Related Experiment Videos
Summary
A rare pediatric orbital tumor, alveolar soft-part sarcoma, was diagnosed in an infant using microscopy and electron imaging. The patient achieved a favorable outcome following surgical resection and radiation therapy.
Area of Science:
- Oncology
- Ophthalmology
- Pediatric Pathology
Background:
- Alveolar soft-part sarcoma (ASPS) is a rare soft tissue malignancy that predominantly affects adolescents and young adults.
- Orbital involvement of ASPS is exceptionally uncommon, particularly in infants, presenting diagnostic challenges.
Observation:
- An 11-month-old infant presented with proptosis and limited ocular motility, indicative of an orbital mass.
- CT imaging revealed a soft-tissue mass within the orbit.
- Histopathological examination demonstrated characteristic polygonal cells with granular cytoplasm, periodic acid-Schiff (PAS)-positive granules, and unique 100 nm needle-shaped crystals.
Findings:
- Electron microscopy confirmed the crystalline structures as diagnostic features of alveolar soft-part sarcoma.
- These cytoplasmic crystals were identified as acid phosphatase-positive, suggesting lysosomal system involvement.
- The tumor cells exhibited uniform nuclei with prominent nucleoli and rare mitoses.
Implications:
- This case highlights the importance of advanced microscopy in diagnosing rare pediatric orbital tumors.
- Early diagnosis and multimodal treatment, including surgery and radiation, can lead to favorable outcomes in pediatric ASPS.
- Understanding the ultrastructural features of ASPS aids in accurate diagnosis and classification.