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

• Published in: Neurosurgery Vol. 67; no. 5; pp. 1341 - 1349
• Main Authors: Pekmezci, Melike, Louie, Jonathan, Gupta, Nalin, Bloomer, Michele M, Tihan, Tarik
• Format: Journal Article
• Language: English
• Published: United States Oxford University Press 01.11.2010; Wolters Kluwer Health, Inc
• Subjects: Adolescent; Adult; Aged; Bone cancer; California - epidemiology; Child; Child, Preschool; Craniopharyngioma - mortality; Craniopharyngioma - surgery; Female; Humans; Infant; Infant, Newborn; Longitudinal Studies; Male; Middle Aged; Neoplasm Recurrence, Local - mortality; Neoplasm Recurrence, Local - prevention & control; Pituitary Neoplasms - mortality; Pituitary Neoplasms - surgery; Postoperative Complications - mortality; Prevalence; Risk Assessment; Risk Factors; Survival Analysis; Survival Rate; Treatment Outcome; Tumors; Young Adult; California; United States > US; San Francisco California; Papillary; Craniopharyngioma; Neoplasia; CNS tumors; WHO grading; Adamantinomatous
• ISSN: 0148-396X; 1524-4040
• DOI: 10.1227/NEU.0b013e3181f2b583

Abstract 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.