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Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma (Hardcover, 1st ed. 2017)
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Basic Research and Clinical Aspects of Adamantinomatous Craniopharyngioma (Hardcover, 1st ed. 2017)
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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 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.
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