RESUMO
Importance: Dermatofibrosarcoma protuberans (DFSP) has the potential for local destruction and recurrence, although it carries a low risk of metastasis. Complete surgical resection with negative margins is considered the gold standard for treatment; however, there are cases that are unresectable owing to tumor extension or size or owing to risk of cosmetic and/or functional impairment. Imatinib treatment has been used for locally advanced or metastatic DFSP. Objective: To evaluate the usefulness of imatinib for treating DFSP. Evidence Review: We conducted a systematic review on the PubMed and Embase databases for articles published from September 2002 through October 2017 using the key words "dermatofibrosarcoma" or "dermatofibrosarcoma protuberans" AND "therapy" AND "imatinib." References within retrieved articles were also reviewed to identify additional studies. Studies of adults with histologically proven DFSP treated with imatinib as monotherapy or as an adjuvant or neoadjuvant therapy to surgery were included. Extracted data were analyzed using descriptive statistics. PRISMA guidelines were followed. All analysis took place October through December 2017. Findings: Nine studies met inclusion criteria; 152 patients were included. The calculated mean patient age was 49.3 years (range, 20-73 years). Calculated mean tumor diameter was 9.9 cm (range, 1.2-49.0 cm). When COL1A1-PDGFß protein translocation (collagen, type 1, alpha 1-platelet-derived growth factor ß) was reported, it was present in 90.9% of patients (111 of 122). Complete response was seen in 5.2% of patients (8 of 152), partial response in 55.2% (84 of 152), stable disease in 27.6% (42 of 152), and progression in 9.2% (14 of 152). Four of the 152 patients (2.6%) were excluded from the analysis owing to unknown or unevaluable response. There were no differences in response rate using 400-mg or 800-mg daily doses (67.5% or 27 of 40 patients for 400-mg dose vs 67.1% or 49 of 73 patients for 800-mg dose complete or partial response; P > .99). Adverse events were present in at least 73.5% of cases (78 of 106); severe adverse events were present in 15.1% of cases (20 of 132). Conclusions and Relevance: Imatinib is a useful directed therapy in patients with DFSP who are not surgical candidates owing to disease extension or significant cosmetic or functional impairment. There seems to be no difference between 400- or 800-mg daily doses.
Assuntos
Dermatofibrossarcoma/tratamento farmacológico , Dermatofibrossarcoma/patologia , Mesilato de Imatinib/uso terapêutico , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia , Adulto , Idoso , Dermatofibrossarcoma/mortalidade , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Esquema de Medicação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Prognóstico , Neoplasias Cutâneas/mortalidade , Análise de Sobrevida , Resultado do Tratamento , Adulto JovemRESUMO
O dermatofibrossarcoma protuberans (DFSP) compreende 1 porcento de todas as neoplasias malignas. Apresenta maior incidência entre a segunda e quinta décadas de vida e maior distribuiçäo no sexo masculino. As lesöes surgem inicialmente como nódulo cutâneo indolor, podendo haver, em alguns casos (12 a 17 porcento), história de trauma pregresso. Geralmente tem curso benigno, mas, após repetidas recidivas, pode disseminar para órgaös a distância. Foram estudados sete casos de DFSP em dois serviços. Observou-se maior incidência em couro cabeludo (42,8 porcento). Todos os pacientes foram submetidos à ressecçäo cirúrgica da lesäo com margens adequadas; a literatura preconiza margem de 3cm de pele normal. A dissecçäo linfonodal só está indicada quando houver metástases linfáticas locorregionais, evento infreqüente no curso natural do DFSP. O mesmo pode ser dito em relaçäo às metástases à distância. Na casuística dos autores näo foram encontrados pacientes com doença avançada. Apenas dois pacientes (28,6 porcento) foram operados para ressecçäo de recidiva local do tumor. A radioterapia tem sido utilizada nos casos de doença irressecável ou quando näo há condiçöes de ressecçäo com margens adequadas por implicar perda funcional ou cosmética extensa. A quimioterapia tem demonstrado efetividade contra doença metastática