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Rhabdomyosarcoma of the lower female genital tract: an analysis of 144 cases

  • Gynecologic Oncology
  • Published:
Archives of Gynecology and Obstetrics Aims and scope Submit manuscript

Abstract

Purpose

The aim of the present study was to elucidate the clinico-pathological characteristics of female patients with lower genital tract rhabdomyosarcoma (RMS) stratified by age group and investigate their prognosis, using a multi-institutional database.

Methods

The National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database was accessed (1973–2013) and a cohort of females diagnosed with RMS of the lower genital tract (vulva, vagina, cervix) was drawn. Five-year overall survival (OS) rate was estimated following generation of Kaplan–Meier curves and compared with the log-rank test.

Results

A total of 144 eligible cases were identified; 51.4 and 48.6% originated from the vagina/vulva and the cervix, respectively. Median patient age was 16 years and distant metastases were rare (ten cases). The majority of tumors were of embryonal histology (75.7%). Non-embryonal RMS was more prevalent in the older patient groups. Tumors originating from the cervix were more common among adolescents and premenopausal women. Rate of LN involvement was 52.9 and 20% for vulvovaginal and cervical tumors (p = 0.02). Five-year OS rate was 68.4%; factors associated with better OS were younger age, absence of distant metastasis, embryonal histology, negative LNs, and performance of surgery. For prepubertal girls and adolescents, radical surgery did not confer a survival benefit compared to local tumor excision.

Conclusion

RMS of the lower genital tract primarily affects prepubertal girls and adolescents, who have excellent survival rates; however, outcomes for adults remain poor.

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References

  1. Dagher R, Helman L (1999) Rhabdomyosarcoma: an overview. Oncologist 4(1):34–44

    CAS  PubMed  Google Scholar 

  2. Ferrari A, Dileo P, Casanova M et al (2003) Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution. Cancer 98(3):571–580

    Article  PubMed  Google Scholar 

  3. Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A (2009) Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol 27(20):3391–3397

    Article  PubMed  Google Scholar 

  4. Walterhouse DO, Meza JL, Breneman JC et al (2011) Local control and outcome in children with localized vaginal rhabdomyosarcoma: a report from the Soft Tissue Sarcoma committee of the Children’s Oncology Group. Pediatr Blood Cancer 57(1):76–83

    Article  PubMed  PubMed Central  Google Scholar 

  5. Kashi VP, Hatley ME, Galindo RL (2015) Probing for a deeper understanding of rhabdomyosarcoma: insights from complementary model systems. Nat Rev Cancer 15(7):426–439

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Yang J, Yang J, Yu M, Yuan Z, Cao D, Keng S (2017) Clinical study on female genital tract rhabdomyosarcoma in childhood: changes during 20 years in one center. Int J Gynecol Cancer 27(2):311–314

    Article  PubMed  Google Scholar 

  7. Bouchard-Fortier G, Kim RH, Allen L, Gupta A, May T (2016) Fertility-sparing surgery for the management of young women with embryonal rhabdomyosarcoma of the cervix: a case series. Gynecol Oncol Rep 26(18):4–7

    Article  Google Scholar 

  8. Surveillance, Epidemiology, and End Results (SEER) Program (https://seer.cancer.gov/data/) SEER*Stat Database: incidence—SEER 18 Regs Research Data, Nov 2015 Sub (1973–2013)—linked to county attributes—total U.S., 1969–2014 counties, National Cancer Institute, DCCPS, Surveillance Research Program, Surveillance Systems Branch, released April 2016, based on the November 2015 submission

  9. Martelli H, Oberlin O, Rey A et al (1999) Conservative treatment for girls with nonmetastatic rhabdomyosarcoma of the genital tract: a report from the Study Committee of the International Society of Pediatric Oncology. J Clin Oncol 17(7):2117–2122

    Article  CAS  PubMed  Google Scholar 

  10. Ghaemmaghami F, Karimi Zarchi M, Ghasemi M (2008) Lower genital tract rhabdomyosarcoma: case series and literature review. Arch Gynecol Obstet 278(1):65–69

    Article  PubMed  Google Scholar 

  11. Villella JA, Bogner PN, Jani-Sait SN, Block AM, Lele S (2005) Rhabdomyosarcoma of the cervix in sisters with review of the literature. Gynecol Oncol 99(3):742–748

    Article  PubMed  Google Scholar 

  12. Greenberg JA, Somme S, Russnes HE, Durbin AD, Malkin D (2008) The estrogen receptor pathway in rhabdomyosarcoma: a role for estrogen receptor-beta in proliferation and response to the antiestrogen 4′OH-tamoxifen. Cancer Res 68(9):3476–3485

    Article  CAS  PubMed  Google Scholar 

  13. Poniewierska-Baran A, Schneider G, Sun W, Abdelbaset-Ismail A, Barr FG, Ratajczak MZ (2016) Human rhabdomyosarcoma cells express functional pituitary and gonadal sex hormone receptors: therapeutic implications. Int J Oncol 48(5):1815–1824

    PubMed  PubMed Central  Google Scholar 

  14. Arndt CA, Donaldson SS, Anderson JR et al (2001) What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract? Cancer 91(12):2454–2468

    Article  CAS  PubMed  Google Scholar 

  15. Solomon LA, Zurawin RK, Edwards CL (2003) Vaginoscopic resection for rhabdomyosarcoma of the vagina: a case report and review of the literature. J Pediatr Adolesc Gynecol 16(3):139–142

    Article  CAS  PubMed  Google Scholar 

  16. Zeisler H, Mayerhofer K, Joura EA et al (1998) Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature. Gynecol Oncol 69(1):78–83

    Article  CAS  PubMed  Google Scholar 

  17. Andrassy RJ, Wiener ES, Raney RB et al (1999) Progress in the surgical management of vaginal rhabdomyosarcoma: a 25-year review from the Intergroup Rhabdomyosarcoma Study Group. J Pediatr Surg 34(5):731–734 (discussion 734–5)

    Article  CAS  PubMed  Google Scholar 

  18. Dumont SN, Araujo DM, Munsell MF et al (2013) Management and outcome of 239 adolescent and adult rhabdomyosarcoma patients. Cancer Med 2(4):553–563

    Article  PubMed  PubMed Central  Google Scholar 

  19. Ditto A, Martinelli F, Carcangiu M et al (2013) Embryonal rhabdomyosarcoma of the uterine cervix in adults: a case report and literature review. J Low Genit Tract Dis 17(4):e12–e17

    Article  PubMed  Google Scholar 

  20. Harel M, Ferrer FA, Shapiro LH, Makari JH (2016) Future directions in risk stratification and therapy for advanced pediatric genitourinary rhabdomyosarcoma. Urol Oncol 34(2):103–115

    Article  PubMed  Google Scholar 

  21. Spunt SL, Sweeney TA, Hudson MM, Billups CA, Krasin MJ, Hester AL (2005) Late effects of pelvic rhabdomyosarcoma and its treatment in female survivors. J Clin Oncol 23(28):7143–7151

    Article  PubMed  Google Scholar 

  22. Altaf S, Enders F, Lyden E, Donaldson SS, Rodeberg D, Arndt C (2014) Age-related toxicity in patients with rhabdomyosarcoma: a report from the children’s oncology group. J Pediatr Hematol Oncol 36(8):599–604

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Russell H, Swint JM, Lal L et al (2014) Cost minimization analysis of two treatment regimens for low-risk rhabdomyosarcoma in children: a report from the Children’s Oncology Group. Pediatr Blood Cancer 61(6):970–976

    Article  PubMed  PubMed Central  Google Scholar 

  24. Archer NM, Amorim RP, Naves R et al (2016) An increased risk of second malignant neoplasms after rhabdomyosarcoma: population-based evidence for a cancer predisposition syndrome? Pediatr Blood Cancer 63(2):196–201

    Article  PubMed  Google Scholar 

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Authors and Affiliations

Authors

Contributions

DN (Protocol/project development, data collection and management, data analysis, manuscript writing/editing), MA (Protocol/project development, data analysis, manuscript writing/editing, EC-D (Protocol/project development, data analysis, manuscript writing/editing), SW (Protocol/project development, manuscript writing/editing), KH (Protocol/project development, data analysis, manuscript writing/editing).

Corresponding author

Correspondence to Dimitrios Nasioudis.

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Funding

The authors declare no source of funding for the research.

Conflict of interest

All authors declare no potential conflict of interest, including and financial or personal relationship which could viewed as one.

Ethical standards

The SEER is a public dataset available to the public for research purpose. All patient data are de-identified.

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Nasioudis, D., Alevizakos, M., Chapman-Davis, E. et al. Rhabdomyosarcoma of the lower female genital tract: an analysis of 144 cases. Arch Gynecol Obstet 296, 327–334 (2017). https://doi.org/10.1007/s00404-017-4438-1

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  • DOI: https://doi.org/10.1007/s00404-017-4438-1

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