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Five-Year-Old Boy With Behavioral Changes and Papilledema
Radha Ram1, Jeremy Y Jones, Carrie A Mohila
1Departments of Ophthalmology (RR, VSS), Radiology (JYJ), and Pathology (CAM), Baylor College of Medicine, Texas Children's Hospital, Houston, Texas.
Insights
A rare malignant peripheral nerve sheath tumor was diagnosed in a 5-year-old boy presenting with neurological and vision symptoms. Temporal lobe biopsy confirmed the diagnosis after initial tests were inconclusive.
Area of Science:
- Neuro-oncology
- Pediatric Neurology
- Pathology
Background:
- Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive neoplasms.
- Pediatric MPNSTs are exceptionally uncommon, posing diagnostic challenges.
- Early symptoms can be nonspecific, delaying diagnosis.
Abstract:
A 5-year-old boy had initial symptoms of behavioral changes, nausea, vomiting, headache, weight loss, and progressive vision failure. Brain MRI revealed abnormal signal intensity in both optic nerves, the optic chiasm, the right medial temporal lobe, and tissues surrounding the right supraclinoid internal carotid artery with associated leptomeningeal and spinal cord enhancement. After nondiagnostic dural and spinal arachnoid biopsies, a temporal lobe biopsy was diagnostic for a rare malignant peripheral nerve sheath tumor.
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