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Endocrine Abstracts (2014) 36 OC3.3 | DOI: 10.1530/endoabs.36.OC3.3

1Manchester Children’s Hospital, Manchester, UK; 2Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; 3Alder Hey Children’s NHS Foundation Trust, Liverpool, UK.


Background: The optimal management of paediatric craniopharyngiomas has been debated for years. Radical surgery aimed at complete resection (CR) was the approach for several decades, with higher reported rates of tumour control compared with incomplete resection (IR). The shift towards conservative surgery with adjuvant radiotherapy (DXT), aimed at reducing post-operative morbidities, especially hypothalamic and visual, has not been systematically studied.

Aims: To review the sequelae of different management strategies in paediatric craniopharyngioma at three tertiary centres over four decades.

Methods: Meta-data from 185 patients was extracted and retrospectively reviewed from three UK tertiary centres. The analysis was undertaken over two time periods: 1973–2000 (study A) and 1998–2011 (study B).

Results: 100 patients from study A and 85 patients from study B either had CR (A – 35% and B – 19%), partial resection (PR: A – 49% and B – 46%) or limited surgery (LS, e.g. cyst decompression: A – 16% and B – 34%). CR without DXT was associated with a lower tumour recurrence rate than IR (PR+LS) with DXT in study A (OR=0.43) but in study B recurrence rates were higher (OR=1.62). 28 patients (28%) in study A received DXT (with nine recurrences) and 53 (62%) in study B (with 14 recurrences) (OR=0.56 for recurrence with DXT vs no DXT). The rates of gonadotrophin deficiency (P<0.001), diabetes insipidus (P=0.04), and panhypopituitarism (P=0.001) were lower in study B than in A. However, post-operative hypothalamic (P=0.1) and visual (P=0.3) morbidity rates remained unchanged. In fact, the proportion of patients with post-operative BMI SDS >+2.0 was higher in study B (49%) than in A (36%).

Conclusion: These metadata show that changing from CR towards more conservative surgery with DXT has improved tumour recurrence and hormone deficiencies. However, visual and hypothalamic morbidities remain a significant challenge. New approaches to managing craniopharyngioma (e.g. proton radiotherapy and in future, novel molecular pathway targeted therapies) are required to improve outcomes.

Volume 36

42nd Meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

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