Journal of the Japanese Society of Pediatric Surgeons
Online ISSN : 2187-4247
Print ISSN : 0288-609X
ISSN-L : 0288-609X
Case Reports
A Neonate With Congenital Fibrosarcoma Associated With Severe Disseminated Intravascular Coagulopathy That Required Postpartum Emergency Surgery
Akira NishiHideki YamamotoFumiaki TokiNorio SuzukiTakuji KyoyaAyako SekiyaAkihito HamajimaJunko Hirato
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JOURNAL FREE ACCESS

2013 Volume 49 Issue 5 Pages 996-1002

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Abstract
We report a female neonate with a congenital fibrosarcoma (CFS) in the chest wall, which was resected immediately after birth. A 27-year-old primigravida woman was referred to our hospital at 24-week gestation because of a large tumor detected on the chest wall by antenatal ultrasound diagnosis. It was 70 mm in diameter, and protruded from the right side of the chest. The tumor had multi-lobular cysts, and a parenchyma fed by a feeding branch from a fetal intercostal vessel. Fetal magnetic resonance imaging also revealed the parenchyma contained a small mass of fatty tissues and calcification, suggesting teratoma. There was no signs of hydrops fetalis, fetal anemia, or fetal heart failure on fetal ultrasound diagnosis. At 31-week gestation, neonate weighing 2,199 g was born by elective cesarean section because the tumor was over 10 cm in diameter. During a period of few hours, the tumor grew progressively with severe anemia associated with thrombocytopenia and disseminated intravascular coagulation (DIC), and was urgently resected 7 hours after birth. Although spillage of the content occurred intraoperatively, the tumor was grossly removed. The tumor resected was 373 g in weight, and was well encapsulated. Histopathologic studies showed the operated site indicated no margin, suggesting complete resection of the tumor, although massive bleeding into the multiple cysts was observed. CFS was then diagnosed pathologically. Her postoperative course was uneventful. She is now 12 months old and shows no signs of recurrence that required adjuvant chemotherapy.
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© 2013 The Japanese Society of Pediatric Surgeons

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https://creativecommons.org/licenses/by-nc-sa/4.0/deed.ja
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