Markers of Recurrence and Long-Term Morbidity in Craniopharyngioma: A Systematic Analysis of 171 Patients

• Published in: The journal of clinical endocrinology and metabolism Vol. 97; no. 4; pp. 1258 - 1267
• Main Authors: Gautier, Alain, Godbout, Ariane, Grosheny, Catherine, Tejedor, Isabelle, Coudert, Mathieu, Courtillot, Carine, Jublanc, Christel, De Kerdanet, Marc, Poirier, Jean-Yves, Riffaud, Laurent, Sainte-Rose, Christian, Van Effenterre, Remy, Brassier, Gilles, Bonnet, Fabrice, Touraine, Philippe, on behalf of the Craniopharyngioma Study Group: Prs. and Drs. Anne Bachelot, Anne-Laure Boch, Raja Brauner, Jean-Claude Carel, Anne Colobert, Hélène Crosnier, Vonny Derennes, Pierre Doyard, Françoise Getin, Isabelle Guilhem, Muriel Hoang, Claire Josseaume, Julianne Leger, Sylvie Nivot, Christian Pauwels, Graziella Pinto, Michel Polak, Raphaël Rappaport, Dinane Samara, Sylvie Sauvion, Caroline Thalassinos, and Elisabeth Thibaud
• Format: Journal Article
• Language: English
• Published: Bethesda, MD Endocrine Society 01.04.2012
• Subjects: Adolescent; Adult; Age of Onset; Bioengineering; Biological and medical sciences; Child; Child, Preschool; Cohort Studies; Craniopharyngioma; Craniopharyngioma - diagnosis; Craniopharyngioma - mortality; Craniopharyngioma - psychology; Craniopharyngioma - therapy; Craniotomy; Craniotomy - adverse effects; Endocrinology and metabolism; Endocrinopathies; Feeding. Feeding behavior; Female; Follow-Up Studies; France; Fundamental and applied biological sciences. Psychology; Human health and pathology; Humans; Intracranial Hypertension; Intracranial Hypertension - etiology; Life Sciences; Male; Medical sciences; Middle Aged; Neoplasm Recurrence, Local; Neoplasm Recurrence, Local - diagnosis; Neoplasm Recurrence, Local - mortality; Neoplasm Recurrence, Local - psychology; Neoplasm Recurrence, Local - therapy; Prognosis; Quality of Life; Retrospective Studies; Social Adjustment; Survival Analysis; Vertebrates: anatomy and physiology, studies on body, several organs or systems; Vertebrates: endocrinology; France; Endocrinopathy; Human; Intracranial; Obesity; Nervous system diseases; Relapse; Statistical analysis; Nutrition; Nutrition disorder; Biological marker; Patient; Metabolic diseases; Epidemiology; Long term; Morbidity; Tumor; Craniopharyngioma; Endocrinology; Nutritional status
• ISSN: 0021-972X; 1945-7197
• DOI: 10.1210/jc.2011-2817

Context: Craniopharyngiomas are often associated with an unfavorable prognosis, but data on their long-term consequences are sparse. Objective: The aim of the study was to identify markers of recurrence and factors associated with compromised social rehabilitation and altered quality of life in a large cohort of patients with either childhood-onset (CO) or adult-onset craniopharyngioma. Methods: Retrospective analysis was performed for 171 patients treated for craniopharyngioma in two academic centers in France between 1972 and 2009. For each subject, data were collected concerning clinical presentation, imaging features, visual sequelae, endocrine and metabolic impact, treatment modalities (surgery, radiotherapy), recurrence-free survival rate, and social insertion, as well as answers to the WHO-QOL BREF questionnaire. Results: A total of 65 CO and 106 adult-onset patients were reviewed. If CO was diagnosed before the age of 10 yr, this was associated with a higher incidence of obesity, blindness, and panhypopituitarism, and only 40.7% of subjects had adequate work or school attendance compared to 72.4% of patients with later disease onset. Initial symptoms of intracranial hypertension (SIHT), pterional surgery, and multiple surgery were associated with obesity and poorer social insertion. No determinant of quality of life was identified. In the subgroup of patients treated in the 1990s and later, the progression rate was 59.4% in patients with residual tumor on magnetic resonance imaging compared with a 19.8% recurrence rate in the group with apparently complete resection. Recurrence/progression correlates significantly with male gender, early onset (before 10 yr), and SIHT, but only SIHT at presentation remained a significant predictor with multivariate analysis. Conclusions: Craniopharyngioma continues to be associated with severe outcomes. Higher morbidity rates are found in patients with early-onset disease (before 10 yr), initial SIHT, or in whom pterional surgery was required. Markers of recurrence are difficult to identify, with SIHT being the most powerful predictor.