Journal Article

DKFZ-2019-00900

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Occurrence of high-grade glioma in Noonan syndrome: Report of two cases.

El-Ayadi, M. ; Ansari, M. ; Kühnöl, C. D. ; Bendel, A. ; Sturm, D.DKFZ* ; Pietsch, T. ; Kramm, C. M. ; von Bueren, A. O.

2019
Wiley New York, NY

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Please use a persistent id in citations: doi:10.1002/pbc.27625

Abstract: Noonan syndrome (NS) is an autosomal dominant disorder commonly caused by PTPN11 germline mutations. Patients are characterized by short stature, congenital heart defects, facial dysmorphism, and increased risk of malignancies including brain tumors. Commonly associated brain tumors are dysembryoplastic neuroepithelial tumor and low-grade glioma. We report two cases of anaplastic astrocytoma with PTPN11-related NS. We conducted a systematic search of medical databases looking for other reported cases of high-grade glioma associated with NS and identified 24 cases of brain tumors, all of which were low-grade glial or glioneuronal tumors except for one case of medulloblastoma.

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Contributing Institute(s):

1. Pädiatrische Neuroonkologie (B062)

Research Program(s):

1. 312 - Functional and structural genomics (POF3-312) (POF3-312)

Appears in the scientific report 2019

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Database coverage:
; BIOSIS Previews ; Clarivate Analytics Master Journal List ; Current Contents - Clinical Medicine ; IF < 5 ; JCR ; NCBI Molecular Biology Database ; SCOPUS ; Science Citation Index ; Science Citation Index Expanded ; Web of Science Core Collection

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