Rhabdomyosarcoma in adults: analysis of treatment modalities in a prospective single-center series

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Abstract

Rhabdomyosarcoma (RMS) is rare in adults and it is generally characterized by poor outcome. In a previous retrospective study, we demonstrated a better prognosis in adults treated with multimodality approach resembling pediatric protocols. Thereafter, we developed specific recommendations based on the principles adopted in pediatric oncology. The present analysis reports the results in a subsequent prospective series. The study included 95 consecutive patients (age 18–77 years) treated from 2002 to 2015 for embryonal and alveolar RMS. As in the previous series, patients were stratified by the appropriateness of their treatment according to therapeutic guidelines for childhood RMS. The 5-year event-free survival (EFS) and overall survival (OS) rates were 33.6% and 40.3%, respectively. The 5-year EFS was 40.8% for patients with the highest treatment score, and 15% for those with lower score, while OS was 44.4% and 24.5%, respectively. The developing of specific recommendations enabled an increase in the number of patients treated with intensive multimodal treatment resembling pediatric strategy (69.7% vs. 39.1% in the retrospective series). This study reinforced the idea that adherence to the principles of pediatric protocols, improves adult RMS outcomes. However, treating adults with pediatric-type strategy is not enough to achieve the results obtained in children. Issues in compliance and a more aggressive biology of adult RMS might have a role in the different outcome according to age. Improving the collaboration between pediatric and adult oncologists in promoting specific clinical and biological research is crucial to improve the outcome for this patient population.

Original languageEnglish
Article number59
JournalMedical Oncology
Volume36
Issue number7
DOIs
Publication statusPublished - Jul 1 2019

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Rhabdomyosarcoma
Pediatrics
Disease-Free Survival
Therapeutics
Alveolar Rhabdomyosarcoma
Embryonal Rhabdomyosarcoma
Combined Modality Therapy
Compliance
Survival Rate
Retrospective Studies
Guidelines
Survival
Research
Population

Keywords

  • Adults
  • Childhood tumors in adults
  • Multimodal treatment
  • Rhabdomyosarcoma
  • Soft tissue sarcoma
  • Treatment score

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this

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title = "Rhabdomyosarcoma in adults: analysis of treatment modalities in a prospective single-center series",
abstract = "Rhabdomyosarcoma (RMS) is rare in adults and it is generally characterized by poor outcome. In a previous retrospective study, we demonstrated a better prognosis in adults treated with multimodality approach resembling pediatric protocols. Thereafter, we developed specific recommendations based on the principles adopted in pediatric oncology. The present analysis reports the results in a subsequent prospective series. The study included 95 consecutive patients (age 18–77 years) treated from 2002 to 2015 for embryonal and alveolar RMS. As in the previous series, patients were stratified by the appropriateness of their treatment according to therapeutic guidelines for childhood RMS. The 5-year event-free survival (EFS) and overall survival (OS) rates were 33.6{\%} and 40.3{\%}, respectively. The 5-year EFS was 40.8{\%} for patients with the highest treatment score, and 15{\%} for those with lower score, while OS was 44.4{\%} and 24.5{\%}, respectively. The developing of specific recommendations enabled an increase in the number of patients treated with intensive multimodal treatment resembling pediatric strategy (69.7{\%} vs. 39.1{\%} in the retrospective series). This study reinforced the idea that adherence to the principles of pediatric protocols, improves adult RMS outcomes. However, treating adults with pediatric-type strategy is not enough to achieve the results obtained in children. Issues in compliance and a more aggressive biology of adult RMS might have a role in the different outcome according to age. Improving the collaboration between pediatric and adult oncologists in promoting specific clinical and biological research is crucial to improve the outcome for this patient population.",
keywords = "Adults, Childhood tumors in adults, Multimodal treatment, Rhabdomyosarcoma, Soft tissue sarcoma, Treatment score",
author = "Luca Bergamaschi and Rossella Bertulli and Michela Casanova and Salvatore Provenzano and Stefano Chiaravalli and Patrizia Gasparini and Paola Collini and Claudia Sangalli and Lorenza Gandola and Barbara Diletto and Carlo Morosi and Marco Fiore and Maura Massimino and Andrea Ferrari",
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T1 - Rhabdomyosarcoma in adults

T2 - analysis of treatment modalities in a prospective single-center series

AU - Bergamaschi, Luca

AU - Bertulli, Rossella

AU - Casanova, Michela

AU - Provenzano, Salvatore

AU - Chiaravalli, Stefano

AU - Gasparini, Patrizia

AU - Collini, Paola

AU - Sangalli, Claudia

AU - Gandola, Lorenza

AU - Diletto, Barbara

AU - Morosi, Carlo

AU - Fiore, Marco

AU - Massimino, Maura

AU - Ferrari, Andrea

PY - 2019/7/1

Y1 - 2019/7/1

N2 - Rhabdomyosarcoma (RMS) is rare in adults and it is generally characterized by poor outcome. In a previous retrospective study, we demonstrated a better prognosis in adults treated with multimodality approach resembling pediatric protocols. Thereafter, we developed specific recommendations based on the principles adopted in pediatric oncology. The present analysis reports the results in a subsequent prospective series. The study included 95 consecutive patients (age 18–77 years) treated from 2002 to 2015 for embryonal and alveolar RMS. As in the previous series, patients were stratified by the appropriateness of their treatment according to therapeutic guidelines for childhood RMS. The 5-year event-free survival (EFS) and overall survival (OS) rates were 33.6% and 40.3%, respectively. The 5-year EFS was 40.8% for patients with the highest treatment score, and 15% for those with lower score, while OS was 44.4% and 24.5%, respectively. The developing of specific recommendations enabled an increase in the number of patients treated with intensive multimodal treatment resembling pediatric strategy (69.7% vs. 39.1% in the retrospective series). This study reinforced the idea that adherence to the principles of pediatric protocols, improves adult RMS outcomes. However, treating adults with pediatric-type strategy is not enough to achieve the results obtained in children. Issues in compliance and a more aggressive biology of adult RMS might have a role in the different outcome according to age. Improving the collaboration between pediatric and adult oncologists in promoting specific clinical and biological research is crucial to improve the outcome for this patient population.

AB - Rhabdomyosarcoma (RMS) is rare in adults and it is generally characterized by poor outcome. In a previous retrospective study, we demonstrated a better prognosis in adults treated with multimodality approach resembling pediatric protocols. Thereafter, we developed specific recommendations based on the principles adopted in pediatric oncology. The present analysis reports the results in a subsequent prospective series. The study included 95 consecutive patients (age 18–77 years) treated from 2002 to 2015 for embryonal and alveolar RMS. As in the previous series, patients were stratified by the appropriateness of their treatment according to therapeutic guidelines for childhood RMS. The 5-year event-free survival (EFS) and overall survival (OS) rates were 33.6% and 40.3%, respectively. The 5-year EFS was 40.8% for patients with the highest treatment score, and 15% for those with lower score, while OS was 44.4% and 24.5%, respectively. The developing of specific recommendations enabled an increase in the number of patients treated with intensive multimodal treatment resembling pediatric strategy (69.7% vs. 39.1% in the retrospective series). This study reinforced the idea that adherence to the principles of pediatric protocols, improves adult RMS outcomes. However, treating adults with pediatric-type strategy is not enough to achieve the results obtained in children. Issues in compliance and a more aggressive biology of adult RMS might have a role in the different outcome according to age. Improving the collaboration between pediatric and adult oncologists in promoting specific clinical and biological research is crucial to improve the outcome for this patient population.

KW - Adults

KW - Childhood tumors in adults

KW - Multimodal treatment

KW - Rhabdomyosarcoma

KW - Soft tissue sarcoma

KW - Treatment score

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