Primary central nervous system lymphoma
ORPHA:46135· ICD-10 C83.3
Definition
Primary central nervous system lymphoma (PCNSL) is a rare nervous system tumor, predominantly due to diffuse large B-cell lymphoma, that involves brain, leptomeninges, eyes, or rarely spinal cord, in the absence of systemic diffusion at the time of diagnosis. It is characterized by a solitary tumor that, depending on its location, can lead to a variety of symptoms such as headache, nausea, vomiting (and other signs of raised intracranial pressure), focal neurologic deficits, neuropsychiatric and ocular symptoms, seizures and personality changes.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly