Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma

1. Front Matter

   Pages i-xii

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2. Pathogenesis of Human ACP

   • Annett Hölsken

   Pages 1-26

3. Transcriptomic and Genomic Analyses of Human Craniopharyngioma

   • Leslie Robinson, Sandro Santagata, Todd C. Hankinson

   Pages 27-39

4. Genetically Modified Mouse Models of Adamantinomatous Craniopharyngioma

   • Cynthia Lilian Andoniadou, Juan Pedro Martinez-Barbera

   Pages 41-55

5. Clinical Diagnosis of Human ACP

   • Ashley J. Vardon, Niki Karavitaki

   Pages 57-66

6. Endocrine Deficits in Patients with Human Craniopharyngioma

   • Hoong-Wei Gan, Helen A. Spoudeas, Mehul T. Dattani

   Pages 67-83

7. Obesity and Metabolic Disturbances in Adamantinomatous Craniopharyngioma Patients

   • Eva Marie Erfurth

   Pages 85-99

8. Radiology and Radiotherapy of Craniopharyngioma

   • Babita Jyoti, Daniel J. Indelicato, Julie A. Bradley, Ronny L. Rotondo

   Pages 101-135

9. Surgical Treatment of Human ACP

   • Kristian Aquilina, Michael Buchfelder

   Pages 137-158

10. Intracystic Administration of Interferon-Alpha for Reduction of Cystic Tumour Burden

    • John-Paul Kilday, Ute Bartels

    Pages 159-177

11. Long-Term Management and Clinical Trials in Adamantinomatous Craniopharyngioma

    • Hermann L. Müller

    Pages 179-214

12. Back Matter

    Pages 215-220

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This astute volume brings together the latest expert research on adamantinomatous craniopharyngiomas (ACPs). ACPs are histologically benign but clinically aggressive tumors exhibiting a high propensity for local invasion into the hypothalamus, optic and vascular structures. These tumors, as well as the current treatments, may result in pan-hypopituitarism, diabetes insipidus, morbid obesity followed by type II diabetes mellitus, blindness, as well as serious behavioral and psychosocial impairments. Exploring in detail advances in both the understanding of tumor biology as well as clinical advances in patient management are explored in detail, this book will also look towards potential new treatment approaches.


Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma is the first book compiling all current research on ACPs. Mouse and human studies have unequivocally demonstrated that mutations in CTNNB1 encoding -catenin underliethe etiology of the majority, if not all ACP tumors. Genetic studies in mice have shown that ACPs are tumors of the pituitary gland and not of the hypothalamus as previously thought, and are derived from Rathke’s pouch precursors. In addition, a role for tissue-specific adult pituitary stem cells has been revealed as causative of ACP. Together, these studies have provided novel insights into the molecular and cellular etiology as well as the pathogenesis of human ACP. Finally, this volume covers new treatment approaches that have been shown to be effective both in reducing ACP burden as well as reducing the morbidity associated with therapy.      

• Stem cell
• adult pituitary stem cells
• pituitary development
• radiology
• tumor biology

• Developmental Biology and Cancer Programme, Birth Defects Research Centre, Institute of Child Health, University College London, London, United Kingdom

  Juan Pedro Martinez-Barbera

• Craniofacial Development and Stem Cell Biology, King’s College London, London, United Kingdom

  Cynthia Lilian Andoniadou

Professor Juan Pedro Martínez-Barberá is a leading expert in pituitary development and associated congenital disorders. He is a researcher at the Birth Defects Research Centre, Developmental Biology and Cancer Programme of the UCL Great Ormond Street Institute of Child Health.
Dr. Cynthia Lilian Andoniadou is a researcher at the Department of Craniofacial Development and Stem Cell Biology of King’s College London focusing on the biology of normal and pathogenic pituitary stem cells.

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