Vulva, vagina & female urethra

Mesenchymal neoplasms


Topic Completed: 1 March 2003

Minor changes: 1 February 2021

Copyright: 2002-2021,, Inc.

PubMed Search: Rhabdomyosarcoma vagina [title]

Nat Pernick, M.D.
Page views in 2020: 1,626
Page views in 2021 to date: 1,638
Cite this page: Pernick N. Rhabdomyosarcoma. website. Accessed October 15th, 2021.
Definition / general
  • Most common malignant soft tissue tumor in children and adolescents
  • 6% of all malignancies in children; 20% occur in pelvic portion of GU tract
  • Usually embryonal or botryoid subtype
  • 10 year survival is 73% for classic embryonal subtype
  • Histologic maturation after treatment often occurs but these cells may be malignant
  • Botyroid variant of embryonal rhabdomyosarcoma is also known as sarcoma botryoides
    • Usually children < 5 years (2/3 under age 2) in anterior vaginal wall
Gross description
  • Grape-like clusters that fill and project out vagina
Microscopic (histologic) description
  • Small tumor cells with oval nuclei, cytoplasm protrudes from one end
  • Resemble tennis rackets with bright, eosinophilic granular cytoplasm suggesting of rhabdomyoblastic differentiation
  • May lack cross striations
  • Tumor cells crowded into cambium layer beneath intact vaginal epithelium and around blood vessels
  • In deep regions, lie within loose, fibromyxoid stroma with edema and inflammatory cells
  • May invade overlying epithelium
  • Neoplastic cartilage seen in older patients, may be associated with a better prognosis
Positive stains
Differential diagnosis
  • Polyposis vaginalis of pregnancy
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