Craniopharyngioma

Snapshot

• A 6-year-old boy has had a daily dull headache for the past 6 months. He says that it is a continuous, dull headache that increases a bit every day. He can’t focus on playing with friends at school anymore due to the pain. On physical exam, his physician is concerned when the patient’s visual field is notably decreased bitemporally. Additionally, physical exam reveals papilledema bilaterally. A stat CT scan is done, revealing suprasellar calcifications.

Introduction

• Childhood tumor in the brain
• Pathogenesis
  • derived from remnants of Rathke pouch 
    • recall that Rathke pouch forms the adenohypophysis in week 4 of gestation
    • arises from surface ectoderm
  • benign on histology
  • slow growing
  • malignant behavior with invasion in local structures
    • may recur after resection
    • metastases rare
  • blood supply typically from anterior circulation of brain
  • tumor characteristics include dystrophic calcification, cyst formation, and hemorrhage 
• Epidemiology
  • most common childhood supratentorial tumor
• most common cause of pituitary hypofunction in children

Presentation

• Symptoms/physical exam 
  • most commonly headaches
    • progressive, dull, and continuous
  • bitemporal hemianopia 
    • recall pituitary adenomas present similarly
    • due to compression of optic chiasm
  • can compress pituitary gland and cause hypopituitarism
    • hypothyroidism
    • adrenal failure
• diabetes insipidus

Evaluation

• CT scan with contrast for diagnosis
  • suprasellar calcified cyst
• Histology
  • cholesterol crystals
• calcifications

Differential Diagnosis

• Pituitary adenoma
• Glioma
• Medulloblastoma
• Meningioma

Treatment

• Surgical resection
• If not completely resectable
• follow with radiotherapy

Prognosis, Prevention, and Complications

• Prognosis
  • better prognosis for patients < 20 years
    • 5-year-survival rate 99%
  • poorer prognosis for older than 65 years
    • 5-year-survival rate 38%
• Complications
  • vision loss