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Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports

Gardner fibromas (GFs) have only recently been described as poorly circumscribed tumor-like lesions, which are exceedingly rare in children. GFs are associated with APC gene mutations and therefore with familial adenomatous polyposis (FAP). So far there is only very limited literature on GF in the n...

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Autores principales: Schäfer, Mattias, Kadmon, Martina, Schmidt, Wolfgang, Treiber, Irmgard, Moog, Ute, Sutter, Christian, Stehr, Maximilian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Georg Thieme Verlag KG 2016
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5177561/
https://www.ncbi.nlm.nih.gov/pubmed/28018803
http://dx.doi.org/10.1055/s-0036-1582443
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author Schäfer, Mattias
Kadmon, Martina
Schmidt, Wolfgang
Treiber, Irmgard
Moog, Ute
Sutter, Christian
Stehr, Maximilian
author_facet Schäfer, Mattias
Kadmon, Martina
Schmidt, Wolfgang
Treiber, Irmgard
Moog, Ute
Sutter, Christian
Stehr, Maximilian
author_sort Schäfer, Mattias
collection PubMed
description Gardner fibromas (GFs) have only recently been described as poorly circumscribed tumor-like lesions, which are exceedingly rare in children. GFs are associated with APC gene mutations and therefore with familial adenomatous polyposis (FAP). So far there is only very limited literature on GF in the neonatal period. We present two children with GF diagnosed at birth and subsequent FAP with very different clinical courses. In one case, the disease led to extensive surgery of the thoracic wall and detection of FAP in the father with the need of immediate proctocolectomy. In the other patient (with a positive family history for FAP) the disease remained stable. Our cases indicate that the diagnosis of GF in the neonatal period requires the exclusion of FAP both in the child as well as the parents. Since the clinical behavior of GF cannot be predicted, continuous monitoring is mandatory. Depending on tumor site and growth, individual therapeutic options must be thoroughly considered. Surgical resection, if necessary, has to be inevitably total to prevent recurrence.
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spelling pubmed-51775612016-12-23 Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports Schäfer, Mattias Kadmon, Martina Schmidt, Wolfgang Treiber, Irmgard Moog, Ute Sutter, Christian Stehr, Maximilian European J Pediatr Surg Rep Gardner fibromas (GFs) have only recently been described as poorly circumscribed tumor-like lesions, which are exceedingly rare in children. GFs are associated with APC gene mutations and therefore with familial adenomatous polyposis (FAP). So far there is only very limited literature on GF in the neonatal period. We present two children with GF diagnosed at birth and subsequent FAP with very different clinical courses. In one case, the disease led to extensive surgery of the thoracic wall and detection of FAP in the father with the need of immediate proctocolectomy. In the other patient (with a positive family history for FAP) the disease remained stable. Our cases indicate that the diagnosis of GF in the neonatal period requires the exclusion of FAP both in the child as well as the parents. Since the clinical behavior of GF cannot be predicted, continuous monitoring is mandatory. Depending on tumor site and growth, individual therapeutic options must be thoroughly considered. Surgical resection, if necessary, has to be inevitably total to prevent recurrence. Georg Thieme Verlag KG 2016-05-13 2016-12 /pmc/articles/PMC5177561/ /pubmed/28018803 http://dx.doi.org/10.1055/s-0036-1582443 Text en © Thieme Medical Publishers
spellingShingle Schäfer, Mattias
Kadmon, Martina
Schmidt, Wolfgang
Treiber, Irmgard
Moog, Ute
Sutter, Christian
Stehr, Maximilian
Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports
title Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports
title_full Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports
title_fullStr Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports
title_full_unstemmed Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports
title_short Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports
title_sort neonatal gardner fibroma leads to detection of familial adenomatous polyposis: two case reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5177561/
https://www.ncbi.nlm.nih.gov/pubmed/28018803
http://dx.doi.org/10.1055/s-0036-1582443
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