The unusual occurrence of a glomus tumor within a small, deep dermal n
erve is reported. This phenomenon has been described previously only o
nce in the literature. The lesion appeared as a painful cutaneous nodu
le in the shoulder of a 67-year-old woman. Microscopically, a typical
solid, smooth muscle actin-positive glomus tumor was seen, encircled c
ompletely by neural tissue in the deep dermis. There was no extension
of the tumor beyond the nerve. Immunohistochemically, the nerve fibers
surrounding and within the tumor were positive for S-100 protein and
neurofilament. The presence of perineurium surrounding the glomus tumo
r was confirmed by a layer of epithelial membrane antigen-positive cel
ls. In view of the absence of normal glomus bodies in the nerves, an o
rigin from a less specialized perivascular cell is proposed.