Frequency of acute-onset symptoms and other features of craniopharyngioma presentation in children and adults
E H Nielsen1, J O Jørgensen2, P Bjerre2, M Andersen3, C Andersen3, U Feldt-Rasmussen4, L Poulsgaard4, L Ø Kristensen5, Jens Astrup6, Jörgen Lindholm1 & Peter Laurberg1
Introduction: Clinical symptoms in craniopharyngioma have been repeatedly described in the literature, but few studies have addressed the issue of acute-onset symptoms in children or adults. Refinements of diagnostic procedures and altered scanning routines over the last two decades may in theory have affected patterns of clinical presentation.
Objective: To study the clinical phenotype of patients with newly diagnosed craniopharyngioma, with focus on variations according to age, gender and calendar year period, and to estimate the frequency of acute-onset symptoms.
Material and methods: In a retrospective cohort of all Danish craniopharyngioma patients (n=189) diagnosed during the period 19852004, initial symptoms, clinical findings (including pituitary function) and results of neuroimaging procedures were systematically collected from medical records of each patient. Comparisons were made between subgroups based on age, gender and calendar year period, using Pearsons χ2 test or Fishers exact test, as appropriate.
Results: Acute-onset symptoms related to vision, cranial nerve function or intracranial pressure were reported in 24 patients (13%); most frequently among children, who also more often presented with hydrocephalus. Cold intolerance and depression were more frequent among adults. Frequencies of common endocrine and non-endocrine characteristics were similar among men and women.
Hypopituitarism was observed in 2656% of children and 2682% of adults, respectively, depending on the axis in question. The use of CT and MRI differed between the two periods. Apart from radiologic calcification and 3rd ventricle involvement being more frequently reported during the first half of the study period, clinical symptoms and signs did not differ between the two periods.
Conclusions: Acute-onset symptoms were seen in 13% of patients with newly diagnosed craniopharyngioma and, like hydrocephalus, more commonly among children than adults. Presenting symptoms and the extent of hypopituitarism were largely similar in men and women and across the study period.