Open AccessMON-296 Craniopharyngiomas Presenting as Incidentalomas: Results of Kraniopharyngeom 2007
Author(s) -
Svenja Boekhoff,
Brigitte Bison,
Maria Eveslage,
Panjarat Sowithayasakul,
Hermann L. Müller
Publication year - 2020
Publication title -
journal of the endocrine society
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.046
H-Index - 20
ISSN - 2472-1972
DOI - 10.1210/jendso/bvaa046.259
Subject(s) - medicine , craniopharyngioma , cohort , body mass index , hydrocephalus , endocrine system , surgery , hormone
Objective: Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). We investigated clinical manifestations and outcome in incCPs and symCPs. Design and methods: IncCP were discovered in 4 (3m/1f) and symCP in 214 CP (101m/113f) recruited 2007–2014 in KRANIOPHARYNGEOM 2007. Age, sex, height, body mass index (BMI), tumor size, degree of resection, pre- and postsurgical hypothalamic involvement/lesions, pituitary function and outcome were compared between both subgroups. Results: Reasons for imaging in incCP were cerebral palsy, head trauma, nasal obstruction, and tethered-cord syndrome, whereas headache (44%) visual impairment (25%), and growth retardation (17%) lead to imaging in symCP. Tumor size at diagnosis was smaller in incCP (median tumour size 3.26 cm2; range: 0.56–5.13 cm2) when compared with symCP (median tumour size 12.16 cm2; range: 0.004–79.54 cm2). Age, gender, BMI, height, hydrocephalus, tumor location, and hypothalamic involvement at diagnosis of incCP were within the range of these parameters in symCP. Complete resections were achieved more frequently (3/4 patients) in incCP when compared with symCP (20%). Surgical hypothalamic lesions were distributed similar in incCP and symCP. Irradiation was performed only in symCP (33%). No noticeable differences were observed concerning survival rates, endocrine deficiencies, BMI, height, functional capacity and quality of life of the 4 incCP cases when compared with the symCP cohort. Conclusions: IncCP are rare (1.8%) and characterized by lack of endocrine deficiencies, resulting in normal height and BMI, no hydrocephalus, and smaller tumor size at diagnosis when compared with symCPs. Outcome of the observed incCP is similar with symCP.