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Primary intramedullary spinal cord primitive neuroectodermal tumor with intracranial seeding in an infant
1Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Yongon-dong, Chongno-gu, Korea.
Summary
Primary spinal cord primitive neuroectodermal tumors (PNETs) are rare, especially in infants. This case highlights the aggressive nature and rapid progression of spinal PNETs in infants, even with initial treatment response.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
- Oncology
Background:
- Primary spinal cord primitive neuroectodermal tumors (PNETs) are exceptionally rare pediatric neoplasms.
- Literature review indicates only 13 reported cases, with a few instances of intracranial seeding.
Observation:
- A 3-month-old infant presented with a spinal cord PNET below the mid-thoracic level.
- Brain MRI confirmed intracranial subarachnoid seeding.
- The infant underwent biopsy, duraplasty, and laminotomy flap removal.
Findings:
- The patient showed initial positive response to a postoperative chemotherapy regimen.
- Treatment was subsequently refused, leading to rapid disease progression.
- The infant succumbed to the illness 21 days after symptom onset.
Implications:
- This case underscores the aggressive behavior and rapid progression of spinal cord PNETs in infants when left untreated or incompletely managed.
- It emphasizes the critical need for timely diagnosis and comprehensive treatment strategies for this rare pediatric malignancy.
- Highlights the challenges in managing infant PNETs due to their rarity and aggressive nature.