-old patient was diagnosed with progressive diffuse large B-cell lymphoma (DLBCL) with extra- and intracranial localization. He complained of headache, left diplopia, marked rigidity of the neck muscles, and difficulty in swallowing and articulating words, caused by bilateral palsy of the XII cranial nerve. Computed tomography (CT) and positron emission tomography (PET) scans showed disease localizations in the occipital-cervical soft tissue, and cerebellar parenchyma. Due to the severity of the clinical picture, high-dose dexamethasone was immediately administered. Mild improvement was observed during the first 2 days of treatment, but dramatic reduction of symptoms and nerve palsy was documented only in the 48 h after the first intrathecal administration of liposomal Ara-C. Systemic R-MegaCEOP chemotherapy was started 7 days later. Concomitant intrathecal liposomal Ara-C injections were continued for a total of nine administrations during the eight cycles of immunochemotherapy without any toxicity observed. Interim and post-therapy PET showed complete resolution of radionuclide accumulation in the involved sites. Consolidation radiotherapy (36 Gy) was administered in involved areas after the completion of the immunochemotherapy program. At the time of writing, no cumulative neurotoxicity is evident at follow-up of 17 months from diagnosis and 9 months after the overall therapeutic program has been accomplished.