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Endodermal germ cell carcinoma (endodermal sinus tumor) of the vagina in infant girls
Insights
A rare vaginal endodermal sinus tumor in an infant was successfully treated with surgery and chemotherapy. The child remains cancer-free, showing good development, highlighting effective treatment for this pediatric malignancy.
Area of Science:
- Gynecologic Oncology
- Pediatric Oncology
- Pathology
Background:
- Endodermal sinus tumors (yolk sac tumors) are rare, highly malignant germ cell neoplasms.
- Vaginal endodermal sinus tumors are exceptionally uncommon in infants, presenting diagnostic and therapeutic challenges.
Observation:
- A case of a 1-year-old girl with a vaginal endodermal sinus tumor presenting with hemorrhage.
- Complete surgical resection followed by two years of adjuvant polychemotherapy (Actinomycin D, Adriamycin, Vincristine, Cyclophosphamide) was administered.
- The patient achieved a 26-month tumor-free survival with near-normal somatic and psychic development.
Findings:
- The study reviews 25 published cases of vaginal endodermal sinus tumors in young girls (5-26 months).
- Histological patterns and clinical course of these pediatric vaginal tumors are detailed.
- Key differentiation is emphasized between infant endodermal sinus tumors and adult-onset clear-cell adenocarcinoma, which is linked to maternal stilbestrol exposure.
Implications:
- Early diagnosis and aggressive multimodal treatment (surgery and chemotherapy) can lead to favorable outcomes in infant endodermal sinus tumors.
- Distinguishing this rare pediatric neoplasm from other vaginal tumors is crucial for appropriate management.
- This case contributes to understanding the clinical behavior and treatment efficacy of endodermal sinus tumors in very young children.
Abstract:
An endodermal sinus tumor (endodermal germ cell carcinoma) was diagnosed in a 1-year-old girl in the vagina after hemorrhage; the tumor was completely removed by radical abdominal surgery. Postoperative polychemotherapy was performed for two years with Actinomycin D, Adriamycin, Vincristin, and Cyclophosphamide. The infant is now tumor-free for 26 months, showing almost normal somatic and psychic development. The characteristic histological patterns and clinical course of this strongly malignant tumor are demonstrated, based on 25 published case reports of endodermal sinus tumors in the vagina of little girls (aged 5-26 months). This neoplasm in early infancy has to be separated from the clear-cell adenocarcinoma of the vagina which occurs after puberty in adolescent girls and young women, and is induced by stilbestrol therapy to the mother during early pregnancy.