Freiburg Neuropathology Case Conference: A Pineal Region Tumour in a Child
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Clinical Case
Freiburg Neuropathology Case Conference: A Pineal Region Tumour in a Child C. A. Taschner · S. Brendecke · V. van Velthoven · C. J. Maurer · M. Prinz
Published online: 20 October 2013 © Springer-Verlag Berlin Heidelberg 2013
Case Report A 7-year-old boy, complaining headaches and vomiting, was admitted with suspicion of meningitis. Magnetic resonance imaging (MRI), however, revealed a large tumour in the pineal cistern, causing obstructive hydrocephalus. An endoscopic ventriculocisternostomy and a biopsy of the tumour were performed primarily. Histopathological diagnosis was difficult and final classification was pending. Since the symptoms of intracranial pressure were dissolved and the neurological condition of the patient improved, he was dismissed from hospital until the final therapeutic decision would be settled. Two months later the boy was hospitalised with headache and vomiting. MRI showed an increase of tumour volume with consecutive hydrocephalus. An external ventricular drainage was positioned followed by a suboccipital, infratentorial, supracerebellar approach. The tumour had a well-defined vascularised capsula, the interior being consistent and rubber-like, so that inner debulking was only possible by ultrasonic aspirator. The most upper and ventrally localised part of the tumour included hair. After progressive diminishing, the capsula could easily be mobilised and the tumour removed in total. The post-
C. A. Taschner () · C. J. Maurer Department of Neuroradiology, University Hospital Freiburg, Freiburg, Germany e-mail: [email protected] S. Brendecke · M. Prinz Department of Neuropathology, University Hospital Freiburg, Freiburg, Germany V. van Velthoven Department of Neurosurgery, University Hospital Freiburg, Freiburg, Germany
operative course of the patient was uneventful without neurological deficits, the external drainage was removed. After final diagnosis, the patient was treated following the SIOP GCT CNS II protocol with chemo- and radiation therapy. Imaging T2-weighted MR images show a cystic lesion located in the pineal region. The lesion displays a nodular component adherent to the wall of the cyst (Fig. 1a). The surrounding tissue shows an increase in signal intensity (Fig. 1b). Circulation of cerebrospinal fluid (CSF) is impaired because of obstruction of the aqueduct with clear distension of the lateral ventricles (not shown). After cisternotomy, CSF circulation is back to normal. On non-enhanced T1-weighted images the solid component of the lesion appears isointense when compared to the grey matter (Fig. 2a). The solid component of the lesion shows homogenous enhancement of Gadolinium (Fig. 2b–d). On non-enhanced computed tomography, the solid component of the lesion appeared hyperdense (Fig. 3). On follow-up images after an initial biopsy the lesion clearly increased in size (Fig. 4). Differential Diagnosis Germinoma The pineal region is the most common location for intracranial germinomas followed by the suprasellar region. Interestingly, ge
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