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

Positive stains
=========================================================================

• Desmin, actin (usually)

General
=========================================================================

• Aka 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
=========================================================================

• Grapelike clusters that fill and project out vagina

Micro 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
=========================================================================

• Desmin, actin

Differential diagnosis
=========================================================================

• Polyposis vaginalis of pregnancy

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).