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Archives of Gynecology and Obstetrics

Erschienen in:

20.06.2017 | Gynecologic Oncology

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

verfasst von: Dimitrios Nasioudis, Michail Alevizakos, Eloise Chapman-Davis, Steven S. Witkin, Kevin Holcomb

Erschienen in: Archives of Gynecology and Obstetrics | Ausgabe 2/2017

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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.

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

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):571–580CrossRefPubMed

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):3391–3397CrossRefPubMed

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):76–83CrossRefPubMedPubMedCentral

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

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):311–314CrossRefPubMed

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):4–7CrossRef

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

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):2117–2122CrossRefPubMed

10.

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

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):742–748CrossRefPubMed

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):3476–3485CrossRefPubMed

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):1815–1824PubMedPubMedCentral

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):2454–2468CrossRefPubMed

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):139–142CrossRefPubMed

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):78–83CrossRefPubMed

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) CrossRefPubMed

18.

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

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–e17CrossRefPubMed

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):103–115CrossRefPubMed

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):7143–7151CrossRefPubMed

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):599–604CrossRefPubMedPubMedCentral

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):970–976CrossRefPubMedPubMedCentral

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):196–201CrossRefPubMed

Titel: Rhabdomyosarcoma of the lower female genital tract: an analysis of 144 cases
verfasst von: Dimitrios Nasioudis
Michail Alevizakos
Eloise Chapman-Davis
Steven S. Witkin
Kevin Holcomb
Publikationsdatum: 20.06.2017
Verlag: Springer Berlin Heidelberg
Erschienen in: Archives of Gynecology and Obstetrics / Ausgabe 2/2017
Print ISSN: 0932-0067
Elektronische ISSN: 1432-0711
DOI: https://doi.org/10.1007/s00404-017-4438-1

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