International Journal of Medical and Pharmaceutical Case Reports

Primary central nervous system lymphoma (PCNSL) constitutes a rare group of extra nodal Non -Hodgkin’s lymphoma (NHL). Immunodeficiency is the main risk factor phenotype and involvement of the cranial vault is an unusual manifestation of aggressive PCNSL.

We report a case of Primary central nervous system anaplastic lymphoma in an immunocompetent patient.

A 25 year-old man with a history of left hemiplegia, headache, raised intracranial pressure and 8 cm sized cerebral tumor was admitted in Neurosurgery Department of Sahloul Hospital in Sousse, Tunisia in July 2003.

Magnetic resonance imaging (MRI) showed of 3 cm parasagital right heterogeneous cerebral tumor with perilesionnel edema. Biopsy concluded to the diagnosis of meningioma, a resection of the tumor mass was done. Ten months later an occipital cerebral tumor appeared, the biopsy concluded to the diagnosis of large cell anaplastic lymphoma ALK+, CD30+. The patient underwent 4 cycles of chemotherapy ACVBP regimen followed by 2 cycles of high dose Methotrexate and 4 cycles of Etoposide- Ifosfamide and consolidation therapy with whole brain radiotherapy with great tolerability, the patient remain in complete remission without late neurological toxicity within a follow-up of 8 years.

This case illustrate the difficulty of pathological diagnosis of PCNSL and the possibility of complete remission with combination of chemotherapy regimens with drugs passing the blood brain barrier and radiotherapy have given the best reported results up to now.