Prognostic Impact of Hypothalamic Perforation in Adult Patients With Craniopharyngioma: A Cohort Study

Author:

Gaillard Stephan1,Benichi Sandro2,Villa Chiara34,Jouinot Anne3,Vatier Camille56ORCID,Christin-Maitre Sophie57,Raffin-Sanson Marie-Laure89,Jacob Julian10,Chanson Philippe1112ORCID,Courtillot Carine13,Bachelot Anne13,Bertherat Jérôme314,Assié Guillaume314ORCID,Baussart Bertrand13ORCID

Affiliation:

1. Department of Neurosurgery, Assistance Publique-Hôpitaux de Paris, La Pitié-Salpêtrière University Hospital , 75013 Paris , France

2. Department of Neurosurgery, Assistance Publique-Hôpitaux de Paris, Necker University Hospital , 75015 Paris , France

3. CNRS, INSERM, Institut Cochin, Université Paris Cité , 75014 Paris , France

4. Department of Neuropathology, Assistance Publique-Hôpitaux de Paris, La Pitié-Salpêtrière University Hospital , 75013 Paris , France

5. Endocrine Unit, Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement (CRMERC), Endo-ERN (ID 739527), Saint-Antoine Hospital, Sorbonne University, AP-HP , 75012 Paris , France

6. INSERM UMRS938, Saint-Antoine Research Center, Sorbonne University , 75012 Paris , France

7. INSERM UMR-833, Trousseau Hospital , 75012 Paris , France

8. Department of Endocrinology, Assistance Publique-Hôpitaux de Paris, Hôpital Ambroise Paré , 92100 Boulogne Billancourt , France

9. Université de Versailles Saint-Quentin-en-Yvelines UFR des Sciences de la Santé Simone Veil , 78180 Montigny-le-Bretonneux , France

10. Radiation Oncology Department, Assistance Publique-Hôpitaux de Paris, La Pitié-Salpêtrière University Hospital, Sorbonne University , 75013 Paris , France

11. Inserm, Physiologie et Physiopathologie Endocriniennes, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Service d'Endocrinologie et des Maladies de la Reproduction, Centre de Référence des Maladies Rares de l'Hypophyse, Université Paris-Saclay , 94270 Le Kremlin-Bicêtre , France

12. Faculté de Médecine, Inserm UMR_1185, Physiologie et Physiopathologie Endocriniennes, Université Paris-Saclay , 94270 Le Kremlin-Bicêtre , France

13. Department of Endocrinology and Reproductive Medicine, Centre de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement, CRMERC, Endo-ERN, Pitié-Salpêtrière Hospital, Sorbonne University, AP-HP , 75013 Paris , France

14. Department of Endocrinology, Center for Rare Adrenal Diseases, Assistance Publique-Hôpitaux de Paris, Hôpital Cochin , 75014 Paris , France

Abstract

Abstract Context Outcome of craniopharyngioma is related to its locoregional extension, which impacts resectability and the risk of surgical complications. To maximize resection and minimize complications, optic tract localization, temporal lobe extension, and hypothalamic involvement are essential factors for surgical management. Objective To assess the outcome of craniopharyngiomas depending on their relation to the hypothalamus location. Methods We conducted a retrospective analysis of 79 patients with a craniopharyngioma who underwent surgery from 2007 to 2022. Craniopharyngiomas were classified in 3 groups, depending on the type of hypothalamus involvement assessed by preoperative magnetic resonance imaging: infra-hypothalamic (type A, n = 33); perforating the hypothalamus (type B, n = 40); and supra-hypothalamic (type C, n = 6). Surgical strategy was guided by the type of hypothalamic involvement, favoring endonasal approaches for type A and type B, and transcranial approaches for type C. Results Long-term disease control was achieved in 33/33 (100%), 37/40 (92%), and 5/6 (83%) patients in type A, B, and C, respectively. In type B, vision was improved in 32/36 (89%) patients, while hypothalamic function was improved, stable, or worsened in 6/40 (15%), 32/40 (80%), and 2/40 (5%) patients, respectively. Papillary craniopharyngiomas were found in 5/33 (15%), 9/40 (22%), and 3/6 (50%) patients in types A, B, and C, respectively. In 4 patients, BRAF/MEK inhibitors were used, with significant tumor shrinkage in all cases. Conclusion Craniopharyngiomas located below the hypothalamus or perforating it can be safely treated by transsphenoidal surgery. For supra-hypothalamic craniopharyngiomas, postoperative results are less favorable, and documenting a BRAF mutation may improve outcome, if targeted therapy was efficient enough to replace surgical debulking.

Publisher

The Endocrine Society

Subject

Biochemistry (medical),Clinical Biochemistry,Endocrinology,Biochemistry,Endocrinology, Diabetes and Metabolism

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