Frontiers in Oncology (Oct 2022)

Case report: First case of undifferentiated embryonal sarcoma of the liver in a child with neurofibromatosis type 1, treated by hepatic chemoperfusion with transcatheter arterial chemoembolization

  • Ksenia Sinichenkova,
  • Ludmila Yasko,
  • Dmitry Akhaladze,
  • Anton Petrushin,
  • Dmitry Konovalov,
  • Ruslan Abasov,
  • Yulia Mareeva,
  • Olga Melekhina,
  • Natalia Usman,
  • Alexander Karachunsky,
  • Galina Novichkova,
  • Dmitry Litvinov,
  • Alexander Druy,
  • Alexander Druy

DOI
https://doi.org/10.3389/fonc.2022.981230
Journal volume & issue
Vol. 12

Abstract

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IntroductionIn this report we firstly describe undifferentiated embryonal sarcoma of the liver (UESL) in a patient with neurofibromatosis type 1 (NF1), fatally complicated by synchronous malignant peripheral nerve sheath tumor (MPNST) with a highly aggressive metastatic course. The case also represents our first experience of chemoperfusion involving the transcatheter arterial chemoembolization (TACE) in a pediatric patient, applied as a treatment for UESL.Case presentationA 13-year-old girl was diagnosed with NF1 and presented with a liver tumor identified as UESL by histological assessment. The tumor was refractive to the conventional first-line chemotherapy. The patient received hepatic chemoperfusion with TACE, which was efficacious; however, the overall curative outcome was unsatisfactory due to synchronous unresectable retroperitoneal MPNST with mesenteric metastases and ultimate progression of the UESL.ConclusionThis is the first reported case of UESL in a patient with NF1. The results demonstrate the efficacy of hepatic chemoperfusion with TACE in pediatric UESL.

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