Characteristics and prognosis of embryonal rhabdomyosarcoma in children and adolescents: An analysis of 464 cases from the SEER database

Abstract

Importance As the most common subtype of pediatric rhabdomyosarcoma (RMS), the prognosis of embryonal RMS has rarely been investigated solely. Objective To perform a population‐based study to characterize the prognosis of embryonal RMS in children and adolescents. Methods Demographic and clinical features were retrospectively evaluated in selected patients with embryonal RMS registered in the Surveillance, Epidemiology, and End Results (SEER) program from 1988 to 2016. Survival curves were compared using the log‐rank test. A multivariate Cox proportional hazards model was developed to assess the impact of each factor on the overall survival. A nomogram was constructed based on the results of Cox regression model. Results A total of 464 patients were included in the analysis, among which 64.6% were male and 70.2% were white patients. About 38.6% and 26.3% of the patients were at 1–4 years and 5–9 years, respectively. Cox analysis showed that patients at age group 5–9 years had the lowest risk of mortality (hazard ratio [ HR ], 0.277; 95% confidential interval [ CI ], 0.123–0.620), compared with patients diagnosed at less than 1‐year‐old, and age group 1–4 years had the second‐best prognosis. Patients having distant tumors had significantly higher mortality risk ( HR , 4.842; 95% CI , 2.804–8.362) than the patients with localized tumor. Compared with receiving no surgery or radiotherapy, receiving any combination of surgery and radiotherapy would lower the risk of mortality significantly (for surgery without radiotherapy: HR , 0.418; for radiotherapy without surgery: HR , 0.405; and for surgery plus radiotherapy: HR , 0.410). Interpretation Age, stage at diagnosis, and treatment received were found to be the most important predictors of the overall survival of pediatric embryonal RMS.

Primary Pleomorphic Omental Rhabdomyosarcoma in an Adult: A Report of a Unique Case

“…RMS is a very rare, highly malignant neoplasm thought to arise in immature striated skeletal muscle cells at any age and any site of the body. Generally, RMS is the most often encountered subtype of soft-tissue sarcomas, and it only accounts for 3% of childhood cancers and 2% of adolescent cancers [ 1 ]. It is extremely infrequent in adults and it accounts for less than 1% of all adult cancers [ 2 ].…”

“…The tumour nomenclature is derived from the Greek words rhabdo, which means rod shape, and myo, which means muscle. Rhabdomyosarcoma (RMS) is recognised as the most frequent childhood soft-tissue sarcomas, and it is encountered in more than 50% of all soft-tissue sarcomas in the paediatric and adolescent age groups [ 1 ]. It is less frequently encountered in the adult population, and in the published literature, particularly, the primary omental RMS has been rarely reported [ 2 , 3 ].…”

DCX and CRABP2 are candidate genes for differential diagnosis between pre‐chemotherapy embryonic and alveolar rhabdomyosarcoma in pediatric patients

“…Cancer is essentially a genetic disease in which many genetic variations accumulate during the multistep process of carcinogenesis, leading to abnormal unrestrained cell growth and malignancy 18 . RMS is a malignant solid tumor occurring most commonly in children 19 . If diagnosis is made after metastasis has occurred, the 5‐year survival rate of RMS, even with the best treatment, is below 20%.…”

Clinical characteristics and outcomes of infants compared with children diagnosed with rhabdomyosarcoma: Analysis of surveillance, epidemiology and end results data from 2000 to 2016

“…Patients were dichotomized into age groups including infants (aged <1 year) and children aged 1–9 years. The age cutoff of <1 year was selected given literature indicating that infants with RMS experience worse outcomes than older children 5,8,9,16,17,19 . Primary tumor sites were grouped in accordance with anatomic site designation utilized by the Children's Oncology Group 20 .…”

“…Prior studies show age at diagnosis of RMS influences risk for treatment failure, with children <1 year and >10 years having worse outcomes than those aged 1–9 years 5,6,8,9 . In general, children aged 0–12 years have better prognoses than those 13–19 years when assessing all histological subtypes of RMS 5 .…”