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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

INTRODUCTION: In 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 repre...

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Autores principales: Sinichenkova, Ksenia, Yasko, Ludmila, Akhaladze, Dmitry, Petrushin, Anton, Konovalov, Dmitry, Abasov, Ruslan, Mareeva, Yulia, Melekhina, Olga, Usman, Natalia, Karachunsky, Alexander, Novichkova, Galina, Litvinov, Dmitry, Druy, Alexander
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9637945/
https://www.ncbi.nlm.nih.gov/pubmed/36353528
http://dx.doi.org/10.3389/fonc.2022.981230
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author Sinichenkova, Ksenia
Yasko, Ludmila
Akhaladze, Dmitry
Petrushin, Anton
Konovalov, Dmitry
Abasov, Ruslan
Mareeva, Yulia
Melekhina, Olga
Usman, Natalia
Karachunsky, Alexander
Novichkova, Galina
Litvinov, Dmitry
Druy, Alexander
author_facet Sinichenkova, Ksenia
Yasko, Ludmila
Akhaladze, Dmitry
Petrushin, Anton
Konovalov, Dmitry
Abasov, Ruslan
Mareeva, Yulia
Melekhina, Olga
Usman, Natalia
Karachunsky, Alexander
Novichkova, Galina
Litvinov, Dmitry
Druy, Alexander
author_sort Sinichenkova, Ksenia
collection PubMed
description INTRODUCTION: In 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 PRESENTATION: A 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. CONCLUSION: This 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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spelling pubmed-96379452022-11-08 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 Sinichenkova, Ksenia Yasko, Ludmila Akhaladze, Dmitry Petrushin, Anton Konovalov, Dmitry Abasov, Ruslan Mareeva, Yulia Melekhina, Olga Usman, Natalia Karachunsky, Alexander Novichkova, Galina Litvinov, Dmitry Druy, Alexander Front Oncol Oncology INTRODUCTION: In 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 PRESENTATION: A 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. CONCLUSION: This 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. Frontiers Media S.A. 2022-10-24 /pmc/articles/PMC9637945/ /pubmed/36353528 http://dx.doi.org/10.3389/fonc.2022.981230 Text en Copyright © 2022 Sinichenkova, Yasko, Akhaladze, Petrushin, Konovalov, Abasov, Mareeva, Melekhina, Usman, Karachunsky, Novichkova, Litvinov and Druy https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Sinichenkova, Ksenia
Yasko, Ludmila
Akhaladze, Dmitry
Petrushin, Anton
Konovalov, Dmitry
Abasov, Ruslan
Mareeva, Yulia
Melekhina, Olga
Usman, Natalia
Karachunsky, Alexander
Novichkova, Galina
Litvinov, Dmitry
Druy, Alexander
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
title 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_short 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
title_sort 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
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9637945/
https://www.ncbi.nlm.nih.gov/pubmed/36353528
http://dx.doi.org/10.3389/fonc.2022.981230
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