Images in Neurosurgery

Images in neurosurgery
A 26-year-old female with h/o migraines presented to ED after episode of lightheadedness and 1 month of worsening headaches, and diplopia. Physical exam was notable for bilateral papilledema and left partial third nerve palsy. Magnetic resonance imaging showed a heterogeneously enhancing pineal region mass (Figure 1A (axial), 1B (sagittal), 1C (coronal)), with differential diagnosis including primary pineal and germ cells tumors. Patient underwent an endoscopic third ventriculostomy and biopsy. Intraoperative histopathology was consistent with pineocytoma. Patient had an uncomplicated postoperative course. Unexpectedly, final diagnosis was diffuse midline glioma, WHO IV. Extensive microvascular proliferation (black circles) and cellular atypia was noted on H&E (Figure 1D). Immunohistochemistry was positive for H3-K27M mutation (Figure 1E). To our knowledge, there has been a single other reported case of a pineal region H3-K27M diffuse midline glioma.1

1   D. A. Solomon, M. D. Wood, T. Tihan, A. W. Bollen, N. Gupta, J. J. Phillips, A. Perry, Diffuse Midline Gliomas with Histone H3-K27M Mutation: A Series of 47 Cases Assessing the Spectrum of Morphologic Variation and Associated Genetic Alterations. Brain pathology 26, 569-580 (2016)

Submitted by:
Todd Hollon, Catherine Ziats, Steven E. Sullivan

Affiliations: Department of Neurosurgery, University of Michigan