Craniopharyngiomas in Adults : a cohort study of 151 patients
T. Cuny*a (Dr), H. Loiseaub (Prof), F. Alkhiamic (Dr), F. Castinettia (Prof), F. Albarela (Dr), T. Graillond (Dr), H. Ramarosone (Dr), I. Morangea (Dr), A. Tabarinc (Prof), T. Bruea (Prof), H. Dufourd (Prof), B. Gatta-Cherific (Prof)
a Department of Endocrinology - Aix Marseille University, Marseille, FRANCE ; b Department of Neurosurgery - University Hospital of Bordeaux, Bordeaux, FRANCE ; c Department of Endocrinology - University Hospital of Bordeaux, Bordeaux, FRANCE ; d Department of Neurosurgery - Aix Marseille University, Marseille, FRANCE ; e Department of Public Health - University Hospital of Bordeaux, Bordeaux, FRANCE
Introduction: Craniopharyngiomas (CPs) are rare brain tumours located either in the sella turcica or above the sella turcica (suprasellar). They are managed
primarily with surgery and radiotherapy, with high rate of postoperative pituitary deficiencies. We, here, describe clinical and paraclinical data of a french cohort of adult patients (age of diagnosis ≥ 18yo) diagnosed with CPs.
Methods: 151 adult patients from a large cohort of 220 patients from two French university hospital centers wer enrolled in this study.
Results: there were 86 women and 65 men, with diagnosis made in 51% of patients between 36 and 55 yo, and in 29% after 56 yo. A total of 5 patients died of CP-related events. At the diagnosis, clinical symptoms were visual disturbances (67%), headaches (30%), clinical symptoms of anterior pituitary deficiencies (17%), signs of intracranial hypertension (16%), neurocognitive impairments (14%), polyuria-polydipsia (9%) and, eventually, incidentaloma (2%). Pituitary MRI showed cystic content in 95% of tumors and, based on the degree of hypothalamic invasion, Puget's grade was 0 in 15%, 1 in 30% and 2 in 55% of cases, respectively. Grade 2 tumors were significantly associated with neurocognitive impairements at diagnosis (p = 0.05). When the pituitary stalk was unseen or resected during the surgery, a higher risk of postoperative diabetes insipidus occured (p = 0.008). Only 10(6.6%)patients did not have any pituitary deficiency at the last follow-up, including 2 non operated patients. Of note, at the last follow-up, 35% of patients had an hypothalamic obesity (BMI ≥ 30kg/m2) and 34% complained of eating disorders. Amongst these patients, 20% of them had a normal BMI (19-24), underlining the limit of this clinical parameter for truly appreciating the impact of eating disorders. Finally, 18% and 23% of patients had sleep apnea syndrome and type 2 diabetes at the last follow-up, respectively.
Discussion: Besides hormonal deficiencies, craniopharyngiomas in adults are characterized by a high prevalence of neurocognitive impairments, metabolic complications and eating disorders, that are inappropriately appreciated by only looking at the BMI.
The author has declared no conflict of interest.