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

Abstract

BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents aged <20 years; its etiology remains largely unknown. It is believed that embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS), the most common subtypes, arise through distinct biologic mechanisms. The authors of this report evaluated incidence and survival trends by RMS demographic subgroups to inform future etiologic hypotheses. METHODS: Incidence and survival trends in RMS among children and adolescents aged <20 years were analyzed using data from the Surveillance, Epidemiology, and End Results Program. Frequencies, age‐adjusted incidence and survival rates, and joinpoint regression results, including annual percentage change (APC) and 95% confidence interval (CI), were calculated. RESULTS: Between 1975 and 2005, the incidence of ERMS was stable, whereas a significant increase in the incidence of ARMS was observed (APC, 4.20%; 95%CI, 2.60%‐5.82%). This trend may have been attributable in part to shifts in diagnosis, because a significant negative trend in RMS, not otherwise specified was observed concurrently. A bimodal age peak for ERMS was observed, with the second, smaller peak in adolescence noted for males only; ARMS incidence did not vary by age or sex. Five‐year survival rates for RMS and ERMS increased during the period from 1976 to 1980 (52.7% and 60.9%, respectively) to the period from 1996 to 2000 (61.8% and 73.4%, respectively), whereas there was little improvement for ARMS (40.1% and 47.8%, respectively). CONCLUSIONS: Observed differences in incidence and survival for 2 major RMS subtypes across sex and age subgroups further supported the hypothesis that there are unique underlying etiologies for these tumors. Exploration of these differences presents an opportunity to increase current knowledge of RMS. Cancer 2009. © 2009 American Cancer Society.

Uncovering metabolism in rhabdomyosarcoma

“…21,22 Patients who have localized RMS have a 5-year survival greater than 70% following a multimodal approach that includes chemotherapy, radiation therapy, and surgery; yet, overall survival of patients with metastasis remains poor. 23,24 Cells mainly deriving from myogenic lineages have been shown to contribute to RMS development in mouse [25][26][27][28] and zebrafish models, 29 yet even nonmyogenic lineages could participate in the formation of RMS. 30 As a result, these tumors can arise in or near to skeletal muscle districts as well as in sites that lack skeletal muscle, such as the biliary and genitourinary tract.…”

Birth characteristics and the risk of childhood rhabdomyosarcoma based on histological subtype

“…Further, the two major histological subtypes (embryonal and alveolar RMS) have distinct localisations, ages of onset, incidence and survival trends (Ries, 1999;Ognjanovic et al, 2009) and distinct tumour gene expression signatures (Davicioni et al, 2006;Wachtel et al, 2006;Lae et al, 2007), suggesting that their aetiologies may be distinct. Embryonal RMS is characterised by earlier age of onset than alveolar RMS; the majority of embryonal RMS cases are diagnosed in the first decade of life, with more than half of cases occurring before the age of 5 years.…”

Post Traumatic Dorsal Mass of a Child Hand: Rhabdomyosarcoma Must Be Suspected

“…The trauma can occur years before the appearance of the tumor or a very short time before its recognition. In both cases, rapidly growing, persistent firm, or non-mobile lesions should raise the suspicion of malignancy [4]. Also, biological disturbance as in our described case should have drawn the attention and suggested malignancy.…”