Iyad Sultan

PURPOSE: To compare clinical features and outcomes of adults and children reported to have rhabdomyosarcoma. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

BACKGROUND: Soft tissue sarcomas (STS) are a heterogeneous group of mesenchymal malignancies that occur throughout the lifespan. The impact of age on disease features and outcome is unclear. METHODS: We analyzed the clinical features and outcome of all STS cases registered between 1973 and 2006 in the SEER database. RESULTS: There were 48,012 cases that met the selection criteria. Individuals less than 20 years of age represented 5.6%, with rhabdomyosarcoma being the most common subtype. In adults, the most common types were Kaposi sarcoma, fibrohistiocytic tumors, and leiomyosarcoma. Rhabdomyosarcoma was the only entity with a median age <20 years. Male predominance (male/female of 1.5:1) was noticed for almost all types of STS, except for alveolar soft part sarcoma and leiomyosarcoma. Tumor stage was similar across different age groups. Younger patients (<50 years) had significantly better survival than older patients (88.8 ± 0.2% vs. 40 ± 0.3%, P < 0.001), but for most histologies the survival decline with advancing age was gradual and did not occur abruptly at the onset of adulthood. The decline in survival with advancing age was particularly significant for rhabdomyosarcoma. CONCLUSION: With few exceptions, the clinical features of STS are similar in children and adults. However, individuals over 50 years of age have an inferior survival.

BACKGROUND: Synovial sarcoma (SS) is a typical soft tissue sarcoma subtype crosswise between the pediatric and the adult age groups. Less satisfactory overall outcome has been recorded in adult series. METHODS: This study compares clinical features and outcomes of SS across the different age groups, by analyzing 1268 cases, 213 children/adolescents (<or=18 years) and 1055 adults, registered in the Surveillance, Epidemiology, and End Results (SEER) 17 database from 1983 to 2005. Cancer-specific survival estimates were compared with univariate and multivariate models. RESULTS: No major differences in stage distribution (localized, regional, and distant stage) were observed comparing the 2 age groups. The estimated 5-year cancer-specific survival was 83% for children/adolescents and 62% for adults (P<.001). Female sex, nonblack race, tumors located in the extremities, localized tumors, and tumors <5 cm in size were associated with better survival. In multivariate analysis, adult patients had significantly higher mortality rates than children after adjusting for other variables. CONCLUSIONS: Children and adults with SS have a similar clinical presentation but a dissimilar outcome, suggesting that factors other than unfavorable clinical features might be involved in the unsatisfactory outcome of adult SS patients. It remains to be ascertained whether this difference is related to biological variables or to historically different treatment approaches adopted in pediatric versus adult patients.

BACKGROUND: In estimating the global burden of cancer, adolescents and young adults with cancer are often overlooked, despite being a distinct subgroup with unique epidemiology, clinical care needs, and societal impact. Comprehensive estimates of the global cancer burden in adolescents and young adults (aged 15-39 years) are lacking. To address this gap, we analysed results from the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2019, with a focus on the outcome of disability-adjusted life-years (DALYs), to inform global cancer control measures in adolescents and young adults. METHODS: Using the GBD 2019 methodology, international mortality data were collected from vital registration systems, verbal autopsies, and population-based cancer registry inputs modelled with mortality-to-incidence ratios (MIRs). Incidence was computed with mortality estimates and corresponding MIRs. Prevalence estimates were calculated using modelled survival and multiplied by disability weights to obtain years lived with disability (YLDs). Years of life lost (YLLs) were calculated as age-specific cancer deaths multiplied by the standard life expectancy at the age of death. The main outcome was DALYs (the sum of YLLs and YLDs). Estimates were presented globally and by Socio-demographic Index (SDI) quintiles (countries ranked and divided into five equal SDI groups), and all estimates were presented with corresponding 95% uncertainty intervals (UIs). For this analysis, we used the age range of 15-39 years to define adolescents and young adults. FINDINGS: There were 1·19 million (95% UI 1·11-1·28) incident cancer cases and 396 000 (370 000-425 000) deaths due to cancer among people aged 15-39 years worldwide in 2019. The highest age-standardised incidence rates occurred in high SDI (59·6 [54·5-65·7] per 100 000 person-years) and high-middle SDI countries (53·2 [48·8-57·9] per 100 000 person-years), while the highest age-standardised mortality rates were in low-middle SDI (14·2 [12·9-15·6] per 100 000 person-years) and middle SDI (13·6 [12·6-14·8] per 100 000 person-years) countries. In 2019, adolescent and young adult cancers contributed 23·5 million (21·9-25·2) DALYs to the global burden of disease, of which 2·7% (1·9-3·6) came from YLDs and 97·3% (96·4-98·1) from YLLs. Cancer was the fourth leading cause of death and tenth leading cause of DALYs in adolescents and young adults globally. INTERPRETATION: Adolescent and young adult cancers contributed substantially to the overall adolescent and young adult disease burden globally in 2019. These results provide new insights into the distribution and magnitude of the adolescent and young adult cancer burden around the world. With notable differences observed across SDI settings, these estimates can inform global and country-level cancer control efforts. FUNDING: Bill & Melinda Gates Foundation, American Lebanese Syrian Associated Charities, St Baldrick's Foundation, and the National Cancer Institute.

BACKGROUND: Pediatric gliomas are rare and heterogeneous tumors. The Surveillance, Epidemiology, and End Results (SEER) database allows a large-scale analysis of the clinical characteristics and prognostic features of these tumors. METHODS: The authors analyzed available SEER data on 6212 patients younger than 20 years at diagnosis of glioma (1973-2005), according to 4 age categories: <1 year, 1-3 years, 3-5 years, and 5-20 years. RESULTS: The overall 5- and 10-year survival estimates were 71%+/-0.62% (standard error) and 68%+/-0.67%, respectively. Forty-one percent of gliomas were cerebral; the frequency of cerebellar tumors (22%-32% of gliomas) increased sharply after the first year of life. Of the tumors for which grade was available, 77% were low grade (grade I or II). Tumor grade emerged as the most significant independent prognostic factor in all age groups except the youngest age group, in which extent of resection was most significant. Surgery other than gross total resection was an adverse prognostic factor (hazard ratio, 2.18; 95% confidence interval, 1.78-2.67). Age<3 years predicted a greater likelihood of survival in patients with high-grade gliomas and brainstem tumors. Conversely, age<3 years predicted a lower likelihood of survival in patients with low-grade gliomas. Children aged<1 year received less radiotherapy than older patients (P<.0001) and were less likely to undergo gross total resection (P<.0001). CONCLUSIONS: The survival of children with gliomas is influenced by histologic subtype, age, and extent of resection. Despite its limitations, the SEER database provides a useful tool for studies of rare tumors such as pediatric gliomas.