A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome

Noonan syndrome (NS) is an autosomal dominant disorder in some cases caused by PTPN11 mutations. Since somatic mutations in PTPN11 are seen in several tumor types, NS which causes germline PTPN11 mutations are also increase the risk of hematologic malignancies and brain solid tumors. However, the re...

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Main Authors: Boonchai Boonyawat, Mongkon Charoenpitakchai, Piradee Suwanpakdee
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2019/6091059
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author Boonchai Boonyawat
Mongkon Charoenpitakchai
Piradee Suwanpakdee
author_facet Boonchai Boonyawat
Mongkon Charoenpitakchai
Piradee Suwanpakdee
author_sort Boonchai Boonyawat
collection DOAJ
description Noonan syndrome (NS) is an autosomal dominant disorder in some cases caused by PTPN11 mutations. Since somatic mutations in PTPN11 are seen in several tumor types, NS which causes germline PTPN11 mutations are also increase the risk of hematologic malignancies and brain solid tumors. However, the report of brain tumors in Noonan syndrome remains rather rare. Here, we report the first case of an 11-year-old Thai boy with Noonan syndrome who presented with symptoms related to hydrocephalus secondary to subependymoma in the fourth ventricle, and PTPN11 mutation was identified in this patient.
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series Case Reports in Neurological Medicine
spelling doaj-art-e4b94e50e5d54d699540cd4d2511d4222025-08-20T02:21:18ZengWileyCase Reports in Neurological Medicine2090-66682090-66762019-01-01201910.1155/2019/60910596091059A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan SyndromeBoonchai Boonyawat0Mongkon Charoenpitakchai1Piradee Suwanpakdee2Division of Genetics, Department of Pediatrics, Phramongkutklao Hospital and Phramongkutklao College of Medicine, Bangkok, ThailandDepartment of Pathology, Phramongkutklao Hospital and Phramongkutklao College of Medicine, Bangkok, ThailandDivision of Neurology, Department of Pediatrics, Phramongkutklao Hospital and Phramongkutklao College of Medicine, Bangkok, ThailandNoonan syndrome (NS) is an autosomal dominant disorder in some cases caused by PTPN11 mutations. Since somatic mutations in PTPN11 are seen in several tumor types, NS which causes germline PTPN11 mutations are also increase the risk of hematologic malignancies and brain solid tumors. However, the report of brain tumors in Noonan syndrome remains rather rare. Here, we report the first case of an 11-year-old Thai boy with Noonan syndrome who presented with symptoms related to hydrocephalus secondary to subependymoma in the fourth ventricle, and PTPN11 mutation was identified in this patient.http://dx.doi.org/10.1155/2019/6091059
spellingShingle Boonchai Boonyawat
Mongkon Charoenpitakchai
Piradee Suwanpakdee
A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome
Case Reports in Neurological Medicine
title A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome
title_full A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome
title_fullStr A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome
title_full_unstemmed A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome
title_short A First Case Report of Subependymoma in PTPN11 Mutation-Associated Noonan Syndrome
title_sort first case report of subependymoma in ptpn11 mutation associated noonan syndrome
url http://dx.doi.org/10.1155/2019/6091059
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