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Basimeningeal/spinal axis primitive neuroectodermal tumor without intraaxial lesion: radiological features
E W Lazzara1, R L Ragland, T W Smith
1Department of Radiology, University of Massachusetts Medical Center, Worcester, USA.
Summary
This study reports two rare cases of primitive neuroectodermal tumors (PNET) affecting the spinal axis and basimeninges without intraaxial involvement. These findings highlight an unusual PNET presentation in the central nervous system.
Area of Science:
- Neuro-oncology
- Spinal cord oncology
- Pediatric neurosurgery
Background:
- Primitive neuroectodermal tumors (PNETs) are rare malignant neoplasms.
- Intraaxial PNETs within the brain and spinal cord are recognized entities.
- Extramedullary PNETs, particularly involving the spinal meninges, are exceptionally uncommon.
Observation:
- Two patients, a 48-year-old woman and a 10-year-old boy, presented with PNET.
- Imaging revealed hydrocephalus, abnormal basimeningeal enhancement, and spinal axis intradural, extramedullary compartment involvement.
- No intraaxial tumor focus was identified in either case.
Findings:
- The patients exhibited a unique pattern of PNET infiltration.
- Tumor involvement was confined to the spinal meninges and basimeninges.
- This presentation is distinct from typical intraaxial PNETs.
Implications:
- This case series expands the understanding of PNET presentation.
- It underscores the importance of considering extramedullary PNETs in the differential diagnosis of spinal and basimeningeal lesions.
- Further research is needed to elucidate the pathogenesis and optimal management of these rare tumors.