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Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children

Embryonal rhabdomyosarcoma (ERMS) is a rare malignancy and occurs primarily in the first two decades of life. Botryoid rhabdomyosarcoma is an aggressive subtype of ERMS that often manifests in the genital tract of female infants and children. Due to its rarity, the optimal treatment approach has bee...

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Autores principales: Margioula-Siarkou, Chrysoula, Petousis, Stamatios, Almperis, Aristarchos, Margioula-Siarkou, Georgia, Laganà, Antonio Simone, Kourti, Maria, Papanikolaou, Alexios, Dinas, Konstantinos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10000398/
https://www.ncbi.nlm.nih.gov/pubmed/36900067
http://dx.doi.org/10.3390/diagnostics13050924
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author Margioula-Siarkou, Chrysoula
Petousis, Stamatios
Almperis, Aristarchos
Margioula-Siarkou, Georgia
Laganà, Antonio Simone
Kourti, Maria
Papanikolaou, Alexios
Dinas, Konstantinos
author_facet Margioula-Siarkou, Chrysoula
Petousis, Stamatios
Almperis, Aristarchos
Margioula-Siarkou, Georgia
Laganà, Antonio Simone
Kourti, Maria
Papanikolaou, Alexios
Dinas, Konstantinos
author_sort Margioula-Siarkou, Chrysoula
collection PubMed
description Embryonal rhabdomyosarcoma (ERMS) is a rare malignancy and occurs primarily in the first two decades of life. Botryoid rhabdomyosarcoma is an aggressive subtype of ERMS that often manifests in the genital tract of female infants and children. Due to its rarity, the optimal treatment approach has been a matter of debate. We conducted a search in the PubMed database and supplemented it with a manual search to retrieve additional papers eligible for inclusion. We retrieved 13 case reports and case series, from which we summarized that the current trend is to approach each patient with a personalized treatment plan. This consists of a combination of local debulking surgery and adjuvant or neoadjuvant chemotherapy (NACT). Effort is made in every approach to avoid radiation for the sake of preserving fertility. Radical surgeries and radiation still have a role to play in extensive disease and in cases of relapse. Despite the rarity and aggressiveness of this tumor, disease-free survival and overall prognosis is excellent, especially when it is diagnosed early, compared with other subtypes of rhabdomyosarcoma (RMS). We conclude that the practice of a multidisciplinary approach is appropriate, with favorable outcomes; however, larger-scale studies need to be organized to have a definite consensus on optimal management.
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spelling pubmed-100003982023-03-11 Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children Margioula-Siarkou, Chrysoula Petousis, Stamatios Almperis, Aristarchos Margioula-Siarkou, Georgia Laganà, Antonio Simone Kourti, Maria Papanikolaou, Alexios Dinas, Konstantinos Diagnostics (Basel) Review Embryonal rhabdomyosarcoma (ERMS) is a rare malignancy and occurs primarily in the first two decades of life. Botryoid rhabdomyosarcoma is an aggressive subtype of ERMS that often manifests in the genital tract of female infants and children. Due to its rarity, the optimal treatment approach has been a matter of debate. We conducted a search in the PubMed database and supplemented it with a manual search to retrieve additional papers eligible for inclusion. We retrieved 13 case reports and case series, from which we summarized that the current trend is to approach each patient with a personalized treatment plan. This consists of a combination of local debulking surgery and adjuvant or neoadjuvant chemotherapy (NACT). Effort is made in every approach to avoid radiation for the sake of preserving fertility. Radical surgeries and radiation still have a role to play in extensive disease and in cases of relapse. Despite the rarity and aggressiveness of this tumor, disease-free survival and overall prognosis is excellent, especially when it is diagnosed early, compared with other subtypes of rhabdomyosarcoma (RMS). We conclude that the practice of a multidisciplinary approach is appropriate, with favorable outcomes; however, larger-scale studies need to be organized to have a definite consensus on optimal management. MDPI 2023-03-01 /pmc/articles/PMC10000398/ /pubmed/36900067 http://dx.doi.org/10.3390/diagnostics13050924 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Margioula-Siarkou, Chrysoula
Petousis, Stamatios
Almperis, Aristarchos
Margioula-Siarkou, Georgia
Laganà, Antonio Simone
Kourti, Maria
Papanikolaou, Alexios
Dinas, Konstantinos
Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children
title Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children
title_full Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children
title_fullStr Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children
title_full_unstemmed Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children
title_short Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children
title_sort sarcoma botryoides: optimal therapeutic management and prognosis of an unfavorable malignant neoplasm of female children
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10000398/
https://www.ncbi.nlm.nih.gov/pubmed/36900067
http://dx.doi.org/10.3390/diagnostics13050924
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