Craniopharyngioma

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Craniopharyngioma
CraniosagMRI.jpg
Sagittal MRI of suprasellar craniopharyngioma
Tumor Class unknown
Cell of origin ectoblastic cells of the the craniopharyngeal duct
WHO Grade I
Survival Young (<20 years) = 99% at 5 years

Old (>65) = 38% at 5 years Overall 80% at 5 years, 64% at 10 years

Treatment Surgery and/or radiation
Histologic Features
Gross cystic with "crankcase oil" fluid but varies
Micro Adamantinomatous (peds) = columnar epithelium with keratin

Papillary (adult) = primarily squamous epithelium

Radiographic Features
MRI usually a cystic suprasellar mass with a nodular component
CT hypodense, may have calcification
  • i. Unknown histogenesis
  • ii. Two types:
    • 1. adamantinomatous
      • a. groups of squamous cells surrounded by a peripheral palisade of columnar cells along with cohesive clusters or sheets of keratinized anuclear ghost cells, calcification, and cholesterol clefts
    • 2. papillary
      • a. groups of squamous cells that form keratin pearls, no anuclear ghost cells, rare calcifications, solid, encapsulated, well differentiated solid sheet like cells; stains positive for cytokeratin and EMA
  • iii. adamantinomatous type tends to occur in children while both types occur in adults
  • iv. patients present with symptoms of hydrocephalus
  • v. most common tumor to produce suprasellar calcification
  • vi. nearly all children with a craniopharyngioma will have significant endocrine problems after surgery; nearly all have anterior and posterior pituitary deficiency whether or not complete tumor removal is achieved; most serious complication is obesity that develops in about ½ of patients due to hypothalamic damage; DI occurs in about 90% of postoperative craniopharyngioma patients
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