Dermatofibrosarcoma Protuberans on Adult Toes: A Case Report and Review of the Literature.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-grade, dermal soft-tissue neoplasm with high recurrence, but low metastatic potential. It mainly occurs on the trunk, proximal extremity, head and neck, but rarely on the toes. Herein we report a case of DFSP on the right hallux. CASE REPORT: A 39-year-old male presented with a mass in the right great toe of 3.5 years. After surgical excision, histopathological evaluation of the mass showed elongated monomorphic spindle cells arranged in a storiform pattern. The tumor cells infiltrated into adjacent adipose tissue in a honeycomb formation. Immunostaining for CD34 showed diffuse and strong cytoplasmic expression in neoplastic cells, whereas that for alpha smooth muscle actin, factor XIIIa, S-100 and melan-A were negative. The tumor was diagnosed as DFSP. We further reviewed the literature of DFSP on the toes with the aim to reveal, for the first time as far as we are aware, its clinical presentations, histopathology, differential diagnosis and treatment options. CONCLUSION: DFSP on adult toes is a rare neoplasm characterized by monomorphic spindle cells arranged in storiform pattern, and can be treated with partial or total toe amputation, or wide local excision after primary excision, with excellent prognosis.