History and symptoms at primary diagnosis of childhood craniopharyngioma in 311 patients

Caniopharyngioma are connatal embryogenic midline malformations of low grade malignancy developing from remnants of Rathkes pouch. 30–50% of all cases are diagnosed during childhood and adolescence with a peak of incidence at an age of 8 years. In spite of a high overall survival rate (92%) the long-term prognosis is severely impaired by late effects of tumour and treatment. The records of 311 patients with childhood craniopharyngioma recruited in HIT-Endo and KRANIOPHARYNGEOM 2000 were evaluated in regard to clinical complaints, symptoms and history before diagnosis of craniopharyngioma.

Results: The diagnosis of craniopharyngioma was incidental without any observed symptoms and complaints in the history of 3% of all patients. First symptoms and duration of history (months) until diagnosis of craniopharyngioma were headache (52%; median duration of history 24 mo [Range: 0.5–96]), visual impairment (18%; 6 mo [1–48]), growth retardation (15%; 33 mo [12–96]), impairment of vigilance (8%; 2 mo [0.1–6]), polyuria/polydypsia as a symptom of diabetes insipidus neurohormonalis (5%; 26 mo [12–48]) and weight gain (5%; 24 mo [24–48]). The median duration of history in 311 patients was 12 months (range: 0.5–96 months). The long duration of history could be confirmed by analysis of anthropometric data collected in 90 patients before diagnosis of childhood craniopharyngioma in a nation wide health survey (Vorsorgeuntersuchungen). Already at the time point U6 (10. - 12. month of age) a significantly and persistingly impaired growth rate was found especially for patients with hypothalamic involvement of craniopharyngioma. The correlation between the duration of history and functional capacity (Fertigkeitenskala Münster-Heidelberg [FMH]) as a parameter of quality of life did not reach statistical significance.

Conclusions: A combination of the symptoms headache, visual impairment, polyuria/polydypsia and growth impairment should lead differential diagnostic efforts towards craniopharyngioma. Monitoring of growth is of importance in early diagnosis of craniopharyngioma.