The Egyptian Journal of Neurology, Psychiatry and Neurosurgery (Dec 2019)

Cerebellar glioblastoma multiforme in an adult patient with neurofibromatosis type 1: an extremely rare report with review of literature

  • Nima Derakhshan,
  • Nazanin Azadeh,
  • Arash Saffarian,
  • Mousa Taghipour,
  • Keyvan Eghbal,
  • Amirreza Dehghanian

DOI
https://doi.org/10.1186/s41983-019-0135-2
Journal volume & issue
Vol. 55, no. 1
pp. 1 – 6

Abstract

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Abstract Background Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder with autosomal dominant inheritance which predisposes the affected individuals to increased risk of developing certain benign and malignant central nervous system (CNS) tumors. NF1 patients are most notably prone to develop low-grade optic pathway, brainstem, and cerebellar astrocytoma. Current literature suggests that brain tumors in patients with NF1 tend to be less aggressive compared to sporadic ones. Glioblastoma multiforme (GBM) is a high-grade glioma which is relatively rare in patients with NF1 and is most commonly seen in supratentorial regions of the brain. Case presentation A 33-year-old patient was admitted in neurosurgery ward with acute hydrocephalus caused by a cerebellar mass lesion. On primary assessment, the patient was diagnosed with NF1. He was followed for 2 months and underwent surgical resection of the mass due to worsening symptoms. The pathology report revealed the malignant nature of the lesion. Patient received adjuvant chemo-radiotherapy with diagnosis of cerebellar GBM. Up to 19 months following surgery, he had gained a relatively well ability to walk and talk again.