Childhood-onset Craniopharyngioma

Area of Science:

• Pediatric neuro-oncology
• Endocrinology
• Neurosurgery

Background:

• Craniopharyngiomas are rare, low-grade (WHO I) embryonic tumors in the sellar/parasellar region.
• The adamantinomatous subtype often presents in childhood with cystic and calcified areas.
• Diagnosis commonly involves hypothalamic/pituitary deficits, visual disturbances, and increased intracranial pressure.

Observation:

• Complete resection is preferred for tumors without hypothalamic involvement, prioritizing optical and hypothalamic function.
• Hypothalamus-sparing surgery followed by irradiation is recommended for tumors with hypothalamic involvement.
• Recurrences and progression are common, despite high overall survival rates (92%).

Findings:

• Disease or treatment can cause severe quality of life impairment.
• Comorbidities include metabolic syndrome, hypothalamic obesity, and neurological deficits.
• Childhood-onset craniopharyngioma is often a chronic condition.

Implications:

• Lifelong, continuous care by multidisciplinary teams is essential.
• Management must address clinical sequelae and quality of life.
• Further research into long-term outcomes and management strategies is warranted.