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This report details the first intrapericardial yolk sac tumor in a child. Despite treatment, the rare cardiac tumor led to fatal outcomes, highlighting diagnostic and therapeutic challenges.
Area of Science:
- Pediatric Oncology
- Cardiovascular Pathology
- Surgical Oncology
Background:
- Yolk sac tumors (endodermal sinus tumors) are rare germ cell tumors.
- Intrapericardial location of yolk sac tumors is exceptionally uncommon.
- Early diagnosis and treatment are crucial for pediatric oncology patients.
Observation:
- A 14-month-old girl presented with cardiac failure symptoms, including a systolic murmur and cardiomegaly.
- Intraoperative findings revealed a large, friable intrapericardial mass.
- No extrapericardial spread of the tumor was detected.
Findings:
- Histologic and immunohistochemical analyses confirmed the diagnosis of yolk sac tumor.
- Elevated serum alpha-fetoprotein levels correlated with tumor progression.
- Despite surgical resection and chemotherapy, the patient succumbed to the disease 24 months post-diagnosis.
Implications:
- This case highlights the rarity and aggressive nature of intrapericardial yolk sac tumors in pediatric patients.
- Effective management strategies for this rare cardiac malignancy require further investigation.
- The association with alpha-fetoprotein underscores its utility as a tumor marker in diagnosis and monitoring.
Abstract:
This article is a report on the first known case of an intrapericardial yolk sac (endodermal sinus) tumor discovered in a 14-month-old girl. The patient presented with a systolic murmur, cardiomegaly, and manifestations of cardiac failure. At surgery a large, friable, intrapericardial mass with no evidence of extrapericardial spread was found. Histologic and immunohistochemical findings were diagnostic of yolk sac tumor. Following initial resection, chemotherapy, and a second resection 13 months after the first, the patient died 24 months after diagnosis. Tumor enlargement was associated with elevated serum alpha-fetoprotein levels.