Journal of Pediatric Surgery Case Reports (Mar 2021)

Multi-disciplinary treatment of undifferentiated embryonal sarcoma of the liver with tumor embolus extending to the heart

  • Kunio Fukuda,
  • Ai Yoshimi,
  • Miki Toma,
  • Satoru Matsushima,
  • Norihiko Kitagawa,
  • Masato Shinkai,
  • Noritaka Ota,
  • Toshihide Asou,
  • Mio Tanaka,
  • Yukichi Tanaka,
  • Chie Kobayashi,
  • Takayuki Masuko,
  • Asai Nobuyoshi,
  • Keisuke Kato,
  • Junko Shiono,
  • Haruo Ohtani,
  • Kazutoshi Koike,
  • Tatsuo Kono,
  • Toshihiro Yanai,
  • Masahiro Tsuchida

DOI
https://doi.org/10.1016/j.epsc.2020.101705
Journal volume & issue
Vol. 66
p. 101705

Abstract

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Undifferentiated embryonal sarcoma of the liver (UESL) is a highly malignant neoplasm occurring predominantly in children. Complete resection is the only curative treatment. We encountered an 11-year-old boy with UESL arising in the right lobe of the liver extending to the pulmonary valve. He was treated with IREC (irinotecan, etoposide, and carboplatin) neoadjuvant chemotherapy, concurrent extended radical right hepatectomy, and intracardiac tumorectomy under cardiopulmonary bypass, and adjuvant IREC chemotherapy. He remains in remission without complications 48 months after diagnosis. Multidisciplinary treatment, particularly the combination of radical surgery and effective neoadjuvant chemotherapy, may contribute to UESL patients' survival.

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