Surgical Principles for Children/Adolescents With Newly DiagnosedRhabdomyosarcoma: A Report from the Soft Tissue Sarcoma Committeeof the Children′s Oncology Group

Rodeberg, David A.; Paidas, Charles N.; Lobe, Thom L.; Brown, Kenneth L.; Andrassy, Richard J.; Crist, William M.; Wiener, Eugene S.

doi:10.1080/1357714021000066359

Cited by 34 publications

(18 citation statements)

References 72 publications

(78 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Chemotherapy is still a matter of debate and must be discussed in a dedicated multidisciplinary committee with an individual approach basis 28. For all adult sarcoma in a localized phase, R0 surgery remains the cornerstone of treatment 29, 30, 31, 32, 33, 34, 35. The present study underlines the heterogeneity of adult RMS management.…”

Section: Discussionmentioning

confidence: 74%

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

et al. 2018

View full text Add to dashboard Cite

Five‐year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients’ characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E‐RMS) for 21% of patients, alveolar (A‐RMS) for 35% of patients, and “adult‐type” P‐RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A‐RMS: 18%; E‐RMS: 17%; and P‐RMS 65%. With a median follow‐up of 8.5 years, 5‐year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E‐RMS, A‐RMS, and P‐RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A‐RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes.

show abstract

Section: Discussionmentioning

confidence: 74%

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

et al. 2018

View full text Add to dashboard Cite

show abstract

“…All these properties may cause poor prognosis, and explain the necessity for more aggressive systemic treatment in patients with parameningeal RMS (1). Surgical treatment of malignant neoplasia originating in the skull base requires great experience and collaboration of different specialists, including H&N surgeons, neurosurgeons, oral and maxillofacial surgeons, and plastic surgeons (14). Therefore, only selected medical centers offer such treatment.…”

Section: Discussionmentioning

confidence: 99%

“…At present, the surgeon is a vital member of a multidisciplinary team that plans individual treatment for every RMS patient (14). Surgical duties include assessing preoperative classification, performing proper biopsies, planning and conducting surgical resections with consideration of chemo-and radiotherapy, and evaluating postoperative classification (14). The essential aim of surgical treatment is the complete and radical resection of the primary RMS tumor with an acceptable functional and cosmetic result (27).…”

Section: Discussionmentioning

confidence: 99%

“…The necessity for radiotherapy is based on the site of the primary tumor and the completeness of surgical excision (9). At present, the surgeon is a vital member of a multidisciplinary team that plans individual treatment for every RMS patient (14). Surgical duties include assessing preoperative classification, performing proper biopsies, planning and conducting surgical resections with consideration of chemo-and radiotherapy, and evaluating postoperative classification (14).…”

Section: Discussionmentioning

confidence: 99%

“…Oncological advancements during the last decades have improved RMS treatment outcomes substantially (13). During the early 20th century, surgery was the only available therapy for RMS (13,14). Radical excision was the standard of care, and survival rates were poor, with survival being 7-70% at that time (9).…”

Section: Management Of Pediatric Head and Neck Rhabdomyosarcomamentioning

confidence: 99%

See 2 more Smart Citations

Management of pediatric head and neck rhabdomyosarcoma: A case-series of 36 patients

Radzikowska

Kukwa

et al. 2016

Oncology Letters

View full text Add to dashboard Cite

Abstract. Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the pediatric population. In 35% of cases, RMS develops in the head and neck (H&N) region, and only combined therapy is recognized as a curative treatment. However, recent advances in skull base and reconstructive surgery, along with microsurgery and endoscopic surgery, have strengthened the role of surgery as an important part of RMS treatment. In the present study, 36 pediatric RMS cases (24 males and 12 females) were analyzed after surgical treatment. The average age at diagnosis was 7 years. In total, 67% of tumors were localized in the parameningeal region. Alveolar RMS was the most common histopathological type. A total of 16 patients were treated due to disease recurrence or a previous non-radical surgical procedure, while 19 cases had inductive chemotherapy and/or radiotherapy preceding surgical treatment due to locally advanced disease. In 1 case, only diagnostic biopsy was performed. It is recommended that the management of H&N RMS is interdisciplinary from the beginning. Extensive surgical dissection in the H&N region for RMS may result in severe cosmetic defects and functional impairment; thus, these risks should be considered during treatment planning, and the surgical approach should be based on the individual characteristics of each patient.

show abstract

Rhabdomyosarcoma and related pelvic tumors of the genitourinary tract

Harel

Ferrer

2015

Pediatric Urology

View full text Add to dashboard Cite

Surgical Principles for Children/Adolescents With Newly DiagnosedRhabdomyosarcoma: A Report from the Soft Tissue Sarcoma Committeeof the Children′s Oncology Group

Cited by 34 publications

References 72 publications

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study

Management of pediatric head and neck rhabdomyosarcoma: A case-series of 36 patients

Rhabdomyosarcoma and related pelvic tumors of the genitourinary tract

Contact Info

Product

Resources

About