Intracranial undifferentiated malign neuroglial tumor in Smith-Lemli-Opitz syndrome: A theory of a possible predisposing factor for primary brain tumors via a case report

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• 作者：Ayfer Aslan ; Alp Ozgun Borcek ; Selma Pamukcuoglu…
• 关键词：Brain surgery ; Intracranial undifferentiated malignant tumor ; Smith ; Lemli ; Opitz syndrome ; Sonic Hedgehog signaling pathway
• 刊名：Child's Nervous System
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：33
• 期：1
• 页码：171-177
• 全文大小：
• 刊物类别：Medicine
• 刊物主题：Neurosurgery; Neurosciences;
• 出版者：Springer Berlin Heidelberg
• ISSN：1433-0350
• 卷排序：33

文摘

BackgroundSmith-Lemli-Opitz Syndrome (SLOS) is a rare hereditary autosomal recessive disorder with broken cholesterol synthesis causing by 7-dehydrocholesterol reductase deficiency. Although the clinical features and pathogenesis is well-defined, it is unknown whether there is a relationship between SLOS and neoplastic processes, especially brain neoplasms.