Kaiser Margarita, Liegl-Atzwanger Bernadette, Nagy Eszter, Sperl Daniela, Singer Georg, Till Holger
Department of Paediatric and Adolescent Surgery, Medical University of Graz, Graz, Austria.
Institute of Pathology, Medical University of Graz, Graz, Austria.
Case Rep Pediatr. 2017;2017:2969473. doi: 10.1155/2017/2969473. Epub 2017 Jun 12.
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.
先天性婴儿纤维肉瘤（CIF）是一种罕见的恶性间充质肿瘤，仅有14例伴有胃肠道表现的病例报道。我们报告了一名在妊娠34周时通过急诊剖宫产出生的女婴。出生后，她迅速出现急腹症，超声检查显示肠道穿孔，出生第一天就需要进行剖腹手术。术中发现空肠有一个2×3厘米大小的穿孔球形肿瘤结构。由于当时组织病理学结果不明且切缘不清楚，她接受了临时回肠造口术，两个月后造口关闭。组织病理学显示为先天性肠道纤维肉瘤，无特征性的ETV6-NTRK3融合转录本。总之，这种罕见肿瘤必须被视为新生儿肠道穿孔的鉴别诊断之一。
