Diagnosis and management of children and adult craniopharyngiomas: A French Endocrine Society/French Society for Paediatric Endocrinology & Diabetes Consensus Statement
Thomas Cuny, Rachel Reynaud, Gérald Raverot, R. Coutant, Philippe Chanson, Dulanjalee Kariyawasam, Christine Poitou, Cécile Thomas‐Teinturier, Bertrand Baussart, Dinane Samara‐Boustani, L. Feuvret, Carine Villanueva, Chiara Villa, Benjamin Bouillet, M. Tauber, Stéphanie Espiard, Sarah Castets, Albert Beckers, Jessica Amsellem, Marie‐Christine Vantyghem, Brigitte Delemer, Nicolas Chevalier, Thierry Brue, Nicolás André, V. Kerlan, Thomas Graillon, I. Raingeard, Claire Alapetite, Véronique Raverot, Sylvie Salenave, A. Boulin, Romain Appay, Florian Dalmas, Sarah Fodil, Lucie Coppin, Camille Buffet, Philippe Thuillier, Frédéric Castinetti, Guillaume Vogin, Laure Cazabat, Emmanuelle Kuhn, Magalie Haissaguerre, Yves Reznik, Bernard Goichot, Anne Bachelot, Peter Kamenický, Bénédicte Decoudier, Charlotte Planchon, Jean‐Arthur Micoulaud‐Franchi, Pauline Romanet, David Jacobi, Pauline Faucher, Claire Carette, H. Bihan, D. Drui, Sylvie Rossignol, Lucile Gonin, Emmanuelle Sokol, Laurent Wiard, Carine Courtillot, Marc Nicolino, Solange Grunenwald, Olivier Chabre, Sophie Christin-Maître, Rachel Desailloud, Dominique Maiter, Laurence Guignat, Aude Brac de la Perrière, P. Salva, Didier Scavarda, Fabrice Bonneville, Philippe Caron, Alexandre Vasiljevic, Delphine Leclercq, Christine Cortet, S. Gaillard, F. Albarel, Karine Clément, Emmanuel Jouanneau, Henry Dufour, Pascal Barat, Blandine Gatta‐Cherifi
Abstract
Craniopharyngiomas are rare hypothalamic-pituitary tumors found in young children, adolescents and adults, and their multidisciplinary management required, calls for consistent practices for practicioners, patients and families. The French Endocrine Society and French Society for Pediatric Endocrinology & Diabetes enlisted and coordinated adult and paediatric endocrinologists, neurosurgeons, pathologists, radiotherapists as well as psychologists, dieticians and a patient association, to draft a reference document on this severe disease. The management of craniopharyngiomas remains complex due to their aggressive nature, invasive behavior, and propensity for recurrence, requiring a sequential and measured therapeutic approach and follow-up in expert centers. Although patient survival rates are high, the consequences of both the tumor and its treatment can lead to serious comorbidities and impaired quality of life, particularly in those patients with lesional hypothalamic syndrome. Recent advances have allowed the two described tumor types - papillary and adamantinomatous - to be associated with distinct molecular signatures, specific pathophysiological mechanisms and ipso facto, distinct therapeutic approaches, including innovative medications for hyperphagia, that will continue to evolve. This consensus statement covers all stages in the management of patients with craniopharyngioma, from diagnosis to therapeutic strategies including the long-term follow-up.