A 78-year-old woman presented with a one-year history of a painful skin tumor on her left forearm. Physical examination revealed a skin-colored, slightly elevated, smooth, elastic, and firm intradermal nodule, 4 mm in diameter. Histopathological examination revealed a well-demarcated nodular lesion in the lower dermis, which consisted of dense proliferation of spindle cells without nuclear atypia. Immunohistochemical staining showed that most nuclei and cytoplasm of the spindle-shaped tumor cells were positive for the S-100 protein, suggesting that the tumor cells originated from Schwann cells. Additionally, a subset of cytoplasm was positive for neurofilament, which appeared to be axons, and the surrounding capsule was positive for the epithelial membrane antigen (EMA). A diagnosis of palisaded encapsulated neuroma (PEN) was made based on these histopathological findings.
PEN, also called as a solitary circumscribed neuroma, is a benign, solitary tumor of neural tissue that occurs most commonly in the head and neck area in middle-aged or older individuals. The occurrence of PEN on the extremities is rare. Immunohistochemical staining is required to narrow the differential diagnosis from other neural tumors such as neurilemmoma, traumatic neuroma, and neurofibroma. Particularly, neurilemmoma is positive for the S-100 protein in tumor cells and EMA on the capsule, but negative for neurofilament because of the lack of axons. Traumatic neuroma is positive for the S-100 protein and neurofilament, but negative for EMA on the surroundings of the tumor. Neurofibroma occasionally has axons that may be partially positive to neurofilament; however, it is negative for EMA because of the lack of a capsule.
In conclusion, expression patterns of the S-100 protein, neurofilament, and EMA in PEN are characteristic features compared to those in other neural tumors. Thus, the use of immunohistochemical staining is feasible for the diagnosis of PEN.
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