Clinicopathological Characteristics of Adamantinomatous and Papillary Craniopharyngiomas: University of California, San Francisco Experience 1985-2005

BACKGROUND: Craniopharyngiomas are rare epithelial tumors that are presumed to arise from the remnants of Rathke's pouch. OBJECTIVE: This study was designed to evaluate the outcome characteristics of craniopharyngiomas treated in a single institution and to determine whether the adamantinomatous craniopharyngioma should be considered more aggressive than a World Health Organization (WHO) grade I neoplasm. METHODS: We identified all patients with craniopharyngioma given their diagnoses at University of California, San Francisco in a 20-year period and performed a retrospective analysis of clinicopathological and outcome characteristics. Statistical analyses were performed to determine factors that affect survival characteristics. RESULTS: Eighty patients were included in the study based on the selection criteria. Sixty-nine tumors were adamantinomatous, 9 were papillary, and 2 were unclassified. All pediatric tumors were adamantinomatous. Visual field examination in 60 patients revealed a defect in 39 and only 14 showed bitemporal field defects. Hormonal tests with abnormal results were more common in younger patients. During a median follow-up of 82 months, 38 tumors recurred. Four of 9 gross total resections and 34 of 59 subtotal resections recurred. Median time to recurrence was 16.3 months for gross total and 11.7 for subtotal resections. Progression-free survival did not differ between males and females or children and adults. There was a negative correlation between age and overall survival. CONCLUSION: Adamantinomatous craniopharyngioma is a locally aggressive neoplasm with a significant rate of recurrence. This is not in keeping with the current designation of a WHO grade I neoplasm. Subtotal resection is associated with less mortality/morbidity but a higher recurrence rate. Given the high numbers of silent defects, formal visual field testing should be performed in all patients with craniopharyngiomas.