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

Autor: 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
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: Frontiers in Oncology, Vol 12 (2022)
Druh dokumentu: article
ISSN: 2234-943X
DOI: 10.3389/fonc.2022.981230
Popis: 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.
Databáze: Directory of Open Access Journals