title: Neonatal Gardner Fibroma Leads to Detection of Familial Adenomatous Polyposis: Two Case Reports.
abstract: Gardner fibromas (GFs) have only recently been described as poorly circumscribed tumor-like lesions, which are exceedingly rare in children. GFs are associated withAPCgene 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.
authors.affiliation: Department of Pediatric Surgery and Urology, Cnopfsche Kinderklinik, Nürnberg, Germany.
authors.forename: Mattias
authors.initials: M
authors.lastname: Schäfer
authors.affiliation: Medicine and Health Sciences, Carl von Ossietzky Universität Oldenburg, Oldenburg, Germany.
authors.forename: Martina
authors.initials: M
authors.lastname: Kadmon
authors.affiliation: Department of Pediatrics, Section Pediatric Oncology, Cnopfsche Kinderklinik, Nürnberg, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schmidt
authors.affiliation: Department of General, Visceral, and Transplant Surgery, Universitätsklinikum Heidelberg, Germany.
authors.forename: Irmgard
authors.initials: I
authors.lastname: Treiber
authors.affiliation: Institute of Human Genetics, Universitätsklinikum Heidelberg, Germany.
authors.forename: Ute
authors.initials: U
authors.lastname: Moog
authors.affiliation: Institute of Human Genetics, Universitätsklinikum Heidelberg, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Sutter
authors.affiliation: Department of Pediatric Surgery and Urology, Cnopfsche Kinderklinik, Nürnberg, Germany.
authors.forename: Maximilian
authors.initials: M
authors.lastname: Stehr
keywords: FAP; Gardner fibroma; familial adenomatous polyposis; neonatology; premalignant lesion