Introduction/Background Uterine embryonal rhabdomyosarcoma (uERMS) in adult women is a malignant entityof mesenchymal origin that very rarely occurs in adult age. Almost all information’s about uERMS is coming by case reports, and probably remains an under-recognized neoplasm. The treatment is not codified, and recommended strategies are mostly based on treatments for RMS of paediatric population.
Methodology A comprehensive database searches were performed for studies ofadult uERMS (>19 years). Survival probabilities were estimated using Kaplan-Meier Method and potential risk factors for survival were tested using the the Cox model.
Results 116 patients from 58 articles were included.Mean ages at diagnosis was 36.6 years. Forty-ninewomen were older than 40 years. In half of them, the most common presenting symptoms was vaginal bleeding only. The median tumor size was 5 cm(ranged 0.5–27 cm). Nearly 90% of patients were classified in the IRS Group-I. Surgery was the main therapeutic modality. With a median follow-up time of 24 months, the estimate overall survival (OS) and progression free survival (PFS) at 5 years was 81.4% and respectively 80.6%. In univariate analysis, age >36.6 years, deeply invasive tumors, and IRS Group >IA were associated with worst OS. In bivariate analysis the age higher than 36.6 years and IRSG >IA remained associated with death from uERMS. A trend towards for recurrence was seen in patients with age >36.6 years, deeply invasive tumors, and IRS Group >IA.
Conclusion Uterine ERMS is a very rare disease among adult women. Multidisciplinary approach is essential taking into account that therapeutic management is still controversial. A relatively good 5-years OS and PFS is possible with a combination of surgery, radiotherapy, and multiagent chemotherapy, but a significantly risk of death exist in women with advanced age, with deep tumor invasion, and with IRS Group >IA.
Disclosure Nothing to disclose.
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.