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primary CNS paraganglioma; spinal cord paraganglioma
filum terminale paragangliomas correspond to WHO grade I.
Epidemiology:
- ~120 cases reported as of 2000
- generally occur in adults (age range 13 - 71 years)
- most present as intradural tumors in the cauda equina;
- other spinal levels less often affected
- rare examples of purely intracranial paragangliomas
Microscopic Pathology:
- resemble normal paraganglia
- chief cells (type I) in nests or lobules (zellballen) surrounded by inconspicuous layer of sustentacular (type II) cells
- may contain mature ganglion cells, cells transitional between chief and glanglion cells,
- may contain melanin pigment
- may have spindle cell component
Immunohistochemistry:
- neuron specific enolase +
- synaptophysin +
- chromogranin +
- S100 protein +/variable
- leu-enkephalin 47%+
- somatostatin 34%+
- GFAP, focal 30%+
- keratin, focal 21%+
- neurofilament proteins 13%+, marker of chief cells
- serotonin, somatostatin, met-enkephalin, ACTH variable +/-
General
paraganglioma
References
- WHO Classification Tumours of the Nervous System.
Kleihues & Cavenee eds. IARC Press 2000
- Moran et al. Histopathology 31:167-73, 1997