Introduction

Neurofibromas are soft, skin-colored, pink or tannish nodules that resemble intradermal nevi.

Epidemiology

They are most likely to appear in adolescence.

Etiology

Neurofibroma is a benign nerve sheath tumor in the peripheral nervous system. Two subtypes have been identified: dermal and plexiform. Dermal neurofibromas, which usually involve only one nerve, do not have malignant potential, while plexiform types do. Analysis of the ultrastructure of neurofibromas shows the presence of three different types of cells: Schwann-like cells, perineurial-like cells, and fibroblast-like cells.¹

Symptoms and clinical features

Asymptomatic, but the patient may notice a “lump.”

When located in the vulvar area, neurofibromas are likely to be found in the periclitoral area or the labia majora. They may be soft or slightly firm to touch and may “invaginate” when touched gently. They are non-tender. Solitary nodules are benign and do not need treatment. Multiple nodules can be a sign of neurofibromatosis (Von Recklinghausen’s disease), a rare autosomal dominant condition that affects skin, nervous system, bones, and endocrine glands which is often associated with “café-au-lait” macules, as well.² This condition occurs in 1 in 3,000 to 4,000 people.

Diagnosis

Usually made clinically. Excision can be carried out.

Pathology/Laboratory Findings

Histologically, the lesions contain the elements of peripheral nerve (Schwann cells, neurons, and fibroblasts) in a disorganized pattern.³

Treatment/management

None needed for solitary, asymptomatic lesions. Excision for larger, bothersome lesions. Laser is used in more extensive disease elsewhere on the skin.

1. Sanguinetti C, Greco F, de Palma L, Specchia N, Toesca A, Nori S. The ultrastructure of schwannoma and neurofibroma of the peripheral nerves. Ital J Orthop Traumatol. 1991 Jun;17(2):237-246.
2. Wolff K, Johnson RA. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology, sixth edition, 2009. McGraw Hill Medical. 453.
3. Heller DS, Wallach RC, ed. Vulvar disease: a clinicopathological approach. Informa Healthcare, 2007. 157.