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Review

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

by
Chrysoula Margioula-Siarkou
1,
Stamatios Petousis
1,*,
Aristarchos Almperis
1,
Georgia Margioula-Siarkou
1,
Antonio Simone Laganà
2,
Maria Kourti
3,
Alexios Papanikolaou
1 and
Konstantinos Dinas
1
1
Gynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, Greece
2
Unit of Gynecologic Oncology, ARNAS “Civico–Di Cristina–Benfratelli”, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, 90127 Palermo, Italy
3
3rd Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, Greece
*
Author to whom correspondence should be addressed.
Diagnostics 2023, 13(5), 924; https://doi.org/10.3390/diagnostics13050924
Submission received: 22 December 2022 / Revised: 5 February 2023 / Accepted: 14 February 2023 / Published: 1 March 2023
(This article belongs to the Special Issue Prognostic Factors for Pediatric Tumors)
Review Reports Versions Notes

Abstract

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.
Keywords: sarcoma botryoides; fertility-sparing surgery; embryonal rhabdomyosarcoma; genital tract; prognosis; treatment; local debulking; neoadjuvant chemotherapy; radiation sarcoma botryoides; fertility-sparing surgery; embryonal rhabdomyosarcoma; genital tract; prognosis; treatment; local debulking; neoadjuvant chemotherapy; radiation

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MDPI and ACS Style

Margioula-Siarkou, C.; Petousis, S.; Almperis, A.; Margioula-Siarkou, G.; Laganà, A.S.; Kourti, M.; Papanikolaou, A.; Dinas, K. Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children. Diagnostics 2023, 13, 924. https://doi.org/10.3390/diagnostics13050924

AMA Style

Margioula-Siarkou C, Petousis S, Almperis A, Margioula-Siarkou G, Laganà AS, Kourti M, Papanikolaou A, Dinas K. Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children. Diagnostics. 2023; 13(5):924. https://doi.org/10.3390/diagnostics13050924

Chicago/Turabian Style

Margioula-Siarkou, Chrysoula, Stamatios Petousis, Aristarchos Almperis, Georgia Margioula-Siarkou, Antonio Simone Laganà, Maria Kourti, Alexios Papanikolaou, and Konstantinos Dinas. 2023. "Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children" Diagnostics 13, no. 5: 924. https://doi.org/10.3390/diagnostics13050924

APA Style

Margioula-Siarkou, C., Petousis, S., Almperis, A., Margioula-Siarkou, G., Laganà, A. S., Kourti, M., Papanikolaou, A., & Dinas, K. (2023). Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children. Diagnostics, 13(5), 924. https://doi.org/10.3390/diagnostics13050924

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