Capillary hemangioblastoma (HB) is a benign, highly vascular tumor lim
ited almost exclusively to the central nervous system (CNS). It occurs
primarily in the posterior fossa and less often in the spinal cord. W
e report three cases of HE occurring in peripheral nerve, two intradur
al tumors arising in a C4 and a cauda equina nerve root, respectively,
and a third lesion in the sciatic nerve at mid thigh. The patients, 1
woman and 2 men, ranged in age from 25 to 49 years, Two had von Hippe
l-Lindau disease, an association usually found in one-third of CNS HBs
, and one had a family history of pheochromocytoma. In every way, HBs
of peripheral nerve were indistinguishable from their CNS counterpart.
Ranging in size from 1.5 to 5.5 cm in diameter, the tumors were well
circumscribed and contained a myriad of small caliber vessels lined by
endothelial cells and surrounded by pericytes. Throughout, the lesion
s were rich in large, often vacuolated stromal cells. In all of the ca
ses, these stained strongly for vimentin and neuron-specific enolase;
only one showed focal S100 protein reactivity. Surgical therapy requir
ed excision of the affected nerve roots in the first two cases. In the
third case, prominent extension of the tumor within epineurium permit
ted a microsurgical resection with sparing of sciatic nerve fascicles.
No tumor recurred during a follow-up period of 5 to 20 months.