Diffuse leptomeningeal seeding from a malignant spinal cord astrocytoma in a child with neurofibromatosis.
A 5-year-old child typical clinical features of neurofibromatosis presented with a history of suspected basilar meningitis and CT findings of enlarged optic nerves and an expanding left cavernous sinus mass. CSF cytologies and meningeal biopsy were unremarkable. At craniotomy, a mass confluent with the left trigeminal nerve was resected which had histologic characteristics of a nerve sheath tumor but was GFAP (glial fibrillary acidic protein) stain positive. Postmortem examination, 1 month following surgical resection, demonstrated a clinically unsuspected primary thoracic spinal cord astrocytoma with dissemination throughout the subarachnoid space, invasion of the trigeminal nerve and encasement of other cranio-spinal nerves. This unusual case emphasizes the occurrence of leptomeningeal spread in a clinically silent spinal cord glioma and the diagnostic value of immunohistochemistry.
D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D005909:Glioblastoma / Q000473:pathology*; D006801:Humans; D008297:Male; D008578:Meninges / Q000473:pathology; D009361:Neoplasm Invasiveness; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010525:Peripheral Nerves / Q000473:pathology; D010524:Peripheral Nervous System Neoplasms / Q000556:secondary*; D013120:Spinal Cord Neoplasms / Q000473:pathology*