Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn

Congenital infantile fibrosarcoma (CIF) is a rare malignant mesenchymal tumor and only 14 cases have been reported with gastrointestinal manifestation. We report about a female newborn delivered per emergency cesarean section at 34 weeks of gestation. Postnatally, she rapidly developed an acute abdo...

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Main Authors: Margarita Kaiser, Bernadette Liegl-Atzwanger, Eszter Nagy, Daniela Sperl, Georg Singer, Holger Till
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Pediatrics
Online Access:http://dx.doi.org/10.1155/2017/2969473
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author Margarita Kaiser
Bernadette Liegl-Atzwanger
Eszter Nagy
Daniela Sperl
Georg Singer
Holger Till
author_facet Margarita Kaiser
Bernadette Liegl-Atzwanger
Eszter Nagy
Daniela Sperl
Georg Singer
Holger Till
author_sort Margarita Kaiser
collection DOAJ
description Congenital infantile fibrosarcoma (CIF) is a rare malignant mesenchymal tumor and only 14 cases have been reported with gastrointestinal manifestation. We report about a female newborn delivered per emergency cesarean section at 34 weeks of gestation. Postnatally, she rapidly developed an acute abdomen and sonographic evidence of intestinal perforation requiring laparotomy on the first day of life. A perforated 2 × 3 cm sized spherical tumorous structure of the jejunum was identified. Due to unknown histopathology at this point and unclear resectional margins, she received a temporary ileostomy, which was closed two months later. Histopathology revealed a congenital intestinal fibrosarcoma without the characteristic ETV6-NTRK3 fusion transcript. In conclusion, this rare tumor must be considered as differential diagnosis of intestinal perforations in newborns.
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spelling doaj-art-cb3700ec5a02412fab8e90848a581d942025-02-03T06:42:21ZengWileyCase Reports in Pediatrics2090-68032090-68112017-01-01201710.1155/2017/29694732969473Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a NewbornMargarita Kaiser0Bernadette Liegl-Atzwanger1Eszter Nagy2Daniela Sperl3Georg Singer4Holger Till5Department of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, AustriaInstitute of Pathology, Medical University of Graz, Graz, AustriaDivision of Paediatric Radiology, Medical University of Graz, Graz, AustriaDivision of Pediatric Hemato-Oncology, Medical University of Graz, Graz, AustriaDepartment of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, AustriaDepartment of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, AustriaCongenital infantile fibrosarcoma (CIF) is a rare malignant mesenchymal tumor and only 14 cases have been reported with gastrointestinal manifestation. We report about a female newborn delivered per emergency cesarean section at 34 weeks of gestation. Postnatally, she rapidly developed an acute abdomen and sonographic evidence of intestinal perforation requiring laparotomy on the first day of life. A perforated 2 × 3 cm sized spherical tumorous structure of the jejunum was identified. Due to unknown histopathology at this point and unclear resectional margins, she received a temporary ileostomy, which was closed two months later. Histopathology revealed a congenital intestinal fibrosarcoma without the characteristic ETV6-NTRK3 fusion transcript. In conclusion, this rare tumor must be considered as differential diagnosis of intestinal perforations in newborns.http://dx.doi.org/10.1155/2017/2969473
spellingShingle Margarita Kaiser
Bernadette Liegl-Atzwanger
Eszter Nagy
Daniela Sperl
Georg Singer
Holger Till
Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn
Case Reports in Pediatrics
title Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn
title_full Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn
title_fullStr Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn
title_full_unstemmed Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn
title_short Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn
title_sort congenital infantile fibrosarcoma causing intestinal perforation in a newborn
url http://dx.doi.org/10.1155/2017/2969473
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