Management of craniopharyngioma: the Liverpool experience following the introduction of the CCLG guidelines. Introducing a new risk assessment grading system

Abstract

The Children's Cancer Leukaemia Group (CCLG) proposed a management pathway for craniopharyngioma that advocated limited surgery followed by upfront radiotherapy (RT) for large tumours with hypothalamic involvement and a radical resection only for smaller tumours without hypothalamic involvement. This strategy is not proven to provide optimum care or to be risk-free. The aim of this study is to review our experience of the management of craniopharyngioma diagnosed since the introduction of the CCLG guidelines in 2005. All children diagnosed with craniopharyngioma at Alder Hey Children's Hospital NHS Foundation Trust in the period between 1 January 2005 and 30 June 2011 were included. Management was based on the presence of hypothalamic syndrome, hydrocephalus, tumour size and radiological Paris grading system. Endoscopic drainage of tumour cyst was performed prior to formalising risk grade and surgical strategy. Definitive surgery was performed in 4-6 weeks time. In this respect, we developed a grading criteria. Twenty patients were included. Ten of the children underwent endoscopic cyst drainage prior to definitive surgery. The results of the subsequent surgical excision were complete resection, near total resection or subtotal resection in 30, 25 and 45 % patients, respectively. There was no surgical-related mortality and no new neurological deficits. Nine patients underwent RT at some stage. In this study, we tried to develop an advanced model for the management of craniopharyngioma with a new risk grading system. This may have a direct impact on the surgical strategy and outcome and could be able to improve morbidity.