• Strong S100(+) in Schwann cells and nerve fibers
• Negative for SMA, desmin, melanocytic markers
Top Differential Diagnoses
• Benign nerve sheath tumors
• Superficial (pilar) leiomyoma
• Dermatofibroma
Solitary Circumscribed Neuroma Solitary circumscribed neuroma [“palisaded encapsulated neuroma” (PEN)] represents a well-circumscribed, partially encapsulated, dermal-based neural neoplasm. Note the absence of overlying epidermal hyperplasia.
PEN With Characteristic Clefts Solitary circumscribed neuroma is a circumscribed, sometimes partly encapsulated dermal lesion composed of Schwann cells and associated axons. Characteristic clefts and a small, preexisting nerve at the base of the lesion can be seen.
Bland Spindled Schwann Cells in PEN The neoplastic Schwann cells contain ill-defined, pale, eosinophilic cytoplasm and cytologically bland spindled nuclei with evenly distributed chromatin. No prominent cytologic atypia and no increased mitoses are present.
PEN Tumor Cells Positive for S100 Solitary circumscribed neuroma is composed mainly of S100-positive Schwann cells. Preexisting peripheral nerves can often be seen at the base of these lesions.
TERMINOLOGY
Definitions
• Proliferation of peripheral nerve fibers in which ratio of axons to Schwann cell fascicles is in nearly equal proportions
Solitary circumscribed neuroma [“palisaded encapsulated neuroma” (PEN)] is spontaneous proliferation of peripheral nerve fibers