Three cases of CNS LYG were identified of which two occurred in the setting of previous systemic disease (lung). One of the cases was associated with immunodeficiency (HIV infection).
Neuropathological evaluation of CNS lesions revealed grade 1, 2 and 3 in one case each. Radiological presentation was heterogenous and varied by LYG grade: punctate contrast enhancement without restricted water diffusion (grade 1); larger irregularly contrast enhancing lesions without diffusion-weighted imaging abnormalities (grade 2); peripheral contrast enhancement with patchy internal restricted diffusion (grade 3).
In immunodeficiency associated CNS LYG grade 1 immune reconstitution resulted in sustained complete remission. Higher grade CNS LYG was managed with high dose methotrexate and rituximab achieving a complete remission (PFS: 48 months, 53 months). At disease progression one patient developed classic Hodgkin lymphoma in close anatomical relation to initial LYG lung lesions.