|LETTER TO THE EDITOR
|Year : 2017 | Volume
| Issue : 1 | Page : 26-27
Solitary neurofibroma over cheek showing Wagner–Meissner bodies
Pragya A Nair, Rahul K Kota
Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat, India
|Date of Submission||11-Sep-2016|
|Date of Acceptance||17-Nov-2016|
|Date of Web Publication||2-Jun-2017|
Pragya A Nair
Department of Dermatology and Venereology, Pramukhswami Medical College, Karamsad, Gujarat - 388 325
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nair PA, Kota RK. Solitary neurofibroma over cheek showing Wagner–Meissner bodies. Egypt J Dermatol Venerol 2017;37:26-7
Neurofibroma (NF) is the designation for a group of common, closely related benign nerve sheath tumors. They typically present either as a localized lesion or as a part of a generalized syndrome of NF commonly known as neurofibromatosis type-1 (NF1), which is an autosomal dominant neurocutaneous disorder . Although NFs occur in people of all ages, they are commonly seen in young adults. The most common form is solitary cutaneous NF. Although solitary NF can occur in any region of the body, it is commonly found in the skin, deep nerves, and in association with other retroperitoneal tissues, but there are reports of solitary NF found over mucosal areas .
A 30-year-old woman attended skin Outpatient department (OPD) with complaints of an asymptomatic lesion over the right cheek, which was present and growing in size since 15 years. There were no similar complaints in the past or in the family. There were no other systemic complaints. On examination, single, skin-colored nodular lesion of size 1×1 was present on the right cheek ([Figure 1]). On examination, no other skin lesion was present elsewhere. Systemic examination was unremarkable. Ophthalmic examination was unremarkable. Clinically, we suspected it as acquired intradermal nevus, and, as the patient wanted it to be removed, excision biopsy was performed and sent for histopathology. The epidermis showed thinning, whereas the dermis showed diffuse proliferation of nerve bundles in tactile corpuscle-like pattern (Wagner–Meissner bodies). These tactile bodies are formed by the stacked lamellar arrangement of spindle cells extending into deep dermis ([Figure 2] and [Figure 3]). Histopathologically, diagnosis of extraneural (diffuse) NF was made.
|Figure 2: A microphotograph showing thinning of the epidermis and diffuse proliferation of nerve bundles in tactile corpuscle-like pattern (Wagner–Meissner bodies) in the dermis (H&E ×10).|
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|Figure 3: A microphotograph showing diffuse proliferation of nerve bundles in tactile corpuscle-like pattern (Wagner–Meissner bodies) in the dermis (H&E ×40).|
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Like any other benign lesion, NF presents with signs and symptoms depending on the site of the tumor and involvement of surrounding structures . Clinically, it manifests as asymptomatic cutaneous or mucosal nodules, with slow evolution and with three patterns of growth − namely, localized, diffuse, and plexiform . The localized form is the most common form and presents usually as skin-colored dome-shaped, or pedunculated papules that show the pathognomonic ‘buttonhole’ sign . They can be associated with NF or can be sporadic. Diffuse NFs are a distinctive but uncommon subtype of NF, which are usually solitary lesions and are progressively enlarging, infiltrating, simulating malignant tumors. In the head and neck region, the NF can be cutaneous, mucosal, or related to deep nervous bundle, related to facial, glossopharyngeal, vagus, or hyppoglossus nerves .
Typically, solitary NF appears during late childhood or during adolescence .
Histopathologically, the NF shows an irregular pattern with interlacing bundles of spindle-shaped cells with round or fusiform nuclei, and eosinophilic cytoplasm within a loose matrix of fine fibrillary collagen . These tumors are unencapsulated and comprise a mixture of Schwann cells, perineurial cells, and endoneurial fibroblasts . On the basis of their morphological features, they are classified into major and minor variants. Plexiform, diffuse, and pacinian NFs are the major variants, whereas minor variants include epithelioid, cellular, myxoid, glandular, xanthomatized, and other NFs .
Histopathological features of 114 NFs were reviewed by Megahed , who demonstrated 10 variants: classic, cellular, myxoid, hyalinized, epithelioid, plexiform, diffuse, pigmented, granular cell, and pacinian. The Wagner–Meissner corpuscles are the touch receptors of the skin situated in the dermal papillae, particularly on the palmar and the plantar surface. Whorled structures simulating these corpuscles may be seen in typical NFs, particularly in diffuse histological type. Although they are characteristic they are not always present . This particular type is often associated with NF1. However, in our case, NF1 was ruled out on the basis of family history and clinical examination. Immunohistochemistry is necessary to determine the presence of Schwann cell, perineurial cell, and fibroblast differentiation . It would be useful to perform immunoperoxidase staining with S-100 protein, Neuron specific enolase (NSE), and CD34. As the facility was not available at our institute, we did not perform.
Solitary NFs follow a more benign course, and cases of malignant transformation have so far not been reported. They are usually treated with surgical excision and exhibit very low recurrence . A prolonged and regular follow-up is necessary to study recurrence.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]