Atypical cellular neurothekeoma treated with Mohs micrographic surgery

Atypical cellular neurothekeoma is a rare neoplasm generally regarded as a benign tumor with locally aggressive behavior. Recurrence is common with inadequate excision, but metastatic disease has yet to be reported.

The objective was to report a case of atypical cellular neurothekeoma of the nasal ala in a young woman treated by Mohs micrographic surgery. The biologic behavior and histopathology will be reviewed.

Case report and review of the literature.

The neoplasm was extirpated in a three-stage, five section Mohs surgery procedure. The resulting defect was repaired by a two-stage, pedicled nasolabial transposition flap with a cartilage graft taken from the ipsilateral ear. No clinical recurrence was noted after 6-months of follow-up.

Mohs micrographic surgery is unsurpassed in its efficacy in treating a wide variety of nonmelanoma skin cancers. Although most commonly used to address basal and squamous cell carcinoma, it has also been reported as a successful treatment for melanoma and a wide variety of cutaneous malignancies. We discuss a case of atypical cellular neurothekeoma removed with the Mohs technique. Debate in the literature is ongoing regarding the true histogenesis of this rare tumor. Because of this tumor's local destructive behavior and propensity to recur with inadequate resection, we recommend Moths micrographic surgery for the treatment of cellular neurothekeomas.