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Prenatal intramedullary gliofibroma. A light and electron microscope study
Insights
This report details a rare congenital spinal cord tumor, a gliofibroma, found in an infant. The tumor, composed of astrocytes and fibroblasts, showed no signs of malignancy.
Area of Science:
- Neurology
- Oncology
- Pediatric Pathology
Background:
- Congenital intramedullary spinal cord tumors are rare pediatric conditions.
- Early diagnosis and characterization are crucial for understanding disease progression and treatment.
Observation:
- An 11-day-old paraplegic infant presented with hypotonia and muscle atrophy.
- Myelography revealed a complete block between T-5 and T-8, indicating spinal cord compression.
- Surgical exploration identified an elongated intramedullary tumor mass seamlessly integrated with neural tissue.
Findings:
- Histopathological analysis revealed a tumor composed of intermingled, well-differentiated astrocytes and fibroblasts.
- Electron microscopy showed these cells were often enveloped by a shared basal lamina, with absent intercellular junctions.
- Abundant gliofibrils and interstitial collagen/reticulin fibers were noted, with no histological evidence of malignancy.
- The tumor was classified as a gliofibroma, likely arising prenatally.
Implications:
- This case expands the understanding of rare congenital spinal cord tumors.
- The findings contribute to the histopathological classification of glial and mesenchymal-derived tumors.
- Further research into the embryogenesis and behavior of such congenital tumors is warranted.
Abstract:
A case of an unusual congenital intramedullary tumor of the spinal cord is reported. A paraplegic 11-day-old boy with hypotonia and atrophy of the abdominal and lower-extremity muscles showed a complete myelographic block between T-5 and T-8. Surgical exploration disclosed an elongated tumor mass within the spinal cord, that blended with the surrounding nervous-system tissue. Light and electron microscopy showed that the tumor was composed of intermingled well differentiated astrocytes and fibroblasts. These two cell types often were surrounded by the same basal lamina. There were no intercellular junctions. Gliofibrils were abundant, and the interstitial spaces contained abundant collagen and reticulin fibers. There were no histological signs of malignancy. We conclude that this is a case of prenatally arising gliofibroma.