Palisaded Encapsulated (Solitary Circumscribed) Neuroma: A Review of 30 Cases

Abstract

Background. Solitary circumscribed neuroma (SCN), also known as palisaded encapsulated neuroma (PEN), is a benign neural tumor. It may be mistaken as either schwannoma or neurofibroma in pathology practice. In this study, we aimed to define clinicopathologic and immunohistochemical features and discuss its differential diagnosis. Materials and Methods. The histopathological features of 30 cases of SCN/PEN were reviewed. The presence of intralesional axons, Schwann, and perineural cell distributions were investigated by performing neuronal immunomarkers. Results. Twelve cases were females, and 18 cases were males. The mean age was 48 years. Lesions were mostly located on the face (27/30). Histologically, 18 had a lobular pattern, 9 were plexiform, 2 fungating, and 1 multilobular. Although the majority of cases were well circumscribed, capsular integrity was at least focally disrupted (73%). Verocay body was noted only in 6 cases (20%). One case showed excessive hyperkeratosis, forming a keratin horn. Adipocytic change was detected in another case. The lesions consisted of S100-positive Schwann cell proliferation and were partially surrounded by perineural cells highlighted by EMA or Claudin-1. The amount of intralesional axons revealed by neurofilament immunostaining was variable. Conclusion. SCN/PEN is a relatively common lesion, and usually seen as an asymptomatic papule on the face of elderly patients. A circumscribed lesion composed of bundles of bland-looking spindle cells thought to be of neural origin is seen in the dermis. Pathologists should be aware of the existence of plexiform and multilobular PEN/SCN variants, to avoid misdiagnosis of plexiform neurofibroma or schwannoma.