Presentation
The patient presents with intermittent episodes of transient alterations of awareness for several years. Episodes include blank staring with loss of memory during the episodes. The episodes last from a few seconds to minutes and may occur as frequently as twice a day or as infrequently as once a week.
Patient Data
MRI of the brain displays a well-circumscribed mass with a lobulated contour centered in the region of the right foramen of the Monro. There is an inferior extent into the suprasellar cistern with encasement of the A1 segment of the right anterior cerebral artery. There is no associated luminal narrowing. The mass is markedly hypointense on T1 and hyperintense on T2, though slightly less so compared to CSF. On FLAIR, there is partial suppression centrally with a peripheral rim of high signal. There is no associated edema, hemorrhage, calcification, diffusion restriction, or enhancement. No other lesions are identified.
Case Discussion
This case displays a classic example of a myxoid glioneuronal tumor, which was pathologically proven upon surgical resection with the presence of the PDGFRA mutation on molecular profiling. On imaging as well as histology and immunohistochemistry, these tumors appear very similar to dysembryoplastic neuroepithelial tumors which are also known to have a bright rim sign. However, the characteristic location of imaging and molecular profiling allows for accurate distinction.
Unable to process the form. Check for errors and try again.