Management and outcome of 239 adolescent and adult rhabdomyosarcoma patients

Dumont, Sarah; Araujo, Dejka M.; Munsell, Mark F.; Salganick, Jason A.; Agathe, Dumont; Raymond, Kevin; Linassier, Claude; Patel, Shreyaskumar; Benjamin, Robert S.; Trent, Jonathan C.

doi:10.1002/cam4.92

Cited by 51 publications

(64 citation statements)

References 31 publications

(32 reference statements)

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“…But in our study, patients older than 25 years received significantly less chemotherapy and numerous treatments. Surgical results were consistent with those previously reported 11, 12, 13, 15, 16, 17, 18, 19. The absence of surgery and incomplete resection were deleterious for OS.…”

Section: Discussionsupporting

confidence: 90%

“…The relapse rate is consistent with that reported in the literature: 33–57% local relapses 11, 12, 13, 14, 19 and up to 48–68% metastatic relapse 14, 16, 19. It is observed often during the first year, leading to death in 6–12 months 11, 12, 14, 15, 16, 17, 19, with median survival of 9, 8, and 7 months for patients with local relapse, metastatic relapse, or both in the present work. This outcome is notably inferior to that achieved in other ASTS of adults in 2016.…”

Section: Discussionsupporting

confidence: 90%

“…These results are consistent with those described in the literature. RMS occurs frequently in men and young adults: 37 versus 26 years in other studies and increased by twofold in P‐RMS 11, 12, 13, 14, 15, 16, 17. HN and extremities tumors represent the main primary site in this study and previous reports 11, 12, 16, 17.…”

Section: Discussionsupporting

confidence: 72%

“…For localized RMS, OS is similar to that reported by the MDA and MSKCC experience 14, 15. Importantly, the outcome of patients initially treated in the reference center in the retrospective cohort (Conticabase series) is superior to that of the nationwide and more recent series, possibly explaining the excellence of the reference center compared with other centers 18.…”

Section: Discussionsupporting

confidence: 71%

“…HN and extremities tumors represent the main primary site in this study and previous reports 11, 12, 16, 17. Synchronous metastases were observed in 20% versus 17–44% in previous studies 11, 13, 14, 15, 16, 17. LN involvement was uncommon (14%) compared with other series (33–46%) 11, 16, 17.…”

Section: Discussionsupporting

confidence: 50%

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

et al. 2018

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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.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 72%

Section: Discussionsupporting

confidence: 71%

Section: Discussionsupporting

confidence: 50%

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

et al. 2018

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Soft Tissue Sarcomas

Maki

Raut

O’Sullivan

2017

Holland‐Frei Cancer Medicine

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Overview The management of soft tissue sarcomas is driven by the anatomic site and histology of the primary and is increasingly affected by the specific genetics of the sarcoma. In this chapter, we focus on the principles of management of this group of over 50 cancer subtypes to highlight commonalities and differences from anatomical constraints of surgery to specifics of adjuvant radiation to identification of systemic therapeutics that are appropriate for each histology. We will discuss in this chapter the etiology, presentation, diagnosis, staging, and multidisciplinary management of patients with sarcomas of soft tissue. Surgery remains paramount to achieve cure for the vast majority of sarcomas. Radiation therapy is used for larger tumors in the appropriate clinical context. The evolution of increasingly sophisticated radiation techniques is highlighted in this chapter. As pertains to systemic therapy, this chapter is written at a time in which first‐line therapies for soft tissue sarcomas may change, raising anew some questions of adjuvant therapy that remain incompletely answered. Where appropriate, we attempt to link specific histologies or molecular changes to therapeutic suggestions, with the understanding and hope that novel agents will supplant the medications that are available but that have not materially affected outcomes for few diagnoses other than GIST in the past several years.

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Soft Tissue Sarcomas

Thornton¹,

Baldini²,

Maki³

et al. 2022

Holland‐Frei Cancer Medicine

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Overview The management of soft tissue sarcomas is driven by the anatomic site as well as the histologic subtype of the primary disease. In this chapter, we focus on the importance of a multidisciplinary approach to this diverse group of over 75 different tumor types. We will discuss in this chapter the etiology, clinical presentation, staging, histopathologic classification and prognostic factors for patients with soft tissue sarcoma. Surgery remains paramount to achieving cure for the vast majority of sarcomas. We will discuss how adjunctive therapies, like radiation and chemotherapy can improve overall outcome, as well as highlight the limitations and unanswered questions, both with respect to local recurrence and distant metastases. Where appropriate, we attempt to link specific histologies or molecular changes to the therapeutic approach to disease. We hope to draw attention to the fact that in a field that has traditionally had stagnation in the development of new therapeutic strategies, there has been a renaissance of new molecularly and mechanistically targeted therapies helping to improve outcomes for soft tissue sarcoma patients.

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Management and outcome of 239 adolescent and adult rhabdomyosarcoma patients

Cited by 51 publications

References 31 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

Soft Tissue Sarcomas

Soft Tissue Sarcomas

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