Journal Information
Vol. 98. Issue 2.
Pages 77-87 (March 2007)
Vol. 98. Issue 2.
Pages 77-87 (March 2007)
Review article
DOI: 10.1016/S1578-2190(07)70400-6
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Dermatofibrosarcoma Protuberans
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O. Sanmartín
Corresponding author
osanmartinj@gmail.com

Correspondence: Servicio de Dermatología, Instituto Valenciano de Oncología, Profesor Beltrán Báguena 8, 46009 Valencia, Spain.
, B. Llombart, J.A. López-Guerrero, C. Serra, C. Requena, C. Guillén
Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, Spain
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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 (> 3cm) 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.

Key words:
dermatofibrosarcoma protuberans
Mohs surgery
COL1A1-PDGFB
imatinib
RT-PCR
Resumen

El dermatofibrosarcoma protuberans (DFSP) es una neoplasia de partes blandas de malignidad intermedia, localizada inicialmente en la piel, desde donde invade tejidos más profundos (grasa, fascia, músculo y hueso). Se trata del tumor fibrohistiocitario más frecuente, constituyendo aproximadamente el 1,8% de todos los sarcomas de partes blandas y un 0,1% de todos los cánceres. Su incidencia se ha estimado entre 0,8-5 casos por millón de habitantes y año. El tratamiento de la enfermedad localizada consiste en la resección quirúrgica completa de la lesión, bien mediante cirugía convencional con margen amplio > 3cm) bien mediante cirugía micrográfica de Mohs. Aunque los casos de DFSP metastásico no llegan al 5% del total, prácticamente todos ellos aparecen tras recaídas locales previas. El pronóstico de los casos metastásicos es muy pobre, con menos de 2 años de supervivencia tras la detección de la enfermedad metastásica. Los pacientes de DFSP con tumores localmente avanzados no son susceptibles de tratamiento quirúrgico radical de inicio, por lo que se precisa tratamiento neoadyuvante previo a la cirugía para disminuir el tamaño tumoral. En este sentido, la quimioterapia y la radioterapia se han mostrado poco eficaces, por lo que se hace necesario contar con nuevas alternativas terapéuticas. La demostración del poder oncogénico de la traslocación COL1A1-PDGFB en el DFSP ha permitido introducir con éxito el tratamiento farmacológico con inhibidores del receptor del PDGFB en los casos metastáticos o localmente avanzados.

Palabras clave:
dermatofibrosarcoma protuberans
cirugía de Mohs
COL1A1-PDGFB
imatinib
TI-PCR
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This article was funded in part by a grant from the regional government of Valencia (Generalitat Valenciana), Spain (grant GV06/274).

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