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

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Pediatric Investigation 2020年第4期4卷 242-249页

作者： Xinyu Wang Jun Feng Zhe Li Xin Zhang Jun Chen Guoshuang Feng Big Data and Engineering Research Center Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Department of Surgical Oncology Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina National Center for Pediatric Cancer Surveillance Beijing Children’s HospitalCapital Medical UniversityNational Center for Children’s HealthBeijingChina Beijing Advanced Innovation Center for Big Data-Based Precision Medicine Beihang University&Capital Medical UniversityBeijingChina

Importance:As the most common subtype of pediatric rhabdomyosarcoma(RMS),the prognosis of embryonal RMS has rarely been investigated ***:To perform a population-based study to characterize the prognosis of embryonal RMS in children and ***: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 *** 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 ***:A total of 464 patients were included in the analysis,among which 64.6%were male and 70.2%were white *** 38.6%and 26.3%of the patients were at 1-4 years and 5-9 years,*** 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 *** having distant tumors had significantly higher mortality risk(HR,4.842;95%CI,2.804-8.362)than the patients with localized *** 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.

关键词： embryonal rhabdomyosarcoma Childhood cancer Cancer survival Soft-tissue sarcoma

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

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World Journal of Obstetrics and Gynecology 2016年第1期5卷 110-117页

作者： Malala Razakanaivo Nam P Nguyen Juliette Thariat Vincent Molinie Anne-Therese Vlastos Claire Verschraegen Vincent Vinh-Hung Joseph Ravoahangy Andrianavalona University Hospital Radiation Oncology Howard University Hospital International Geriatric Radiotherapy Group Antoine-Lacassagne Cancer Center University Nice Sophia Antipolis Rare Cancer Network Department of Pathology University Hospital of Martinique Geneva Gynecology and Obstetrics Division of Hematology Oncology University of Vermont Cancer Center Radiation Oncology University Hospital of Martinique Radiotherapy Department UZ BrusselVrije Universiteit Brussel

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%) *** 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.

关键词： embryonal rhabdomyosarcoma Botryoid sarcoma Cervix Middle-aged adults Chemotherapy Radiotherapy Review

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