Haga su diagnóstico

Abstract

A 30-year-old woman developed a slowly progressive reddish-violet multinodular rubbery lesion, measuring 20 x 10 cm in diameter, on the upper outer quadrant of her right breast four years after the surgical excision of a dermatofibrosarcoma protuberans (DFSP) in the same area. The diagnosis of DFSP was confirmed histologically and by positive immunomarkers at immunohistochemistry. A complete excision was performed and after a 2-year follow-up no recurrence was observed. It should be emphasized that DFSP of the breast is a rare occurrence.

Dermatofibrosarcoma Protuberans: A Case Report

“…It consists of a sarcoma of cutaneous origin corresponding to less than 0.1% of all malignancies and to approximately 1% of all soft tissue sarcomas, with an annual incidence of 0.8–4.5 cases per million individuals [2, 3, 4, 5, 6]. Its growth is indolent, with a high recurrence rate due to its strong capacity to infiltrate subcutaneous tissue, fascia and underlying muscle (infiltration in the form of pseudopods) [6, 7]. …”

“…Asymptomatic multinodular bluish or brownish erythematous plate, developing over years, with its typical ‘protuberant’ aspect, is the most frequently observed clinical aspect [1, 6]. It can also present as an atrophic plaque, resembling morphea, and is often misdiagnosed as such [8, 9].…”

“…In general, the tumor is located in the dermis, but it can sometimes present infiltrative growth in the subcutaneous fatty tissue, forming a pastry pattern (60% of cases; neoplastic cell bands parallel to the epidermis) or a honeycomb pattern (delimitation of adipocyte islets between the tumoral tissue) [3, 6]. Its low potential for metastasis is typical, with less than 5% probability for regional or distant metastases, with these often being restricted to the lungs and less frequently to the lymph nodes [5, 14, 15].…”

“…Our patient had this pattern. Stromelysin-3 is negative (dermatofibroma marker), as is CD117 [1, 6, 16, 17]. …”