Freiburg Neuropathology Case Conference:
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CLINICAL CASE
Freiburg Neuropathology Case Conference: Blurred Vision and Headaches in a 15-year-old Boy S. Doostkam1 · U. Würtemberger2 · V. Coenen3 · H. Urbach2 · M. Prinz1 · C. A. Taschner2 © The Author(s) 2020
Keywords Pineoblastoma · Pineocytoma · Germinoma · Teratoma · Tectal glioma
Case Report
Imaging
This 15-year-old boy was diagnosed with papilledema and papillary venous bleeding diagnosed as a result of a workup after 6–9 months of persistent headaches, which typically occurred twice a week. The character was pounding and at times reached 6–7 points on a 10-point intensity scale. Besides blurred vision and headaches the neurological examination was non-focal, especially no problems passing urine or mental decline were detected. A pineal region tumor was found on brain magnetic resonance imaging (MRI, Figs. 1, 2, 3 and 4). Blood samples were negative for alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (beta- HCG). After interdisciplinary discussion in the tumor board an endoscopic third ventriculostomy was performed together with a stereotactic biopsy of the contrast enhancing mass of the posterior third ventricle. The postoperative course was uneventful and after surgery the patient recovered to the normal baseline with no headaches and improved vision.
On axial T2-weighted images (Fig. 1a) an isointense lesion (arrow) located in the pineal region was found which led to subsequent triventricular dilatation (arrowhead). On sagittal FLAIR images (Fig. 1b) the lesion (arrow) compresses the aqueduct (arrowhead). The lesion (Fig. 1c, arrow) does not show any signs of restricted diffusion on diffusion-weighted images (DWI). On nonenhanced axial T1 weighted images (Fig. 2a) the lesion (arrow) is isointense when compared to the brain parenchyma and does not show any signs of infiltration of the surrounding tissue. After administration of gadolinium the lesion shows marked enhancement (Fig. 2b–d, arrow). Note the deep cerebral veins entrapped within the tumor matrix (Fig. 2c, arrowhead) and the delicate capsule surrounding the lesion (arrowhead, Fig. 2d). On postcontrast computed tomography (CT) images the lesion shows a firm, centrally located calcification (Fig. 3, arrowhead).
Differential Diagnosis Pineoblastoma
C. A. Taschner
[email protected] 1
Department of Neuropathology, Medical Centre—University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany
2
Department of Neuroradiology, Medical Centre—University of Freiburg, Faculty of Medicine, University of Freiburg, Breisacherstraße 64, 79106 Freiburg, Germany
3
Department of Stereotactic and Functional Neurosurgery, Medical Centre—University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany
Pineoblastomas are highly malignant embryonal World Health Organization (WHO) grade IV tumors of the pineal gland, which clinically manifest with symptoms of elevated intracranial pressure due to aqueduct obstruction and subsequent obstructive hydrocephalus, such as nause
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