Clinical Characteristics of Childhood-Onset Craniopharyngioma

Craniopharyngioma (CP) is an intracranial congenital epithelial tumor that can occur at any age. CP tumors are histologically benign (WHO grade I), and childhood‑onset CP (CO-CP) patients have a high rate of survival. The major concern for CO-CP patients is delayed diagnosis. Delayed diagnosis can further lead to serious adverse consequences such as acute and chronic complications, thereby endangering the life of the patient.We evaluated the early-stage clinical characteristics of CO-CP patients to provide clues for making rapid and accurate diagnoses.

This was a retrospective, single-center study. We retrospectively reviewed all pediatric patients (<18 years of age) undergoing CP surgery between 2012 and 2019 at a single institution. Data including demographic data, clinical presentation, neuroendocrine dysfunction, and tumor imaging characteristics at diagnosis were analyzed.

The average age of the 192 children in this study was 7.32±3.94 (0-16) years, 91.0% were diagnosed when under 14 years old, and 92.7% of patients had at least one clinical symptom, and 90.7% of tumors have a diameter greater than 2cm, and 95.9% of tumor consistency was mixed or cystic, and 89.0% of tumors found calcification. The patients with hydrocephalus had higher BMI values than those without hydrocephalus (P = .006), and the incidence of calcification of tumors significantly decreased with age (P = .027).

For pediatric patients with calcification, >2 cm, cystic or mixed intracranial mass lesions, CP tumors should be considered, and early neuroendocrine function evaluation and further surgical treatment should be performed to avoid delayed diagnosis.