Abstract
Study Objective
To review our local experience with urogenital rhabdomyosarcoma (RMS) to determine
the most common clinical presentation(s).
Design
Retrospective case series of all female patients with urogenital RMS who presented
to a tertiary pediatric hospital between 1996 and 2016. All institutional electronic
pathology reports were screened for RMS and those that were pelvic in origin and occurred
in female patients were included for further analysis. Seventeen cases of urogenital
RMS in female patients were identified and reviewed.
Setting
This study was conducted at The Royal Children's Hospital in Melbourne, Australia.
This is a tertiary referral center for the state of Victoria and surrounding areas,
which services more than 1.5 million pediatric patients.
Participants
Female pediatric patients (ages 0-18 years) who presented to The Royal Children's
Hospital with eventual pathologic tissue diagnosis of urogenital RMS.
Main Outcome Measures
The cases were reviewed for clinical presentation, duration of symptoms before initial
presentation, time to tissue diagnosis, and outcomes of treatment.
Results
Of the 17 cases reviewed, 5 (29%) presented with perineal mass, 4 (24%) presented
with each of abdominal mass and grape-like lesions/hemorrhagic mass at the introitus,
3 (18%) with nonspecific symptoms only, and 1 (6%) with vulvar inflammation.
Conclusion
The clinical presentation of urogenital RMS in women is heterogeneous, and the classically
described presentation of grape-like lesions at the introitus and vaginal bleeding
represents only a small proportion of clinical presentations. Awareness of other presentations,
which appear to be more common than previously recognized, needs to be increased to
ensure timely diagnosis and treatment.
Key Words
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of Pediatric and Adolescent GynecologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Soft tissue sarcomas of childhood.Cancer Treat Rev. 2004; 30: 269
- Current status of treatment for pediatric rhabdomyosarcoma in the USA and Japan.Pediatr Int. 2016; 58: 81
- The Intergroup Rhabdomyosarcoma Study-I. A final report.Cancer. 1988; 61: 209
- The Intergroup Rhabdomyosarcoma Study-II.Cancer. 1993; 71: 1904
- The Third Intergroup Rhabdomyosarcoma Study.J Clin Oncol. 1995; 13: 610
- Common musculoskeletal tumors of childhood and adolescence.N Engl J Med. 1999; 341: 342
- Rhabdomyosarcoma.Curr Probl Cancer. 2008; 32: 7
- Malignant gynecologic tumors.in: Sutow W.W. Fernbach D.J. Vietti T.J. Clinical Pediatric Oncology. 3rd ed. CV Mosby Co, St Louis1984 (p. 744-760.)
- Pediatric Surgery.in: Brunicardi F. Andersen D.K. Billiar T.R. Schwartz's Principles of Surgery. 10th ed. McGraw-Hill, New York, NY2015 (chapter 39, p. 75-76)
- Vaginal Cancer.in: Hoffman B.L. Schorge J.O. Bradshaw K.D. Williams Gynecology. 3rd ed. McGraw-Hill, New York, NY2016 (chapter 32, p. 7-8)
- Embryonal rhabdomyosarcoma (botryoid type) of the vagina: a clinicopathologic review.Am J Obstet Gynecol. 1970; 107: 484
- Sarcoma botryoides of the female genital tract.Obstetr Genecol. 1985; 66: 262
- Australian Bureau of Statistics: 2016 Census QuickStats.(Available at:)http://www.censusdata.abs.gov.au/census_services/getproduct/census/2016/quickstat/2RVIC?opendocumentDate accessed: July 29, 2017
- SIOP MMT95: intensified (6 drug) versus standard (IVA) chemotherapy for high risk nonmetastatic rhabdomyosarcoma (RMS).J Clin Oncol. 2004; 22: 802s
- Rhabdomyosarcoma: Review of the Children's Oncology Group (COG) Soft Tissue Sarcoma Committee Experience and rationale for current COG studies.Pediatr Blood Cancer. 2012; 59: 5
- Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995.National Cancer Institute, SEER Program, Bethesda, MD1999 (Publication #NIH 99-4649)
- Childhood soft tissue sarcoma: a 20-years experience.J Pediatr. 1997; 131: 603
Article info
Publication history
Published online: February 05, 2018
Footnotes
The authors indicate no conflicts of interest.
Identification
Copyright
© 2018 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc.