0000000000607967

AUTHOR

Carlos Serra-guillén

Dermatofibrosarcoma protuberans: estudio de factores pronósticos

Introducción: El dermatofibrosarcoma protubernas (DFSP) es un tumor cutáneo de malignidad intermedia clasificado dentro de los tumores fibrohistiocíticos que tiene una alta tasa de recidivas locales tras extirpaciones con cirugía convencional pero baja capacidad de producir metástasis. Hipótesis: Existen determinadas características del DFSP que condicionan un crecimiento más infiltrativo, asimétrico e impredecible, lo que supone mayor morbilidad para el paciente y una mayor dificultad para su tratamiento. Objetivos: 1.-Realizar un análisis observacional y descriptivo de una serie de casos de DFSP que han sido vistos en el Servicio de Dermatología del IVO, según las características epidemio…

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Molecular diagnosis of dermatofibrosarcoma protuberans: A comparison between reverse transcriptase-polymerase chain reaction and fluorescence in situ hybridization methodologies

Dermatofibrosarcoma protuberans (DFSP) is characterized by the presence of the t(17;22)(q22;q13) that leads to the fusion of the COL1A1 and PDGFB genes. This translocation can be detected by multiplex reverse transcriptase-polymerase chain reaction (RT-PCR) or fluorescence in situ hybridization (FISH) techniques. We have evaluated the usefulness of a dual color dual fusion FISH probe strategy for COL1A1/PDGFB detection in a series of 103 archival DFSPs and compared the obtained results with RT-PCR analyses. FISH and RT-PCR were carried out on paraffin embedded tissue samples. Regarding the RT-PCR approach, all COL1A1 exons and exon 2 of PDGFB were evaluated. Sensitivity, specificity, positi…

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Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management

Dermatofibrosarcoma protuberans (DFSP) is a rare superficial tumor characterized by high rates of local recurrence and low risk of metastasis. DFSP occurs most commonly on the trunk and proximal extremities, affects all races, and often develops between the second and fifth decade of life. The tumor grows slowly, typically over years. Histologically, several variants of DFSP have been described and should be well characterized to avoid misdiagnosis with other tumors. These include pigmented (Bednar tumor), myxoid, myoid, granular cell, sclerotic, atrophic DFSP, giant cell fibroblastoma, and DFSP with fibrosarcomatous areas. Of all these variants, only the DFSP with fibrosarcomatous areas is…

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