A Neonate With Congenital Fibrosarcoma Associated With Severe Disseminated Intravascular Coagulopathy That Required Postpartum Emergency Surgery

DOI Web Site 19 References
  • Nishi Akira
    Department of Surgery, Gunma Children's Medical Center
  • Yamamoto Hideki
    Department of Surgery, Gunma Children's Medical Center
  • Toki Fumiaki
    Department of Surgery, Gunma Children's Medical Center
  • Suzuki Norio
    Department of Surgery, Gunma Children's Medical Center
  • Kyoya Takuji
    Department of Obstetrics, Gunma Children's Medical Center
  • Sekiya Ayako
    Department of Neonatology, Gunma Children's Medical Center
  • Hamajima Akihito
    Department of Plastic and Reconstractive Surgery, Gunma Children's Medical Center
  • Hirato Junko
    Department of Pathology, Graduate School of Medicine, Gunma University

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Other Title
  • 出生後に緊急手術を要した巨大な先天性線維肉腫の1例
  • 症例報告 出生後に緊急手術を要した巨大な先天性線維肉腫の1例
  • ショウレイ ホウコク シュッショウゴ ニ キンキュウ シュジュツ オ ヨウシタ キョダイ ナ センテンセイ センイ ニクシュ ノ 1レイ

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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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