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...
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2023-03-01
|
| Series: | Diagnostics |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-4418/13/5/924 |
| _version_ | 1797615547582513152 |
|---|---|
| author | Chrysoula Margioula-Siarkou Stamatios Petousis Aristarchos Almperis Georgia Margioula-Siarkou Antonio Simone Laganà Maria Kourti Alexios Papanikolaou Konstantinos Dinas |
| author_facet | Chrysoula Margioula-Siarkou Stamatios Petousis Aristarchos Almperis Georgia Margioula-Siarkou Antonio Simone Laganà Maria Kourti Alexios Papanikolaou Konstantinos Dinas |
| author_sort | Chrysoula Margioula-Siarkou |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-11T07:28:05Z |
| format | Article |
| id | doaj.art-dcc468c9dc914e529e3e54d89f96f1a7 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-11T07:28:05Z |
| publishDate | 2023-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-dcc468c9dc914e529e3e54d89f96f1a72023-11-17T07:30:01ZengMDPI AGDiagnostics2075-44182023-03-0113592410.3390/diagnostics13050924Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female ChildrenChrysoula Margioula-Siarkou0Stamatios Petousis1Aristarchos Almperis2Georgia Margioula-Siarkou3Antonio Simone Laganà4Maria Kourti5Alexios Papanikolaou6Konstantinos Dinas7Gynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceGynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceGynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceGynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceUnit 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, Italy3rd Department of Pediatrics, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceGynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceGynaecologic Oncology Unit, 2nd Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Hippokration General Hospital, 54642 Thessaloniki, GreeceEmbryonal 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.https://www.mdpi.com/2075-4418/13/5/924sarcoma botryoidesfertility-sparing surgeryembryonal rhabdomyosarcomagenital tractprognosistreatment |
| spellingShingle | Chrysoula Margioula-Siarkou Stamatios Petousis Aristarchos Almperis Georgia Margioula-Siarkou Antonio Simone Laganà Maria Kourti Alexios Papanikolaou Konstantinos Dinas Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children Diagnostics sarcoma botryoides fertility-sparing surgery embryonal rhabdomyosarcoma genital tract prognosis treatment |
| 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 | sarcoma botryoides fertility-sparing surgery embryonal rhabdomyosarcoma genital tract prognosis treatment |
| url | https://www.mdpi.com/2075-4418/13/5/924 |
| work_keys_str_mv | AT chrysoulamargioulasiarkou sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT stamatiospetousis sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT aristarchosalmperis sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT georgiamargioulasiarkou sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT antoniosimonelagana sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT mariakourti sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT alexiospapanikolaou sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren AT konstantinosdinas sarcomabotryoidesoptimaltherapeuticmanagementandprognosisofanunfavorablemalignantneoplasmoffemalechildren |