Comparing Adult and Pediatric Rhabdomyosarcoma in the Surveillance, Epidemiology and End Results Program, 1973 to 2005: An Analysis of 2,600 Patients

St. Jude Children's Research Hospital, Memphis, Tennessee, United States
Journal of Clinical Oncology (Impact Factor: 18.43). 04/2009; 27(20):3391-7. DOI: 10.1200/JCO.2008.19.7483
Source: PubMed


To compare clinical features and outcomes of adults and children reported to have rhabdomyosarcoma.
We analyzed data from 1,071 adults (age > 19 years) and 1,529 children (age < or = 19 years) reported in the public-access Surveillance, Epidemiology and End Results database as having rhabdomyosarcoma, diagnosed from 1973 to 2005. Survival estimates were determined using survival time with the end point being death from any cause.
Adults with rhabdomyosarcoma had significantly worse outcome than children (5-year overall survival rates, 27% +/- 1.4% and 61% +/- 1.4%, respectively; P < .0001). Tumors in adults were more likely to be at an unfavorable site (65% v 55%; P < .0001) and to have histologies that are unusual during childhood, particularly the pleomorphic subtype (19%) and not otherwise specified (43%). Regional and distant spread was not more frequent in adults. Adults had significantly worse outcome than children with similar tumors. The most significant difference was in localized disease; 5-year survival estimates were 82% +/- 2.0% for children and 47% +/- 2.9% for adults (P < .0001). Multivariate analysis showed that age, histologic subtype, primary site location, stage, and local control with surgery and/or radiation were significant predictors of survival. However, alveolar subtype and unfavorable primary site lost significance when analysis was restricted to adults.
Adults reported to have rhabdomyosarcoma had worse survival than children with similar tumors. Predictors of poor outcome in children were valid in adults except for alveolar histology and unfavorable tumor site.

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    • "PRMS usually occurs in adult males in the deep tissue of extremities but may occur at any site. In adult patients, the pleomorphic variant is associated with the worst prognosis [8] "
    Sarcoma 01/2015; 2015:14. DOI:10.1155/2015/232010
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    • "Tumors in adults were more likely to be at an unfavorable site and to have histologies that are unusual during childhood, particularly the pleomorphic and RMS NOS subtype. The most significant difference in outcome was in patients with localized disease: 5-year survival estimates were 82% for children and 47% for adults.7 Another study found that adolescent patients tended to have an increased likelihood for an unfavorable primary tumor site, metastatic disease, and lymph node involvement.55 "
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    ABSTRACT: Rhabdomyosarcoma (RMS), a malignant tumor of mesenchymal origin, is the third most common extracranial malignant solid tumor in children and adolescents. However, in adults, RMS represents <1% of all solid tumor malignancies. The embryonal and alveolar histologic variants are more commonly seen in pediatric patients, while the pleomorphic variant is rare in children and seen more often in adults. Advances in the research of the embryonal and alveolar variants have improved our understanding of certain genes and biologic pathways that are involved in RMS, but much less is known for the other variants. Multimodality therapy that includes surgery and chemotherapy with or without radiation therapy is the mainstay of treatment for RMS. Improvements in the risk stratification of the pediatric patients based on presurgical (primary tumor site, tumor size, regional lymph node involvement, presence of metastasis) and postsurgical parameters (completeness of resection or presence of residual disease or metastasis) has allowed for the treatment assignment of patients in different studies and therapeutic trials, leading to increases in 5-year survival from 25%-70% over the past 40 years. However, for adult patients, in great part due to rarity of the disease and the lack of consensus on optimal treatment, clinical outcome is still poor. Many factors have been implicated for the differing outcomes between pediatric RMS versus adult RMS, such as the lack of standardized treatment protocols for adult RMS patients and the increased prevalence of advanced presentations. Now that there are increased numbers of survivors, we can appreciate the sequelae from therapy in these patients, such as bone growth abnormalities, endocrinopathies, and infertility. Improvements in risk stratification have led to clinical trials using lower doses of chemotherapy or radiation therapy with the intention of decreasing the incidence of side effects without compromising survival outcome.
    Adolescent Health, Medicine and Therapeutics 06/2014; 5:115-25. DOI:10.2147/AHMT.S44582
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    • "Indeed, pediatric cancers are most likely to be referred to tertiary or quaternary cancer center, thus benefiting from a more accurate diagnosis, which is critical in this disease. According to the Surveillance, Epidemiology and End Results (SEER) study comparing adult and pediatric RMS patients between 1973 and 2005 for a total of 2600 patients, the histologic subtype was unknown for about 43% of adult patients versus 13.2% of pediatric patients 5. SEER data reflecting the average population management, we could assume that in the early 70s, many malignant fibrous histiocytoma were mistaken for PRMS by nonsarcoma-trained pathologists. "
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    ABSTRACT: Adult rhabdomyosarcoma (RMS) is a rare tumor that has inferior outcome compared to younger patient population. The present work aims to study the age-related differences in management of adolescents and adults with RMS. Under an institutional review board-approved protocol, we retrospectively analyzed 239 patients, 10 years of age and greater, diagnosed with RMS at MD Anderson Cancer Center from 1957 through 2003. Of the 239 patients, 163 patients were nonmetastatic with a median overall survival (OS) of 3.8 years (95% CI 2.8–7.6). In the multivariate analysis, age >50 was significantly associated with shorter OS and recurrence-free survival (RFS) for primary patients. Metastases were present in 76 patients, the median OS was 1.4 years. Approximately 13% of metastatic patients <50 years old had a long-term survival exceeding 15 years. Multimodality therapy, including surgery, radiotherapy, and chemotherapy was significantly associated with longer OS in primary and metastatic patients. Use of bi- and triple modality treatment decreased in metastatic patients over 50 years of age compared to younger patients. RMS in adolescents and adults has a poor outcome compared with younger individuals. Increased use of multidisciplinary therapy may improve older patient clinical outcome. Adult rhabdomyosarcoma is a rare entity that has inferior outcome compared to younger patient population. This retrospective study emphasizes the age-related differences in management of patients that may partly explain their poor prognosis.
    Cancer Medicine 08/2013; 2(4):553-63. DOI:10.1002/cam4.92 · 2.50 Impact Factor
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