Overview of embryonal rhabdomyosarcoma of cervix in women over 40-year-old

The literature on cervical embryonal rhabdomyosarcoma(RMS) is reviewed here to identify management guidelines for middle-aged women diagnosed with this rare type of gynecologic cancer. Specifically, the Pub Med, Web of Science and Google Scholar databases, were searched to find published case series on cervical embryonal RMS reporting on four or more patients, of whom at least one was > 40-year-old. The χ2 test was used to assess heterogeneity. Five articles published between 1986 and 2013 were identified, reporting on a total of 47 patients, of whom 22(46.8%) were older and 25(53.2%) younger than 40-year-old. Although the two age groups did not differ significantly by stage of disease or radiotherapy treatment, the older age groupreceived less chemotherapy(55% vs 90%, P = 0.008)and had more hysterectomy(86% vs 43%, P = 0.009).Follow-up data was missing for 18/47(38.3%) patients.Among the 29 patients with follow-up data, survival was shorter in the older group, with 8/12(67%) alive and3 with disease at a median follow-up of 2.6 years, as compared with the younger group that had 15/17(88%)alive and none with disease at a median follow-up of 3.5years. The longest survivals among the older women were observed in those who received radiotherapy,including one case with a resected lung metastasis.A prospective multi-institutional collaboration and better follow-up are needed to determine the optimal management of cervical embryonal RMS. Long-term survival appears feasible if management is accompanied by chemotherapy and radiotherapy.

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

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.

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

Importance Rhabdomyosarcoma(RMS)is the most common soft tissue sarcoma in children.More than 90%of cases are classified as embryonic RMS(ERMS)or alveolar RMS(ARMS).ERMS has a worse prognosis than ARMS.Early differential diagnosis is of paramount importance for optimization of treatment.Objective To identify genes that are differentially expressed between ARMS and ERMS,which can be used for accurate rhabdomyosarcoma classification.Methods Three Gene Expression Omnibus datasets composed of ARMS and ERMS samples were screened and 35 differentially expressed genes(DEGs)were identified.Receiver operating characteristic curve analysis and area under the curve analysis was performed for these 35 DEGs and seven candidate genes with the best differential expression scores between ARMS and ERMS were determined.The expression of these seven candidate genes was validated by immunohistochemical analysis of pre-chemotherapy ARMS and ERMS specimens.Results The levels of DCX and CRABP2 were confirmed to be remarkably different between paraffin-embedded ARMS and ERMS tissues,while EGFR abundance was only marginally different between these two RMS subtypes.Interpretation DCX and CRABP2 are potential biomarkers for distinguishing ARMS from ERMS in pre-chemotherapy pediatric patients.