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Title Basic research and clinical aspects of adamantinomatous craniopharyngioma / Juan Pedro Martinez-Barbera, Cynthia Lilian Andoniadou, editors
Published Cham, Switzerland : Springer, 2017
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Description 1 online resource (xii, 220 pages) : illustrations (some color)
Contents 880-01 Pathogenesis of human ACP -- Transcriptomic and Genomic Analyses of Human Craniopharyngioma -- Genetically Modified Mouse Models of Adamantinomatous Craniopharyngioma -- Clinical Diagnosis of Human ACP -- Endocrine Deficits in Patients with Human Craniopharyngioma -- Obesity and metabolic disturbances in Adamantinomatous Craniopharyngioma Patients -- Radiology and Radiotherapy of Craniopharyngioma -- Surgical Treatment of Human ACP -- Intracystic Administration of Interferon-Alpha for Reduction of Cystic Tumour Burden -- Long-term Management and Clinical Trials in Adamantinomatous Craniopharyngioma
880-01 Genetically Modified Mouse Models of Adamantinomatous Craniopharyngioma Normal Development of the Pituitary Gland; Pathology of Human ACP; Over-Activation of the WNT/β-Catenin Pathway Underlies the Molecular Aetiology of Human and Mouse ACP; Childhood-Onset ACP is Likely to be a Tumour of Embryonic Origin; Pituitary Stem Cells are Present in Mouse and Human Pituitaries; Contribution of Stem Cells in the Long-Term Maintenance of the Anterior Pituitary; Paracrine Contribution of Stem Cells to Tumourigenesis; Conclusions; References; Clinical Diagnosis of Human ACP; Introduction
Summary 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 underlie the 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
Notes Includes index
Online resource; title from PDF title page (SpringerLink, viewed April 27, 2017)
Subject Brain -- Tumors.
Cancer in children.
Skull -- Tumors.
Brain Neoplasms.
Form Electronic book
Author Andoniadou, Cynthia Lilian, editor
Martinez-Barbera, Juan Pedro, editor
ISBN 3319518909 (electronic bk.)
9783319518909 (electronic bk.)
(print)