Objectives Adult primary cervical rhabdomyosarcoma is a very rare disease and data regarding treatment are sparce. The goal of this study was to report on our experience with the management of this rare entity, along with an evaluation of the literature.
Methods We conducted a review of the medical records at four centers from January 1990 to December 2017. We reviewed clinical characteristics including age at diagnosis, BMI, medical history and tumor stage, as well as treatment in the primary and recurrent settings and follow-up data. We reclassified tumors according to the Intergroup Rhabdomyosarcoma Study (IRS) clinical group.
Results A total of 15 patients were included in the analysis. Median age at diagnosis was 35 years (range 17–55). Median tumor size at presentation was 5 cm (range 3–10). Eleven patients had the embryonal variant, including five showing the botryoid subtype. Four patients had a pleomorphic rhabdomyosarcoma. Eleven patients had disease classified as IRS Clinical Group I, while the remaining four fell into groups II or III. Fertility-sparing treatment was offered to five patients. Primary treatment types were: surgery alone in eight patients, surgery followed by adjuvant chemotherapy in six patients, and neoadjuvant chemotherapy in two patients. The main risk factors for relapse were: IRS clinical group greater than I, tumor size greater than 5 cm, lymph nodal involvement, and non-embryonal histology. At a median follow-up of 35 months (range 3–282), we observed a 5-year overall survival rate of 78.2% and a progression-free survival of 58.2%. No patient in the IRS I group died of the disease. Three out of four patients in the IRS II-III group died of the disease (survival range 5–16 months following treatment).
Conclusion Our data show that cervical rhabdomyosarcomas account for at least two prognostic groups, demonstrating the existence of low-risk and high-risk patterns. The best predictor of prognosis appearsd to be the IRS clinical group classification system. IRS Group I tumors had an overall good prognosis and rarely recurred; when they did recur they were mainly local, following conservative treatment.
- Cervical Cancer
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Correction notice Since this paper was first published, The Department of Gynecology, Campus Virchow-Klinikum, Charité Medical University, Berlin, Germany has been added as a second affiliation to Helmut Plett. Additionally, affiliations 3, 6 and 8 have been updated to include 'IRCCS'.
Contributors ER conceived the study, collected and analyzed data and wrote the paper. HP collected data. HP, FL, GA, SP, AdB, PH, NC edited the manuscript and supported the data analysis and collection, supervised the findings of this work. VS focused on the pathological aspects of the study, All authors discussed the results and contributed to the final manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests ER reports personal fees from Astra Zeneca, outside the submitted work, HP reports non-financial support from Tesaro, outside the submitted work, SP reports non-financial support from Tesaro, outside the submitted work; PH reports grants and personal fees from Astra Zeneca, grants and personal fees from Roche, personal fees from Sotio, grants and personal fees from Tesaro, personal fees from Stryker, personal fees from Zai Lab, personal fees from MSD, grants and personal fees from Public funding (ASCO, DKH, DFG), personal fees from Clovis, personal fees from Immunogen, grants from GSK, grants from Boehringer Ingelheim, grants from Medac, grants from Genmab, outside the submitted work; AdB reports grants and personal fees from Roche, Astra Zeneca, Tesaro, Clovis, Pfizer, Biocad, Genmab / Seattle Genetics, MSD, outside the submitted work. NC rreports grants and personal fees from Roche, Astra Zeneca, Pharmamar, Tesaro, Clovis, Advaxis, Pfizer, Takeda, Immunogen, Biocad, outside the submitted work.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.