Abstract #4470

A Genetically Engineered Mouse Model Recapitulates Radiological Features of Human Adamantinomatous Craniopharyngioma

Jessica KR Boult¹, Ciaran Hutchinson², John R Apps³, Gabriela Carreno³, Laura S Danielson⁴, Laura M Smith⁴, Alex K Virasami², Alexander Koers⁴, Louis Chesler⁴, Owen J Arthurs², Juan Pedro Martinez-Barbera³, and Simon P Robinson¹

¹Division of Radiotherapy and Imaging, The Institute of Cancer Research, London, United Kingdom, ²Histopathology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³Birth Defects Research Centre, Institute of Child Health, University College London, London, United Kingdom, ⁴Division of Clinical Sciences, The Institute of Cancer Research, London, United Kingdom

Tumours in Hesx1^Cre/+;Ctnnb1^lox(ex3)/+mice resemble human adamantinomatous craniopharyngioma (ACP) molecularly and histologically. MRI and ex vivo micro-CT were used to assess the radiology of this model for the first time. Early enlargement and heterogeneity of Hesx1^Cre/+;Ctnnb1^lox(ex3)/+ pituitaries was evident; enlargement of a solid tumour, and development of cysts and haemorrhage subsequently occurred. Solid components showed heterogeneous T₁-weighted signal enhancement following Gd-DTPA administration, and in some animals cysts were hyperintense on FLAIR and T₁-weighted images, both emulating clinical observations. Cyst calcification was not observed by micro-CT but we show that Hesx1^Cre/+;Ctnnb1^lox(ex3)/+ tumours faithfully recapitulate the MRI radiology of the human disease.

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