Hypothalamic-optochiasmatic glioma

Hypothalamic-optochiasmatic gliomas are a subset of astrocytic tumours which have tendency to occur in patients with neurofibromatosis type 1. These may involve the optic nerves, the optic chiasm, and the optic tracts

Gliomas of the optic chiasm and hypothalamus account for 10-15% of supratentorial tumors in children. Males and females are approximately equally affected. 20-50% of patients with hypothalamic gliomas have a positive family history of neurofibromatosis type 1 (NF1). Gliomas of the optic chiasm and hypothalamus in children with NF1 usually have a more indolent course. Tumours may grow more slowly and occasionally regress spontaneously. 

Most are juvenile pilocytic astrocytomas, but their imaging characteristics are not specific with regard to their histologic features. Varying degrees of cystic change and enhancement are demonstrated. The tumours may appear smooth, fusiform, eccentric, or lobulated.

Clinical features may include:

  • decreased visual acuity / optic atrophy ≈50%
  • diencephalic symptoms (change in alertness, hyperactivity)
  • obesity; sexual precocity; endocrine dysfunction (short stature)
  • diabetes insipidus
MRI

MR imaging is optimal for showing the relationship of the mass to the hypothalamus, optic chiasm, and infundibulum as well as the intraorbital and intracanalicular components of the mass. Large tumours are typically heterogeneous with cystic and solid components.

  • T1: almost always hypointense 
  • T2: hyperintense 
  • FLAIR: hyperintense
  • T1 C+ (Gd): solid components of large tumours often contrast enhance

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Article Information

rID: 1484
Section: Pathology
Tags: refs, cases
Synonyms or Alternate Spellings:
  • Hypothalamic glioma

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