Dermatofibrosarcoma protuberans

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue neoplasm of intermediate malignancy that is initially localized to the skin from where it can invade deep structures (fat, fascia, muscle and bone). It is the most frequent fibrohistiocytic tumor, comprising approximately 1.8 % of all soft tissue sarcomas and 0.1 % of all cancers. It has an estimated incidence of 0.8-5 cases per one million persons per year. Treatment of localized disease consists in complete surgical excision of the lesion by conventional surgery with wide margins (>3 cm) or by micrographic Mohs surgery. Although the cases of metastatic DFSP do not reach 5 % of the total, almost all of them appear after previous local relapses. The prognosis for metastatic cases is very poor with a survival of less than 2 years following detection of metastatic disease. Patients with locally advanced DFSP are not candidates for an initial radical surgical therapy therefore neoadyuvant treatment is required prior to surgery in order to reduce tumor burden. In this regard, chemotherapy and radiotherapy have not been highly efficacious so it is necessary to consider new alternatives. The demonstration of the oncogenic power of the translocation COL1A1-PDGFB in DFSP has allowed the successful introduction of drug therapy with antagonists of the PDGFB receptor for metastatic or locally advanced cases.

Pediatric Dermatofibrosarcoma Protuberans in Madrid, Spain: Multi‐institutional Outcomes

“…The distribution of skin lesions in children is similar to that found in adults, with the majority of lesions affecting the trunk, proximal extremities, and head and neck (2)(3)(4)12,20,30), even in congenital cases (11,15). Unlike in adults, acral locations are frequently affected in pediatric DFSP (9-15%) (10,31,32).…”

“…DFSP is uncommon, accounting for approximately 0.1% of all malignancies in adults (2,3) and 1% of all soft tissue sarcomas (4,5). Its overall annual incidence has been reported to range from 0.8 to 4.5 per 1,000,000 population per year (6,7), an incidence in the pediatric population estimated at 1 per 1,000,000 (8).…”

Dermatofibrosarcoma protuberans: clinical, pathological, and genetic (COL1A1‐PDGFB ) study with therapeutic implications

“…Histology shows a poorly delimited dermal tumour containing a dense proliferation of uniform spindle cells with limited pleomorphism and a low mitotic index 5,6 . On occasions, diagnosis proves difficult and the use of techniques such as immunohistochemistry or molecular biology is necessary.…”

Dermatofibrosarcoma protuberans sobre cicatriz de apendicectomía

“…El tratamiento de elecció n es la exé resis quirú rgica mediante la cirugía de Mohs y, en caso de no poder realizarse, la resecció n con má rgenes amplios (> 3 cm). Un hecho a recalcar es que aproximadamente el 30-50% de las lesiones extirpadas recidivan localmente 1,3 . En tumores inoperables o metá stasis se usa el imatinib como tratamiento paliativo o neoadyuvante 2 .…”