RAISED INTRACRANIAL PRESSURE IN AN ADULT PATIENT REVEALS A VENTRICULAR NEURONAL-GLIAL TUMOUR: A CASE PRESENTATION AND SHORT REVIEW OF LITERATURE

Authors

  • Ανδρέας Ελευθερίου ΕΚΠΑ
  • Martin Nilsson Department of Neurosurgery in Linköping, and Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
  • Νικόλαος Σαριδάκης Department of Neurology and Department of Biomedical and Clinical Sciences, Division of Neurobiology, Linköping University, Linköping, Sweden
  • Αγγελική Παπαγιαννοπούλου Department of Clinical Pathology, and Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
  • Κωνσταντίνος Μελάνης Second Department of Neurology, "Attikon" University Hospital, School of Medicine, National and Kapodistrian University of Athens, Greece.
  • Ida Blystad Department of Radiology in Linköping, and Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden
  • Rafael Holmgren Department of Neurosurgery in Linköping, and Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden

Keywords:

PNET, NOS, ventricular embryonal central nerve system tumor, hydrocephalus, leptomeningal contrast enhancement

Abstract

We present a very rare case of a middle-aged female who presented with clinical and radiological signs of elevated intracranial pressure non responding despite double external ventricular drain. An explorative ventricular endoscopy appealed a tumor which partially obstructed the foramen of Monroe. Immunohistochemical tests showed that tumor cells were positive for synaptophysin, GFAP and focally for CD99 while they were negative for CD20, TTF1 and CK CAM. The Ki67 profileration marker was measured at approximately 70%. Thus, the morphological image and immune phenotype of the tumor were compatible with PNET. Molecular analysis showed no MGMT methylering of mutation showed in TP53, IDH1, hTERT promoter and the picture was associated with glioneuronal tumor. The patient diagnosed with an embryonal tumor with multilayered rosettes, which was initially difficult to identify. According to our knowledge, the location of this type of tumor is very uncommon and less than 5 cases have been described according to a short review of literature.

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Published

2023-07-19

Issue

Vol. 32 No. 3 (2023): Volume 32, No. 3, 2023

Section

Reviews