Prominent myofibroblastic differentiation. A pitfall in the diagnosis of dermatofibroma

Myofibroblastic differentiation occurs in 10-20% of all dermatofibromas, affecting < 25% of cells. We report on a series of 36 dermatofibromas collected from > 1,500 fibrohistiocytic lesions (2%), with more prominent (> 25%) myofibroblastic differentiation characterized by markedly slender and elongated spindle cells positive for smooth muscle markers. While most of the lesions did not otherwise differ from ordinary dermatofibromas, three cases (0.2%) from the neck-shoulder region of male adults showed extensive myofibroblastic features (> 90%). Clinically, these three lesions measured approximately 1 cm and had a firm consistency, with the differential diagnosis of some fibrohistiocytic tissue response. Histologically, densely packed cells and prominent, partially nodular, stromal sclerosis with focal palisading of nuclei indicate some overlap with other rare variants of fibrohistiocytic tissue response, such as cellular benign and palisading cutaneous fibrous histiocytoma. Yet, these features together with focal whorled nesting of more epithelioid cells (one case) also caused considerable diagnostic problems to exclude other myofibroblastic as well as (malignant) spindle cell lesions such as (palisaded) myofibroblastoma, dermatofibrosarcoma protuberans, and neurothekeoma. Immunohistochemically, all lesions were markedly (> 90%) labeled for smooth muscle markers (HHF35, anti-SMA) and with NK1C3 (CD57), while a broad panel for other spindle cell tumors, such as pan-keratin, S100 protein, EMA, desmin, CD34, CD31, and KiM1p, were negative. Electron microscopy of two cases revealed prominent endoplasmic reticulum and Golgi complex, numerous intermediate filaments, attachment plaques, and focal basal lamina formation. No recurrence was seen during a follow-up of 9 (two cases) and two years, respectively.