primary CNS paraganglioma; spinal cord paraganglioma

Introduction

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

Immunophenotype

• 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 +/-

More general terms

• paraganglioma