Synonyms
Definition
A benign, well-circumscribed intraneural proliferation of Schwann cells and axons.
Clinical Features
Incidence
Not rare but probably underdiagnosed.
Age
Middle-aged adults.
Sex
Equal gender distribution.
Site
There is a strong predilection for the skin of the central face and the mucocutaneous junction, but the oral mucosa can also be affected (Koutlas, Scheithauer, 2010, Leblebici et al., 2019). Acral skin and penis are rare locations.
Treatment
Simple excision is curative.
Outcome
This tumor is entirely benign. There is no association with NF1 or MEN2b.
Macroscopy
Small superficial nodule.
Microscopy
Circumscribed neuroma typically presents as a well-circumscribed cellular nodule composed of short fascicles of typical Schwann cells (Fig. 1). Nuclear palisading is usually not prominent, and hyaline vessels are lacking. Encapsulation is at least focal, but the tumor cells can merge with the surrounding tissue in the superficial part....
References
Koutlas, I. G., & Scheithauer, B. W. (2010). Palisaded encapsulated (“solitary circumscribed”) neuroma of the oral cavity: A review of 55 cases. Head and Neck Pathology, 4, 15–26.
Leblebici, C., Savli, T. C., Yeni, B., Cin, M., & AEK, A. (2019). Palisaded encapsulated (solitary circumscribed) neuroma: A review of 30 cases. International Journal of Surgical Pathology, 27, 506–514. https://doi.org/10.1177/1066896919833172.
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Sciot, R. (2020). Solitary Circumscribed Neuroma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5439-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5439-1
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