Vulva, vagina & female urethra
Mesenchymal neoplasms
Rhabdomyosarcoma
Author: Nat Pernick, M.D.
Last author update: 1 March 2003
Last staff update: 1 February 2021
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Rhabdomyosarcoma vagina [title]
Table of Contents
Definition / general | Treatment | Gross description | Microscopic (histologic) description | Positive stains | Differential diagnosisCite this page: Pernick N. Rhabdomyosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/vaginarms.html. Accessed March 22nd, 2023.
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
Treatment
- Surgery, chemotherapy, radiation therapy
- 10 year survival is 91% but may die due to direct extension (Am J Surg Pathol 2001;25:856)
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
Differential diagnosis
- Polyposis vaginalis of pregnancy
