Clinical Investigation
Adult Rhabdomyosarcoma Survival Improved With Treatment on Multimodality Protocols

https://doi.org/10.1016/j.ijrobp.2012.12.016Get rights and content

Purpose

Rhabdomyosarcoma (RMS) is a pediatric sarcoma rarely occurring in adults. For unknown reasons, adults with RMS have worse outcomes than do children.

Methods and Materials

We analyzed data from all patients who presented to Memorial Sloan-Kettering Cancer Center between 1990 and 2011 with RMS diagnosed at age 16 or older. One hundred forty-eight patients met the study criteria. Ten were excluded for lack of adequate data.

Results

The median age was 28 years. The histologic diagnoses were as follows: embryonal 54%, alveolar 33%, pleomorphic 12%, and not otherwise specified 2%. The tumor site was unfavorable in 67% of patients. Thirty-three patients (24%) were at low risk, 61 (44%) at intermediate risk, and 44 (32%) at high risk. Forty-six percent were treated on or according to a prospective RMS protocol. The 5-year rate of overall survival (OS) was 45% for patients with nonmetastatic disease. The failure rates at 5 years for patients with nonmetastatic disease were 34% for local failure and 42% for distant failure. Among patients with nonmetastatic disease (n=94), significant factors associated with OS were histologic diagnosis, site, risk group, age, and protocol treatment. On multivariate analysis, risk group and protocol treatment were significant after adjustment for age. The 5-year OS was 54% for protocol patients versus 36% for nonprotocol patients.

Conclusions

Survival in adult patients with nonmetastatic disease was significantly improved for those treated on RMS protocols, most of which are now open to adults.

Introduction

Rhabdomyosarcoma (RMS) is a rare malignancy, with an incidence of 4.5 per million people younger than 20 years in the United States. It is largely a disease of childhood; more than 50% of cases are diagnosed before the age of 10 (1). Whereas the 5-year overall survival (OS) rates of RMS in the pediatric population have improved in recent years to approximately 70%, the survival in adult populations with RMS is consistently lower, with 5-year OS rates ranging from 40% to 54% (2) In many pediatric studies, age itself is identified as a poor prognostic feature, with children older than 10 demonstrating lower rates of survival than children 1 to 9 years of age (3). Some of this difference in outcome between children and adults has been attributed to increased incidence of poor prognostic features in adults such as unfavorable primary site, unfavorable histologic diagnosis, and higher rates of regional and distant spread (4). The extent to which discrepancies in the treatment of the pediatric and adult populations with RMS plays a role in the lower survival rates among adults is unknown (5).

To better understand the role of demographic, clinical, and treatment variables in adult patients with RMS, we analyzed a large cohort of patients with RMS with a focus on both prognostic variables and treatment parameters.

Section snippets

Patients

We analyzed data from all patients who presented to Memorial Sloan-Kettering Cancer Center (MSKCC) between 1990 and 2011 and received diagnoses of RMS at age 16 or older. Details regarding demographic features, tumor characteristics, surgery, radiation, and chemotherapy were collected from the electronic medical records and the patients' charts. The variables that were analyzed included age at diagnosis, histologic diagnosis, primary site distribution, Children's Oncology Group (COG) risk

Patients

Of the 148 patients who met the study criteria, 10 were excluded for lack of adequate data. The median follow-up time was 43 months (range, 1-254 months) for surviving patients. Demographic and clinical characteristics are shown in Table 1. The median age was 28 years (range, 16-86 years). Embryonal tumors were present in 74 patients (54%), alveolar in 45 patients (33%), pleomorphic in 16 patients (12%), and NOS in 3 patients (2%). The majority of patients had an unfavorable primary site: 24

Discussion

Rhabdomyosarcoma is the most common pediatric sarcoma, and large multi-institutional trials have detailed its biologic and clinical characteristics. In particular, the Intergroup Rhabdomyosarcoma Studies (IRS) provide long-term survival data on thousands of children (<21 years of age) 3, 6, 7, 8. With regard to adult RMS, much of the published literature is from single-institution series, which report on clinical parameters and survival data for adults with RMS.

The data from these large

Conclusions

The results of the current study indicate that adults with RMS have increased rates of poor prognostic features such as unfavorable primary site. For the entire cohort, OS is poor in comparison with pediatric survival rates, as demonstrated in multiple large-scale studies. However, for a subset of patients with nonmetastatic disease treated on prospective RMS protocols, survival is significantly improved and approaches the survival rates seen in the pediatric population.

Acknowledgment

The authors thank Lawrence A. Herman for editorial assistance.

References (19)

  • S.L. Wolden et al.

    Sarcomas across the age spectrum

    Semin Radiat Oncol

    (2010)
  • S. Ognjanovic et al.

    Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005

    Cancer

    (2009)
  • M.A. Smith et al.

    Outcomes for children and adolescents with cancer: Challenges for the twenty-first century

    J Clin Oncol

    (2010)
  • W.M. Crist et al.

    Intergroup rhabdomyosarcoma study-IV: Results for patients with nonmetastatic disease

    J Clin Oncol

    (2001)
  • I. Sultan et al.

    Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: An analysis of 2,600 patients

    J Clin Oncol

    (2009)
  • W. Crist et al.

    The third intergroup rhabdomyosarcoma study

    J Clin Oncol

    (1995)
  • H.M. Maurer et al.

    The intergroup rhabdomyosarcoma study-I. A final report

    Cancer

    (1988)
  • H.M. Maurer et al.

    The intergroup rhabdomyosarcoma study-II

    Cancer

    (1993)
  • H.K. Ahn et al.

    Analysis of prognostic factors of pediatric-type sarcomas in adult patients

    Oncology

    (2011)
There are more references available in the full text version of this article.

Cited by (63)

  • Adolescents and young adults with rhabdomyosarcoma treated in the European paediatric Soft tissue sarcoma Study Group (EpSSG) protocols: a cohort study

    2022, The Lancet Child and Adolescent Health
    Citation Excerpt :

    The inferior outcome of adolescents and young adults with rhabdomyosarcoma has been previously reported,3,12–17 and multiple potential factors have been suggested to play a role in this survival difference. Among others, differences in clinical approach and treatment were considered.9,10,20–23,29,30 Compared with children, adolescent and young adult patients often experience decentralised care and are not often enrolled into clinical trials.

  • Embryonal and Alveolar Rhabdomyosarcoma in Adults: Real-Life Data From a Tertiary Sarcoma Centre

    2020, Clinical Oncology
    Citation Excerpt :

    A recent study reported the overall survival of adult (>16 years) ERMS and ARMS patients to be 21% [12]. Other analyses in adult RMS, including pleomorphic RMS, reported a 5-year overall survival in the range of 21–40% (Table 1) [12,15–17,22,23,27]. Possible explanations for the inferior prognosis of adult patients may be related to biological and/or treatment-related factors.

  • Adolescent and Young Adult (AYA) Oncology, Version 2.2024

    2023, JNCCN Journal of the National Comprehensive Cancer Network
View all citing articles on Scopus

Conflict of interest: none.

View full text