Advances in pediatric surgery

Korean Association of Pediatric Surgeons (대한소아외과학회)
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Undifferentiated (Embryonal) Sarcoma of the Liver in Children

소아의 미분화(태생) 간육종

  • Kim, Dae-Yeon (Department of Surgery, Seoul National University College of Medicine) ;
  • Kim, Ki-Hong (Department of Surgery, Seoul National University College of Medicine) ;
  • Jung, Sung-Eun (Department of Surgery, Seoul National University College of Medicine) ;
  • Lee, Seong-Cheol (Department of Surgery, Seoul National University College of Medicine) ;
  • Park, Kwi-Won (Department of Surgery, Seoul National University College of Medicine) ;
  • Kim, Woo-Ki (Department of Surgery, Seoul National University College of Medicine)
  • 김대연 (서울대학교 의과대학 외과학교실) ;
  • 김기홍 (서울대학교 의과대학 외과학교실) ;
  • 정성은 (서울대학교 의과대학 외과학교실) ;
  • 이성철 (서울대학교 의과대학 외과학교실) ;
  • 박귀원 (서울대학교 의과대학 외과학교실) ;
  • 김우기 (서울대학교 의과대학 외과학교실)
  • Published : 1999.06.30
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Abstract

Undifferentiated (embryonal) sarcoma is a rare malignancy of the liver in children and young adults. Seven cases of undifferentiated (embryonal) sarcoma of the liver pathologically verified at Seoul National University Children's Hospital between 1986 and 1999 were retrospectively analyzed. There were three girls and four boys, and their mean age at diagnosis was 12.1 years (range 7-13 years). Six patients presented with an abdominal mass or pain, and one with weight loss. Tumor size ranged from $8.0{\times}8.0$ cm to $15.0{\times}15.0$ cm. Four tumors were located in the right lobe, two in the left lobe and one in both. One patient died during chemotherapy. Initial complete resection was accomplished in three patients. Two patients underwent complete resection after chemotherapy. Five patients with complete resection survived without evidence of disease for 8, 11, 13, 28, and 84 months. A patient with partial resection and chemotherapy died of sepsis during chemotherapy 19 months after complete surgical resection. Adjuvant chemotherapy and radiotherapy were performed in all patients after complete surgical resection. In conclusion, though undifferntiated (embryonal) sarcoma of the liver is highly malignant, the combination therapy of surgery, chemotherapy and radiotherapy appears to result in a favorable prognosis.

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