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Primary diffuse leptomeningeal oligodendrogliomatosis with an isolated 1p deletion
Samuel Gatzert1, Aditya Durgam1, Karthikram Raghuram2
1Department of Radiology, UT Southwestern Medical School, Dallas, TX, USA.
Abstract:
We report a case of primary diffuse leptomeningeal oligodendrogliomatosis with an isolated 1p deletion confirmed by fluorescent in situ hybridization (FISH) analysis in 52 year-old man. The MRI scan in a patient presenting with progressive headache, nausea and diplopia revealed diffuse leptomeningeal thickening and enhancement without definitive evidence of an intraparenchymal lesion. Biopsy of thickened, enhancing meninges within the left sylvian fissure revealed subarachnoid proliferation of oligodendroglial cells with varying degrees of de-differentiation and sparing of the underlying cortex. An isolated 1p deletion was confirmed by FISH analysis. This is the first reported adult case of a 1p deletion in primary diffuse leptomeningeal oligodendrogliomatosis.
Insights
This study details a rare case of primary diffuse leptomeningeal oligodendrogliomatosis in an adult, characterized by an isolated 1p deletion. This finding is significant for understanding brain tumor genetics.
Area of Science:
- Neuro-oncology
- Genetics of brain tumors
- Neurosurgery
Background:
- Primary diffuse leptomeningeal oligodendrogliomatosis is a rare CNS malignancy.
- Oligodendrogliomas often exhibit 1p/19q co-deletions, but isolated 1p deletions are less common.
- Leptomeningeal spread of oligodendroglioma is exceptionally rare.
Observation:
- A 52-year-old male presented with headache, nausea, and diplopia.
- MRI revealed diffuse leptomeningeal thickening and enhancement without a clear intraparenchymal lesion.
- Biopsy of meningeal enhancement showed subarachnoid oligodendroglial cell proliferation.
Findings:
- Histopathology confirmed diffuse leptomeningeal oligodendrogliomatosis.
- Fluorescence in situ hybridization (FISH) analysis confirmed an isolated 1p deletion.
- This represents the first reported adult case of primary diffuse leptomeningeal oligodendrogliomatosis with an isolated 1p deletion.
Implications:
- This case expands the spectrum of oligodendroglioma presentation and genetic alterations.
- Understanding the genetic landscape of rare CNS tumors is crucial for diagnosis and treatment.
- Further research is needed to elucidate the clinical significance of isolated 1p deletions in leptomeningeal oligodendrogliomatosis.
