Diffuse primary non-Hodgkin's lymphoma of the cranial vault

Primary non-Hodgkin's lymphoma of the skull vault is a rare disease. We describe a case occurring in a 72-year-old woman presenting with generalized tonic clonic seizures on a background of a 1-year history of headaches and progressively enlarging scalp masses. Imaging showed diffuse infiltration of the skull vault with multifocal intra- and extracranial soft tissue masses, causing compression and probably infiltration of the cerebral cortex. Further investigation failed to identify any other evidence of systemic lymphoma. Biopsy of one of the scalp masses showed a small to intermediate cell B cell lymphoma. The other nine reported cases of primary skull vault lymphoma are reviewed. The diffuse vault infiltration as well as the multiple intracranial, scalp and temporalis muscle masses renders this case unique.