Primary cutaneous ganglioneuroma is a rare neoplasm first described by Collins et al. in 1972. Eight cases have been reported in the English literature to date. We report two cases of solitary cutaneous ganglioneuroma, one constituting, to our knowledge, the first reported case of this entity occurring on the face. Clinically, both lesions were firm flesh-colored papules and asymptomatic, being removed primarily for cosmesis. Neither patient had any significant past medical history. Histologically, both neoplasms consisted of relatively well-circumscribed spindle cell proliferations of axons and Schwann cells with interspersed mature ganglion cells. There was also focal myxoid change. The spindle cell component stained positively for S-100 protein, and the ganglion cells stained positively for glial fibrillary acidic protein and neurofilaments by routine immunohistochemistry.
Primary ganglioneuromas are distinctive benign neoplasms that rarely affect the skin. Eight cases of primary ganglion cell tumors of the skin have been reported in the English literature, five of which are ganglioneuromas (1–8). Two cases of ganglion cell choristoma and a single case where the ganglion cells and neuromatous cells were separate but adjacent have also been reported (6–8).
We report two additional cases of primary ganglioneuroma, including the first to occur on the face. Furthermore, we review the literature on ganglion cell tumors of the skin and discuss the differential diagnosis of these tumors.