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Primary orbital yolk sac tumor in an infant: A rare entity
Varsha Mishra1, Sandeep Jain1, Payal Malhotra1
1Department of Pediatric Hematology and Oncology, Rajiv Gandhi Cancer Institute and Research Centre, New Delhi, India.
Insights
This case study highlights a rare pure orbital yolk sac tumor (YST) in an infant presenting with proptosis. Prompt diagnosis and chemotherapy led to a positive response, emphasizing early intervention for orbital tumors.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Pathology
Background:
- Orbital yolk sac tumors (YST) are rare germ cell tumors, particularly in infants.
- Orbital proptosis in young children warrants a high index of suspicion for malignancy.
Observation:
- An 11-month-old infant presented with painless, progressive right eye swelling, proptosis, chemosis, and lid edema.
- MRI revealed a retroocular mass with orbital wall erosion and optic nerve displacement.
- Histopathology confirmed pure YST, with markedly elevated serum alpha-fetoprotein (AFP) levels.
Findings:
- The patient received infant bleomycin, etoposide, and cisplatin (BEP) chemotherapy.
- Significant clinical and radiological improvement was observed post-chemotherapy.
Implications:
- Early diagnosis and a multidisciplinary approach are crucial for managing orbital YST.
- Timely intervention can preserve vision and improve patient outcomes in pediatric orbital malignancies.
Abstract:
We report a case of pure orbital yolk sac tumor (YST) in an 11-month-old infant, which is a rare entity. The child presented with progressive painless swelling of the right eye and on examination had proptosis, chemosis, and lid edema. Systemic examination was within normal limits. Magnetic resonance imaging (MRI) orbit revealed a lobulated heterogeneously enhancing right retroocular mass extending up to the orbital apex, displacing the optic nerve and eroding the medial orbital wall. Biopsy of the lesion revealed pure YST histology. Serum alpha-fetoprotein (AFP) was markedly raised at 76900 ng/mL. She was started on infant bleomycin etoposide cisplatin (BEP) chemotherapy protocol. There was a good clinical and radiological response. A high index of malignancy is required in young children presenting with orbital proptosis. A multidisciplinary approach and early intervention are essential to save both vision and life.
