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Vol. 109. Num. 10.December 2018
Pages e1-e4Pages 851-940
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Vol. 109. Num. 10.December 2018
Pages e1-e4Pages 851-940
Review
DOI: 10.1016/j.adengl.2018.05.034
Guidelines for Diagnosis and Treatment of Cutaneous Sarcomas: Dermatofibrosarcoma Protuberans
Sarcomas cutáneos: directrices para el diagnóstico y tratamiento. Dermatofibrosarcoma protuberans
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B. Llombarta,
Corresponding author
beatriz.llombart@uv.es

Corresponding author.
, C. Serraa, C. Requenaa, M. Alsinab, D. Morgado-Carrascob, V. Travésc, O. Sanmartína
a Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, Spain
b Servicio de Dermatología, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
c Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, Spain
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Tables (4)
Table 1. Fédération National de Centres de Lutte Contre le Cancer (FNCLCC) Grading System for Soft Tissue Sarcomas.7
Table 2. TNM Classification of Soft Tissue Sarcomas From the American Joint Committee on Cancer (AJCC) Staging Manual (7th Edition, 2010), 2016 version.5
Table 3. Main Cutaneous Sarcomas Classified by Histologic Subtype and Approximate 5-Year Survival Rates.
Table 4. Dermatofibrosarcoma Protuberans (DFSP): Histologic Subtypes.
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Abstract

Sarcomas comprise a broad group of tumors, many of whose biological behavior and aggressiveness differ from one type to another. The therapeutic approach is generally multidisciplinary and often complex. Developments in surgical and oncological dermatology during the last few decades have positioned dermatologists as specialists in the diagnosis and treatment of skin cancer. The aim of this article is to review the main soft tissue sarcomas that typically affect the skin. Dermatofibrosarcoma protuberans is a low-grade malignant sarcoma. It exhibits slow-growth, is locally invasive, and has low metastatic potential (<3%). Mohs micrographic surgery is the treatment of choice. The COL1A1-PDGFB translocation should be analyzed in cases of unclear diagnosis and when it is necessary to identify candidates for tyrosine kinase inhibitors. Imatinib is indicated for the treatment of locally advanced and metastatic dermatofibrosarcoma protuberans.

Keywords:
Sarcoma
Dermatofibrosarcoma protuberans
Diagnosis
Treatment
Resumen

Los sarcomas constituyen un grupo amplio de tumores, muchos de ellos con comportamiento biológico y agresividad diferentes entre sí, que habitualmente requieren un tratamiento multidisciplinario, frecuentemente complejo. El desarrollo en las últimas décadas de la dermatología quirúrgica y oncológica ha permitido que los dermatólogos se conviertan en los especialistas responsables del diagnóstico y tratamiento del cáncer cutáneo. El propósito de este artículo es revisar los principales sarcomas de partes blandas de localización típicamente cutánea.

El dermatofibrosarcoma protuberans es un sarcoma de bajo grado de malignidad, con un crecimiento lento e infiltrativo localmente y escasa capacidad metastásica (<3%). El tratamiento de elección es la cirugía micrográfica de Mohs. Es recomendable solicitar el estudio de la translocación COL1A1-PDGFB cuando existen dudas diagnósticas, y para determinar qué pacientes pueden responder a los fármacos inhibidores de la tirosina quinasa. El imatinib está indicado en el dermatofibrosarcoma protuberans localmente avanzado y metastásico.

Palabras clave:
Sarcoma
Dermatofibrosarcoma protuberans
Diagnóstico
Tratamiento

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