Brainstem angiocentric gliomas with MYB–QKI rearrangements

Excerpt

Angiocentric glioma is a low-grade CNS tumor typically found in the cerebral hemispheres where it causes refractory epilepsy [3]. Surgical resection is often sufficient for seizure control and the rate of tumor recurrence is low [1]. On pathology, this neoplasm shares some cytologic features with pilocytic astrocytoma but is differentiated by its specific vascular polarity, its infiltrative growth pattern, and its epithelial membrane antigen (EMA) immunoreactivity [3]. In an initial study, a MYB–QKI rearrangement was present in 6 of 7 angiocentric gliomas and absent in 147 other gliomas, making it a potential diagnostic marker in ambiguous or atypical cases [2]. A subsequent study of a large number of cerebral low-grade gliomas confirmed the specificity of MYB–QKI rearrangements for angiocentric gliomas [5]. A midbrain tumor resembling angiocentric glioma was previously reported but the presence of the rearrangement was not assessed [4]. Here, we report two cases of brainstem angiocentric gliomas with evidence of MYB–QKI rearrangements. …