Dermatofibrosarcoma protuberans

  • Juan Camilo Barrera Instituto Nacional de Cancerología
  • Álvaro Enrique Acosta Universidad Nacional de Colombia
  • Laura Trujillo Fundación universitaria sanitas
Palabras clave: Dermatofibrosarcoma, Terapéutica, Cirugía, Cirugía de Mohs, Patología

Resumen

El dermatofibrosarcoma protuberans (DFSP) es un sarcoma cutáneo de baja incidencia y lento crecimiento que afecta principalmente personas de los 20 a los 39 años y es más frecuente en afrodescendientes.  La localización anatómica más común es el tronco, seguido de las extremidades superiores.  La etiología no está clara, pero la mayoría de los casos presentan rearreglos genéticos que involucran los genes COL1A1 y PDGFB. El riesgo de metástasis es bajo, pero tiene tendencia a la recurrencia local, especialmente en los tumores resecados con márgenes insuficientes, en los tumores profundos y los que presentan cambio fibrosarcomatoso. La primera línea de manejo para la enfermedad local es la cirugía; resección local amplia o cirugía micrográfica de Mohs (CMM). Esta última técnica ha mostrado recurrencias consistentemente bajas.  La radioterapia se ha usado para tumores irresecables o como adyuvancia. El imatinib es de utilidad para enfermedad metastásica.  

Biografía del autor/a

Juan Camilo Barrera, Instituto Nacional de Cancerología

Dermatólogo, Fellow de dermatología oncológica. Instituto Nacional de Cancerología. Bogotá, Colombia.

Álvaro Enrique Acosta, Universidad Nacional de Colombia

Profesor titular de la Universidad Nacional de Colombia. Dermatólogo oncólogo. Instituto nacional de cancerología. Bogotá, Colombia.

Laura Trujillo, Fundación universitaria sanitas

Residente de dermatología. Fundación universitaria sanitas. Bogotá, Colombia.

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Publicado
2019-09-29
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