A Myxoid Neurothekeoma of the Nose
February 2012 | Volume 11 | Issue 2 | Case Report | 252 | Copyright © 2012
Neurothekeoma is a rare neoplasm assigned to the broad category of benign peripheral nerve sheath tumors. Its cell of origin is still unknown, but most ultrastructural and immunohistochemical studies have favoured the Schwann cell perineurium or fibroblast. Neurothekeoma most commonly presents in females, especially in the second and third decades of life. Typically it presents as an asymptomatic solitary, firm, flesh-coloured or hyperpigmented slow-growing papule or nodule that involves the skin and superficial subcutis. It usually involves the face, neck, arm or shoulder but can also involve extracutaneous sites. Neurothekeoma occurs in myxoid (classic), cellular and intermediate (mixed) variants. Here, we report a 45-year-old male patient presenting with a nodule on the nose that was found to be a myxoid variant of neurothekeoma. It was treated with a simple shave excision followed by removal of the lobulated deeper part by forceps. He had no recurrence after tthree years of follow-up. To the best of our knowledge, this is the first case of a myxoid neurothekeoma of the nose to be reported in the Middle East.
J Drugs Dermatol. 2012;11(2):252-255.
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N eurothekeomas are distinctive benign cutaneous tumors of uncertain histogenesis.1 Histologically, neu- rothekeomas contain proliferating cells arranged in lobules or fascicles immersed in an amorphous matrix in the dermis and rarely in subcutaneous tissues.2 Neurothekeomas were originally described in 1969 by Harkin and Reed, who used the name “nerve sheath myxoma.”3 The term “neurothekeoma” was first coined by Gallager and Helwig in 1980 in a study of distinct benign cutaneous tumors that arose predominantly in children and adolescents.4 Most of the lesions reported in those cases were identical to the “nerve sheath myxomas ” previously described by Harkin and Reed,5 which occur in myxoid (classic), cellular and intermediate (mixed) variants.
A 45-year-old Saudi male patient without any known chronic medical illness presented with a nodule on the side wall of the nose. It started four years ago as an asymptomatic nodule which progressively increased in size during the first year with no prior trauma. There was no family history of a similar complaint or of skin cancer. Drug and allergy histories were unremarkable.
Physical examination revealed a solitary, flat, fir m an d red nodule with a diameter of 8 mm on the side wall of the nose (Figure 1). There was no lymph node enlargement. The patient underwent shave diagnostic excision under local anesthesia. During the excision, it was observed that the deeper part was coming off easily as lobules; therefore, the entire lesion was removed by a toothed forceps. No safety margin was included. The resultant 8 mm diameter defect was closed with a simple interrupted suture.
Skin histopathology of the lesion showed a dermal lesion consisting of spindle cells with myxoid material in the background. The cells had thin elongated benign-appearing nuclei arranged in lobules and surrounded by fibrous tissue (Figure 2). Immunohistochemistry showed positivity for S-100 while smooth muscle actin and desmin were negative. Based on biopsy and immunohistochemistry, the diagnosis of a myxoid variant of neurothekeoma was made. The patient had no recurrence after three years of follow-up.
The origin of neurothekeoma is unknown. Some authors consider Schwann cells to be its origin, while others believe that perineural tissues or supporting structures of peripheral nerves, fibroblasts and muscle could be responsible.6 Neurothekeoma has even been considered a variant of the following dermatoses: dermatofibroma, pilar epithelioid leiomyoma, plexiform neurofibroma and nerve sheath myxoma.7