Botryoid Rhabdomyosarcoma of the cervix: A systematic review of the literature from 1937 to 2017 – all registered cases

Purpose: Embryonal rhabdomyosarcoma of the cervix (RMS) is a rare malignancy and occurs mainly in the first two decades of life. Botryoid rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma and is an aggressive malignancy that arises from embryonal rhabdomyoblasts. It is often found in the genital tract of infants and young children. Study design: we conducted a systematic review to accumulate the available evidence in the international literature. We searched Medline (1966–2021), Scopus (2004–2021), Clinicaltrials.gov (2008–2021), EMBASE (1980-2021), Cochrane Central Register of Controlled Trials CENTRAL (1999-2021), and Google Scholar (2004-2021) for relevant observational studies and case reports. Results: We retrieved 156 cases with a mean age of 24.4 years with the oldest patient at the age of 89 years and the youngest 67-day-old infant. Twenty-four patients died. Ninety-seven patients were free of disease (85%). Of those 11 (11.3%) patients died. Fifteen patients (15%) developed relapse and 13 (86.6%) of them died. In 42 cases we could not extract information concerning their survival. Discussion: The findings of our study are mainly limited to case reports and small case series, and there are no standard treatment guidelines available and no consensus on how to manage these cases. Surgery should be guided by the response to initial chemotherapy and should attempt to conserve the function of the bladder, rectum, vagina, and ovaries. As this is a disease of adolescence, fertility preservation in well-selected cases is of paramount importance.