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患有结节性硬化症复合体的儿童中的多形性胶质母细胞瘤。

Glioblastoma multiforme in a child with tuberous sclerosis complex.

作者信息

Vignoli Aglaia, Lesma Elena, Alfano Rosa Maria, Peron Angela, Scornavacca Giulia Federica, Massimino Maura, Schiavello Elisabetta, Ancona Silvia, Cerati Michele, Bulfamante Gaetano, Gorio Alfredo, Canevini Maria Paola

机构信息

Child Neurology Unit - Epilepsy Center, Department of Health Science, University of Milan, San Paolo Hospital, Milano, Italy.

Laboratories of Pharmacology, Department of Health Science, University of Milan, Milano, Italy.

出版信息

Am J Med Genet A. 2015 Oct;167A(10):2388-93. doi: 10.1002/ajmg.a.37158. Epub 2015 May 6.

Abstract

Tuberous Sclerosis Complex (TSC) is characterized by the presence of benign tumors in the brain, kidneys, heart, eyes, lungs, and skin. The typical brain lesions are cortical tubers, subependimal nodules and subependymal giant-cell astrocytomas. The occurrence of malignant astrocytomas such as glioblastoma is rare. We report on a child with a clinical diagnosis of TSC and a rapidly evolving glioblastoma multiforme. Genetic analysis identified a de novo mutation in TSC2. Molecular characterization of the tumor was performed and discussed, as well as a review of the literature where cases of TSC and glioblastoma multiforme are described. Although the co-occurrence of TSC and glioblastoma multiforme seems to be rare, this possible association should be kept in mind, and proper clinical and radiological follow up should be recommended in these patients.

摘要

结节性硬化症(TSC)的特征是在脑、肾、心脏、眼睛、肺和皮肤中出现良性肿瘤。典型的脑部病变是皮质结节、室管膜下结节和室管膜下巨细胞星形细胞瘤。胶质母细胞瘤等恶性星形细胞瘤的发生较为罕见。我们报告了一名临床诊断为TSC且迅速进展为多形性胶质母细胞瘤的儿童。基因分析确定TSC2存在新发突变。对肿瘤进行了分子特征分析并进行了讨论,同时回顾了描述TSC和多形性胶质母细胞瘤病例的文献。尽管TSC和多形性胶质母细胞瘤同时出现似乎很罕见,但应牢记这种可能的关联,并建议对这些患者进行适当的临床和放射学随访。

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