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Endodermal sinus (yolk sac) tumor in infants and children. A clinical and pathologic study: an 11 year review
S Shebib1, R S Sabbah, K Sackey
1Department of Oncology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Insights
This study found extragonadal yolk sac tumors are more common in children than previously thought. Early diagnosis and varied chemotherapy offer hope for survival in these rare pediatric cancers.
Area of Science:
- Pediatric Oncology
- Tumor Biology
Background:
- Yolk sac tumors (endodermal sinus tumors) are rare germ cell neoplasms.
- This study reviews cases from a single center over a decade.
Observation:
- Nine pediatric patients (7 girls, 2 boys) were treated for yolk sac tumors.
- Tumors originated in the vagina, face, sacrum, mediastinum, ovary, and testicle.
- Elevated alpha-fetoprotein (AFP) was noted in all patients.
Findings:
- Extragonadal yolk sac tumors showed an unusual preponderance in this cohort.
- Treatment involved surgery (excision or biopsy) and multi-agent chemotherapy.
- Three of nine patients achieved long-term disease-free survival.
Implications:
- Highlights the need for awareness of extragonadal yolk sac tumors in pediatric oncology.
- Suggests aggressive multimodal therapy may improve outcomes.
- Further research is warranted to optimize treatment strategies for these rare tumors.
Abstract:
We reviewed the clinical features, treatment, and results of children with gonadal and extragonadal yolk sac (endodermal sinus) tumors seen in the King Faisal Specialist Hospital and Research Centre between 1976 and 1987. There were nine children (seven girls and two boys) with ages ranging from 7 months to 12 years (median of 3.5 years). Sites of origin included the vagina (two cases), face (two cases), sacrum (two cases), mediastinum (one case), ovary (one case), and testicle (1 case). All children had elevated alpha-fetoprotein (AFP) at diagnosis. One girl had complete surgical excision of an ovarian tumor at the time of diagnosis, and one boy had surgical excision of the testis. In the remaining seven children, the tumor was unresectable. Surgery was limited to a biopsy in six children. All patients received different combinations of chemotherapy, including vincristine (VCR), actinomycin D (Act-D), cyclophosphamide (Cyclo), adriamycin (Adria), bleomycin (Bleo), cis-platinum (CDDP), vinblastine (VBL), and VP-16. Of the nine patients, one was lost to follow-up while in remission, five died, one was lost to follow-up, and three are alive and disease-free at 15, 55, and 67 months from diagnosis. This review demonstrates an unusual preponderance of the extragonadal form of endodermal sinus tumor among our patients.