RESUMO
Soft-tissue sarcomas constitute an uncommon and heterogeneous group of tumors of mesenchymal origin. Diagnosis, treatment, and management should be performed by an expert multidisciplinary team. MRI/CT of the primary tumor and biopsy is mandatory before any treatment. Wide surgical resection with tumor-free tissue margin is the mainstay for localized disease. Radiotherapy is indicated in large, deep, high-grade tumors, or after marginal resection not suitable for re-excision. Perioperative chemotherapy should be discussed for high-risk sarcomas of the extremities and trunk-wall. In the case of oligometastatic disease, patients should be considered for local therapies. First-line treatment with anthracyclines (or in combination with ifosfamide) is the treatment of choice. Other drugs have shown activity in second-line therapy and in specific histological subtypes but options are limited and thus, a clinical trial should always be discussed.
Assuntos
Sarcoma/diagnóstico , Sarcoma/terapia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/terapia , Antraciclinas/uso terapêutico , Antineoplásicos/uso terapêutico , Lista de Checagem , Quimioterapia Adjuvante/métodos , Dermatofibrossarcoma/terapia , Feminino , Fibromatose Agressiva/genética , Fibromatose Agressiva/terapia , Humanos , Imageamento por Ressonância Magnética , Masculino , Oncologia , Terapia Neoadjuvante/métodos , Radioterapia/métodos , Neoplasias Retroperitoneais/terapia , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Sociedades Médicas , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Tumores Fibrosos Solitários/tratamento farmacológico , Espanha , Tomografia Computadorizada por Raios X , Neoplasias Uterinas/terapiaRESUMO
Dermatofibrosarcoma protuberans (DFSP) is a rare, infiltrative, soft tissue tumor. It has a propensity for deep invasion but a low risk for distant metastasis. The classic presentation is a slowly progressive, painless, and erythematous to purpuric patch on the trunk or arms. A deep, subcutaneous punch biopsy or incisional biopsy should be performed for diagnosis in all suspected cases; wide undermining of the skin is to be avoided for minimizing the risk of tumor seeding and for retaining the feasibility of histopathologic examination of re-excisions. Histopathologic distinction of DFSP from dermatofibroma requires immunohistochemical assessment for CD34, factor XIIIa, nestin, apolipoprotein D, and cathepsin K. Management of this cutaneous sarcoma involves a multidisciplinary oncologic approach. Surgical excision is usually the first step in management. DFSP has a high propensity for local recurrence, even when surgical margins are negative; therefore, radiation therapy or rarely systemic therapy is recommended, especially for locally advanced or metastatic cases. The indolent nature of DFSP requires lifelong surveillance for recurrence; however, most recurrences occur within 3 years of the primary excision. The median time for the development of a local recurrence is estimated to be 32 months. An emerging theragnostic transmembrane receptor target, folate hydrolase-1 (FOLH1; prostate-specific membrane antigen), has been expressed in benign dermatofibromas and in high-grade sarcomatous phenotypes. These findings suggest that DFSP may also express FOLH1, which could allow for surveillance with FOLH1 PET/CT and antibody-mediated brachytherapy.
Assuntos
Dermatofibrossarcoma/terapia , Neoplasias Cutâneas/terapia , Antígenos de Superfície/metabolismo , Biópsia , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/patologia , Glutamato Carboxipeptidase II/metabolismo , Humanos , Margens de Excisão , Recidiva Local de Neoplasia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Fatores de TempoRESUMO
El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, de baja frecuencia, fibrohistiocítico, infiltrante, de lento crecimiento, de agresividad local, de malignidad intermedia; con escasas probabilidades de metástasis pero con alto índice de recurrencia local. El diagnóstico debe sospecharse y confirmarse con histología e inmunohistoquímica. El tratamiento de elección es con cirugía convencional y/o cirugía micrográfica de Mohs, con márgenes de 2-4 cm. Se considera que la prevalencia del DFSP en la edad pediátrica es baja, debido al escaso índice de sospecha. En el presente trabajo compartimos cinco casos de DFSP en la edad pediátrica, estudiados en el Hospital General de Niños Pedro de Elizalde. (AU)
Dermatofibrosarcoma protuberans (DFSP) is a cutaneous, low frequency, fibrohistiocytic, infiltrating, slow growing, local aggressiveness, intermediate malignancy tumor; with little chance of metastasis but with a high rate of local recurrence. The diagnosis should be suspected and confirmed with histology and immunohistochemistry. The treatment of choice is with conventional surgery and / or Mohs micrographic surgery, with margins of 2-4 cm. The prevalence of DFSP in pediatric age is considered to be low, due to the low index of suspicion. In this paper we share five cases of DFSP in the pediatric age, studied at the Pedro de Elizalde Children's General Hospital. (AU)
Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/diagnóstico , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/diagnóstico , Pediatria , Neoplasias Cutâneas/terapia , Dermatofibrossarcoma/terapia , Diagnóstico DiferencialRESUMO
Introdução: Dermatofibrossarcoma protuberante é um tumor cutâneo raro de malignidade intermediária e potencial metastático baixo, apresentando, entretanto, alta taxa de recorrência após tratamento cirúrgico. O tratamento cirúrgico clássico é a ressecção alargada com margens variadas. Métodos: Os pacientes foram submetidos à expansão prévia dos tecidos da região frontal utilizando dois expansores convencionais. Margens laterais de 3 cm de tecido de aparência normal foram tatuadas com tinta nanquim. Os expansores foram incluídos em uma cirurgia anterior, através de incisões cutâneas distantes dessas marcas, em um plano cirúrgico logo acima do periósteo, preservando a área de ressecção alargada previamente delimitada. Os tecidos laterais foram expandidos e, posteriormente, os pacientes foram submetidos à ressecção do tumor. A margem profunda incluiu da lâmina externa do osso frontal. O período médio de expansão foi de 45 dias. Resultados: Foram relatados três casos de dermatofibrossarcoma protuberante da região frontal com envolvimento da lâmina externa do osso frontal. Os retalhos expandidos fecharam facilmente os defeitos. O acompanhamento pós-operatório médio foi de 194 meses, sem nenhuma recorrência tumoral. Conclusão: A expansão tecidual prévia da região frontal no tratamento do dermatofibrossarcoma protuberante, invadindo o osso frontal, usando margens laterais de 3 cm com remoção da lâmina externa, permitiu facilmente o fechamento do defeito, em três pacientes. O tempo de acompanhamento médio de 194 meses sem nenhuma recorrência tumoral mostrou a eficiência do método.
Introduction: Dermatofibrosarcoma protuberans is a rare skin tumor with intermediate malignancy and low metastatic potential, but a high recurrence rate after surgical treatment. Treatment involves extended resection with variable margins. Methods: Patients were managed with preceding expansion of tissues of the forehead using two conventional expanders. The lateral margins of normal-appearing tissue 3-cm apart were tattooed with China ink. In a preceding operation, expanders were inserted through skin incisions at a distance from the marks, by undermining the surgical plane just above the periosteum, while preserving the area of wide resection previously delimited. After the lateral tissues were expanded, the patients underwent surgical resection. The deep margin included the outer table of the frontal bone. The expansion period averaged 45 days. Results: Three cases of dermatofibrosarcoma protuberans of the forehead with involvement of the outer table of the frontal bone are described. The expanded flaps easily closed the defects. The average follow-up was 194 months without tumor recurrence. Conclusion: Preceding expansion of forehead tissue in the treatment of dermatofibrosarcoma protuberans with invasion of frontal bone, using 3-cm lateral margins and removal of the outer table of the frontal bone, allowed easy closure of the defect in 3 different patients. Average follow-up of 194 months with no recurrence of the lesion showed the effectiveness of the method.
Assuntos
Humanos , Masculino , Feminino , Adulto , História do Século XXI , Neoplasias Cutâneas , Procedimentos Cirúrgicos Operatórios , Dispositivos para Expansão de Tecidos , Expansão de Tecido , Estudos Retrospectivos , Dermatofibrossarcoma , Osso Frontal , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/terapia , Procedimentos Cirúrgicos Operatórios/métodos , Dispositivos para Expansão de Tecidos/efeitos adversos , Expansão de Tecido/efeitos adversos , Expansão de Tecido/métodos , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/terapia , Osso Frontal/cirurgiaRESUMO
OPINION STATEMENT: Dermatofibrosarcoma protuberans (DFSP) is a slow growing tumor with a very low metastatic potential but with significant subclinical extension and great capacity for local destruction. Thus, the first surgeon approached with such challenging tumor must attempt to cure the patient with a method that spares healthy tissue and ensures an optimal oncological, functional, and esthetic result. The treatment of DFSP often requires a multidisciplinary approach. Depending on location, dermatologic surgeons, surgical oncologists, head and neck surgeons, neurosurgeons, plastic surgeons, and occasionally medical oncologists may be involved with the management. Mohs micrographic surgery (MMS) is the preferred method when available. In our institution, most of the DFSP cases are often advanced cases; thus, dermatologic surgeons obtain clear margins peripherally and other surgical specialties assist with resection of the fascia and any critical deeper structures. When MMS is not available, wide local excision (at least 2- to 3-cm margins of resection) with exhaustive pathologic assessment of margin status is recommended, and it is best to confirm tumor extirpation prior to any reconstruction. Subclinical extension of the tumor could be related to the size; how long it has been growing or histological markers that are unknown right now. No clinical trials comparing MMS vs WLE are available, and further research should be focused on these subjects as well as the use of imatinib and other targeted therapies for recurrent and metastatic tumors and for neoadjuvant treatment.
Assuntos
Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Biópsia , Terapia Combinada/efeitos adversos , Terapia Combinada/métodos , Dermatofibrossarcoma/epidemiologia , Dermatofibrossarcoma/etiologia , Gerenciamento Clínico , Suscetibilidade a Doenças , Predisposição Genética para Doença , Humanos , Imagem Multimodal/métodos , Estadiamento de Neoplasias/métodos , Fenótipo , Prognóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/etiologia , Resultado do TratamentoRESUMO
Dermatofibrosarcoma Protuberans (DFS-P) es una neoplasia de partes blandas y cutánea poco frecuente, de crecimiento superficial con tendencia a recurrir localmente y bajo potencial metastásico. Ocurre en personas de edad media, preferentemente en tronco y zonas proximales de extremidades. Requiere un alto grado de sospecha clínica, ya que inicialmente puede ser similar a una cicatriz hipertrófica o a un dermatofibroma. A continuación se realiza una revisión de la literatura sobre este tumor poco frecuente, con énfasis en las características clínicas y las opciones terapéuticas actualizadas según las guías 2010 de la National Comprehensive Cancer Network(NCCN).
Dermatofibrosarcoma protuberans (DFS-P) is a soft cutaneous neoplasm, pretty rare, with superficial growing, that tends to local recurrence. Usually it present at medium age, in trunk and the beginning of extremities. It is very difficult to diagnose at the beginning because its similarity with hypertrophic scar or dermatofibroma. We present a review in the literature of DFS-P, with emphasizing the early clinical manifestations and the different therapeutic agents recently approved by National Comprehensive Cancer Network (NCCN).
Assuntos
Humanos , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Evolução Clínica , Dermatofibrossarcoma/epidemiologia , Dermatofibrossarcoma/terapia , Imuno-Histoquímica , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapiaRESUMO
Introdução. O dermatofibrossarcoma protuberante é uma neoplasia de tecidos moles de baixo grau proveniente da derme. É um tipo raro de sarcoma que pode ocorrer em qualquer local do corpo. Ocorre mais comumente em adultos jovens ou de meia idade, se inicia como massa nodular cutânea no tronco ou nas extremidades. O padrão de crescimento é usualmente lento e persistente. Este tumor se desenvolve em anos e torna-se protuberante. Apresenta comportamento localmente agressivo sendo comum a recidiva local. No entanto, as metástases a distância são excepcionais. O tratamento, em geral, consiste em ressecção ampla tridimensional com margens de 2 a 3 cm. Casos. O autor apresenta três casos desse raro tipo de sarcoma, que foram tratados por ressecção radical com boa evolução. Todas as ressecções foram R0 (margens livres). Não houve recidivas, com seguimento de dois equatro anos. Conclusão. A despeito de sua raridade, o dermatofibrossarcoma protuberante pode ter bom prognóstico se adequadamente tratado. Tem sido observada forte correlação entre diagnóstico correto e cirurgia adequada com maior controle dessa neoplasia em longo prazo.
Introduction. Dermatofibrosarcoma protuberans is probably best considered a low-grade dermic soft tissue tumor. It is a rare type of sarcoma that may occur anywhere in the body. More frequently, it occurs in early or middle life beginning as a nodular mass. The pattern of growth is usually slow and persistent. This tumor enlarge over many years, it becomes protuberant. It presents locally aggressive nature furthermore local recurrence is common, although distant metastases are exceptional. The wide resection with 2-3 cm free margins is the best treatment. Cases. The author presents three cases of this rare sarcoma that were treated by means radical resection with a good outcome. All resections were R0 (free-margins). There were no recurrences with follow-up period between two and four years.Conclusion. Despite of its rarity, dermatofibrosarcoma protuberans may present a good prognosis if adequately treated. It has been observed great relationship between correct diagnosis plus adequate surgery with major longterm disease control.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Dermatofibrossarcoma , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/terapia , SarcomaRESUMO
O dermatofibrossarcoma protuberante é um tumor de pele de baixa incidência e difícil tratamento, dado o grande número de recidivas. O tratamento clássico consiste na ressecção com margens amplas, algumas vezes levando a sério comprometimento funcional e ou estético e apesar disto, dados da literatura revelam um índice de recidiva variando de 11% a 53%. Recentemente a radioterapia vem sendo utilizada mudando o curso usual deste tumor, diminuindo drasticamente os níveis de recorrência comparando quando somente a cirurgia é empregada. Neste artigo revisamos estas modalidades e fazemos o relato de três casos de dermatofibrossarcoma protuberante recidivados e de grandes dimensões tratados em nosso serviço com ressecção e radioterapia adjuvante e que permanecem livres de recidivas após dois anos da ressecção cirúrgica.
Assuntos
Dermatofibrossarcoma/terapia , Recidiva Local de Neoplasia , Neoplasias Cutâneas/diagnóstico , Radioterapia/efeitos adversosRESUMO
A review of dermatofibrosarcoma protuberans diagnosed at the University hospital of the West Indies over a 10-year period revealed 23 cases. The mean age of the patients was 36.3 years. There were 13 females and 10 males. The trunk was the most common site. The clinical diagnosis was commonly missed. The results of this study are compared with those from other countries
Assuntos
Adulto , Pessoa de Meia-Idade , Humanos , Masculino , Feminino , Neoplasias Cutâneas/patologia , Dermatofibrossarcoma/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Estudos Retrospectivos , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/terapiaRESUMO
A review of dermatofibrosarcoma protuberans diagnosed at the University hospital of the West Indies over a 10-year period revealed 23 cases. The mean age of the patients was 36.3 years. There were 13 females and 10 males. The trunk was the most common site. The clinical diagnosis was commonly missed. The results of this study are compared with those from other countries (AU)