Clinical, Radiological and Outcome Characteristics of Central Nervous System Lymphomatoid Granulomatosis
Georg Scheffler1, Leon Kaulen1, Joachim Baehring1
1Department of Neurology, Yale School of Medicine
Objective:
We aimed to characterize clinical and radiological presentation and outcome of central nervous system lymphomatoid granulomatosis (CNS LYG).
Background:
CNS LYG is a rare lymphoproliferative disorder affecting the CNS recognized in the current WHO classification of brain tumors. Further clinical and radiological characterization is warranted.
Design/Methods:
The Yale brain tumor data base was retrospectively screened for CNS LYG. Clinical, radiological, pathological and outcome data were collected.
Results:
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.Conclusions:
Distinct radiological phenotypes of CNS LYG may reflect histological grade. In low grade cases immune reconstitution may be sufficient to treat. Excellent outcomes can be achieved with methotrexate/rituximab based polychemotherapy in higher grade disease.
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