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‘Do no harm’: management of craniopharyngioma

Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester M27 4HA, UK¹ Department of Paediatrics, IRCCS, Giannina Gaslini, University of Genova, Genova, Italy

(Correspondence should be addressed to H Gleeson; Email: helena.gleeson{at}cmmc.nhs.uk)

This paper was presented at the 5th Ferring International Paediatric Endocrinology Symposium, Baveno, Italy (2008). Ferring Pharmaceuticals has supported the publication of these proceedings.

Craniopharyngioma management is challenging. Although histology is benign, the tumour can be clinically aggressive with local invasion and frequent recurrences. Extensive morbidity may be present at diagnosis and furthermore, occurs as a consequence of neurosurgery and radiotherapy. Hypothalamic symptoms can have a devastating effect on quality of life and may reduce life expectancy. This case highlights both the challenge of managing hyperphagia and morbid obesity and the importance of initial treatment preserving existing hypothalamic function and the need to avoid tumour recurrence and further surgery.

A 11-year old boy presented with hydrocephalus secondary to a craniopharyngioma (he had visual failure and hypopituitarism but few hypothalamic symptoms). He underwent radical resection followed by radiotherapy. Following this treatment, he developed psychological and behavioural problems and hyperphagia. Weight gain in the first year (an increase from +1.4 to +3.7 S.D.) resulted in poor mobility and a fall which caused a slipped femoral epiphysis. In the next year, there was a 6-month period of unexpected weight loss (+4.2 to +3.8 S.D.) that culminated in emergency treatment for diabetic ketoacidosis secondary to severe insulin resistance. He developed a left hemiplegia, and a subsequent cerebral angiogram identified multiple stenoses of the Circle of Willis with a Moyamoya appearance secondary to radiotherapy. Weight gain has continued (+3.8 to +5.5 S.D.) so that bariatric surgery is a management option.