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A rare germ-cell tumor site: vaginal endodermal sinus tumor
M Arora1, R K Shrivastav, M P Jaiprakash
1Department of Pediatric Surgery, Command Hospital, Kolkata, India. dr_manu_arora@hotmail.com
Pediatric Surgery International
|November 5, 2002
Summary
Endodermal sinus tumors (EST) are rare pediatric vaginal cancers. Early diagnosis via histology and elevated alpha-fetoprotein (AFP), combined with partial vaginectomy and chemotherapy, offers a successful treatment for this aggressive malignancy.
Area of Science:
- Pediatric Oncology
- Gynecologic Oncology
- Pathology
Background:
- Malignant germ-cell tumors (MGCT) are rare, comprising <3% of pediatric cancers.
- Endodermal sinus tumor (EST) is the most frequent MGCT subtype.
- Vaginal MGCTs are exceptionally rare, primarily affecting children under three.
Observation:
- A 9-month-old female presented with vaginal bleeding, a protruding mass, and urinary difficulty.
- Ultrasound revealed a hyperechoic mass posterior to the bladder.
- Histopathology confirmed a vaginal endodermal sinus tumor (EST).
Findings:
- The patient had an elevated serum alpha-fetoprotein (AFP) level.
- Partial vaginectomy with complete tumor excision was performed.
- Six cycles of cis-platinum, etoposide, and bleomycin chemotherapy resulted in disease-free status.
Implications:
- Vaginal EST is a rare, highly malignant pediatric GCT requiring prompt diagnosis and treatment.
- Combination therapy of partial vaginectomy and chemotherapy is the recommended approach.
- Serum AFP is a crucial marker for diagnosis and monitoring recurrence, preserving sexual and reproductive function.