| Literature DB >> 25946256 |
Aglaia Vignoli1, Elena Lesma2, Rosa Maria Alfano3, Angela Peron1, Giulia Federica Scornavacca1, Maura Massimino4, Elisabetta Schiavello4, Silvia Ancona2, Michele Cerati5, Gaetano Bulfamante3, Alfredo Gorio2, Maria Paola Canevini1.
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.Entities:
Keywords: Glioblastoma multiforme; TSC; Tuberous Sclerosis Complex; children; follow-up MR; tumors
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Year: 2015 PMID: 25946256 DOI: 10.1002/ajmg.a.37158
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802