RESUMO
BACKGROUND: Skin cancer is the most common cancer in human beings. Ultrasound is a powerful and non-invasive imaging technique that has expanded its use in dermatology, including in the skin cancer field. The full range of critical anatomical information provided by ultrasound cannot be deduced from a naked eye examination, palpation, or other imaging techniques such as dermoscopy, confocal microscopy, magnetic resonance imaging, or PET-CT (Positron Emission Tomography-Computed Tomography). METHODS: This review practically analyzes the main ultrasonographic features of the most common types of skin cancers and the performance of the locoregional staging according to the literature, which is illustrated by state-of-the-art clinical and ultrasonographic correlations. RESULTS: The most common types of skin cancer show recognizable ultrasonographic patterns. CONCLUSIONS: Among the current radiological imaging techniques, ultrasound has the highest axial spatial resolution. Compared to other imaging techniques used in dermatology, it shows the great advantage of penetrating the soft tissues thoroughly, which allows us to detect and identify the most common skin types of skin cancer, including both the primary tumor and its locoregional metastases.
RESUMO
Dermatofibrosarcoma protuberans is a rare low-grade sarcoma, which rarely metastasizes, but it is locally aggressive with a propensity to recur. It usually affects persons of African descent and is extremely rare in childhood with a favourable prognosis. We present a case of paediatric dermatofibrosarcoma protuberans to the midline of the lower back of a 9-year-old Afro-Caribbean boy who was biopsied with a 2-mm margin. After histological confirmation, a 4-cm margin was then performed. Surveillance for recurrence, though none has been seen thus far after 6-month follow-up, will be done for at least 5 years and possibly longer, given this is the first case of this nature ever seen in our institution and the Caribbean region.
RESUMO
Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive cutaneous sarcoma with a propensity for recurrence. Its management, particularly in the head and neck (H&N) region, presents unique challenges. This study aimed to evaluate the effectiveness of Mohs micrographic surgery (MMS) compared to wide local excision (WLE) in treating H&N DFSP and its impact on recurrence rates and tissue preservation. A comprehensive search was conducted in PubMed/MEDLINE, yielding 29 relevant studies. We included studies comparing MMS and WLE in adult patients with H&N DFSP and reporting local recurrence outcomes. Data were analyzed using random effects analysis, with a meta-analysis performed for comparative studies. Analysis of studies demonstrated a lower recurrence for MMS. Comparative analysis of five studies involving 117 patients showed a significantly lower recurrence rate in the MMS group (2%) compared to the WLE group (19%). Margin status varied between studies, with some achieving negative margins at shorter distances. In the management of H&N DFSP, MMS has emerged as a superior surgical technique, consistently associated with reduced recurrence rates and the potential for tissue preservation. The adoption of MMS should be considered for its capacity to achieve negative margins with fewer processing steps, particularly in anatomically complex regions like the H&N.
Assuntos
Dermatofibrossarcoma , Neoplasias Cutâneas , Adulto , Humanos , Dermatofibrossarcoma/cirurgia , Cirurgia de Mohs/métodos , Recidiva Local de Neoplasia/cirurgia , Estudos Retrospectivos , Neoplasias Cutâneas/cirurgiaRESUMO
INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade mesenchymal skin tumor. Complete surgical excision remains the mainstay of treatment. Head and neck (HN) DFSP features high local recurrence (LR) rates with wide local excision (WLE) due to both anatomical limitations and functional and cosmetic concerns. Mohs micrographic surgery (MMS) allows complete margin control and is particularly suitable for HN-DFSPs. PATIENTS AND METHODS: Between 2000 and 2018, 103 DFSP were managed with MMS; 41 (39.8%) were in the HN. Our case series included 23 females (56.1%); mean age: 44.5 yrs. The most frequent sites were the supraclavicular area (10) and forehead (9). Mean size: 5.1â¯cm (6-16). Previous surgery: 43.9%. We have used permanent-tissue MMS (27 cases) since 2006. RESULTS: Mean first layer lateral margin: 1.6â¯cm. Negative margins after first and second stage: 51.2% and 92.7%, respectively. Mean follow-up: 92.6 months (16-225). LR: 1(2.4%). We observed no regional or distant metastasis. DISCUSSION: We conducted a literature search in PubMed for HN-DFSP and found that this seems to be the largest published series to date. In terms of LR, a substantial difference between WLE 62.5% (9-73%) and MMS 0.8% (0-2.4%) was seen. Forty-one HN-DFSP with unfavorable prognostic factors (mean size: 5.1â¯cm, previous treatment: 43.9%) were treated. LR: 1(2.4%). Mean follow-up: 92.6 months. Randomized controlled trials are needed, though not likely to be conducted, mainly because of the rarity of HN-DFSP, the need for a long follow-up, and the lack of MMS availability in some institutions. We consider MMS to be the standard treatment for HN-DFSP due to its high cure rate and tissue sparing. Modified WLE with complete margin control appears to be a comparable strategy.
Assuntos
Dermatofibrossarcoma/cirurgia , Neoplasias de Cabeça e Pescoço/cirurgia , Cirurgia de Mohs , Adolescente , Adulto , Idoso , Argentina , Dermatofibrossarcoma/patologia , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Inclusão em ParafinaRESUMO
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon mesenchymal tumor of intermediate malignancy. Its rarity and slow progression often imply a delayed diagnosis. There are few previous reports of dermoscopic features of DFSP and most are single case descriptions. OBJECTIVES: To report 2 cases of DFSP and their dermoscopic features, as well as conduct a review of all previous cases published addressing the use of dermoscopy in this tumor. METHODS: We conducted a literature search for all dermoscopic cases of DFSP. In addition, we presented 2 additional cases and compared them to the earlier findings. RESULTS: We summarized the main dermoscopic findings of DFSP based on analysis from 32 patients. The most common features of this tumor are the presence of vessels (81%), followed by a pigmented network (78%) and a pinkish background (66%). CONCLUSIONS: DFSP can mimic benign lesions and the diagnosis may be challenging. Dermoscopy is an important tool that may enhance clinical suspicion toward the diagnosis of DFSP.
RESUMO
RESUMEN El dermatofibrosarcoma protuberante es un raro tumor fibrohistiocitario de la piel y grado intermedio de malignidad. Localmente tiene un comportamiento agresivo, se extiende al tejido celular subcutáneo y músculo subyacente. Su crecimiento es lento e indoloro con una alta frecuencia de recurrencia posterior a la intervención quirúrgica. Se presentó una paciente operada en el Servicio de Cirugía General del "Hospital Universitario Dr. Mario Muñoz Monroy" de Colón, Matanzas. Diagnosticada con dermatofibrosarcoma protuberans se presentó con un tumor de partes blandas, localizado en parrilla costal izquierdo, recidivante sobre una lesión resecada anteriormente hacia 8 meses. El tumor de superficie multinodular e irregular, de aproximadamente 3 a 4 cm de diámetro, presentaba zonas de color azuladas y otras más oscuras con un área enrojecida circundante, consistencia dura, y no adherida a planos profundos. Con el resultado anatomopatológico se ratificó el diagnóstico. Nuevamente se aplicó tratamiento quirúrgico con amplios márgenes libres de tumor. Las metástasis son raras y pocos frecuentes, pero localmente pueden infiltrar, grasa, fascia, músculos y hasta estructuras óseas. Cuando se presentan ocurren generalmente en pulmón y ganglios linfáticos regionales, en estos casos la supervivencia es muy poca (AU).
ABSTRACT Dermatofibrosarcoma protuberant (DFSP) is a rare fibrohistiocytic skin tumor of intermediate level of malignance. Locally it has an aggressive behavior extending to the subcutaneous cellular tissue and underlying muscle. Its grow is slow and painless with a high recurrence frequency after surgery. The authors present the case of a female patient operated in the Service of General Surgery of the University Hospital ¨Dr. Mario Muñoz Monroy¨ of Colon, Matanzas. Diagnosed with dermatofibrosarcoma protuberant, she arrived to the consultation with a tumor of soft tissues located in left rib cage, recidivist on a lesion resected 8 months before. It had a multinodular and irregular surface, of around 3-4 cm diameter, with bluish and dark blue areas and a reddened surrounding area of hard consistence, not adhered to deep planes. The diagnosis was ratified by the neuropathologic result. The surgical treatment was applied again with wide free-tumors margins. Metastases are rare and few frequent, but locally they can infiltrate fat, fascia, muscles and even bone structures. When they present they occur mainly in lung and regional lymphatic ganglia; in these cases survival is scarce (AU).
Assuntos
Humanos , Feminino , Adulto , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/patologia , Dermatofibrossarcoma/diagnóstico por imagem , Neoplasias Cutâneas/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Recidiva Local de NeoplasiaRESUMO
RESUMEN El dermatofibrosarcoma protuberante es un raro tumor fibrohistiocitario de la piel y grado intermedio de malignidad. Localmente tiene un comportamiento agresivo, se extiende al tejido celular subcutáneo y músculo subyacente. Su crecimiento es lento e indoloro con una alta frecuencia de recurrencia posterior a la intervención quirúrgica. Se presentó una paciente operada en el Servicio de Cirugía General del "Hospital Universitario Dr. Mario Muñoz Monroy" de Colón, Matanzas. Diagnosticada con dermatofibrosarcoma protuberans se presentó con un tumor de partes blandas, localizado en parrilla costal izquierdo, recidivante sobre una lesión resecada anteriormente hacia 8 meses. El tumor de superficie multinodular e irregular, de aproximadamente 3 a 4 cm de diámetro, presentaba zonas de color azuladas y otras más oscuras con un área enrojecida circundante, consistencia dura, y no adherida a planos profundos. Con el resultado anatomopatológico se ratificó el diagnóstico. Nuevamente se aplicó tratamiento quirúrgico con amplios márgenes libres de tumor. Las metástasis son raras y pocos frecuentes, pero localmente pueden infiltrar, grasa, fascia, músculos y hasta estructuras óseas. Cuando se presentan ocurren generalmente en pulmón y ganglios linfáticos regionales, en estos casos la supervivencia es muy poca.
ABSTRACT Dermatofibrosarcoma protuberant (DFSP) is a rare fibrohistiocytic skin tumor of intermediate level of malignance. Locally it has an aggressive behavior extending to the subcutaneous cellular tissue and underlying muscle. Its grow is slow and painless with a high recurrence frequency after surgery. The authors present the case of a female patient operated in the Service of General Surgery of the University Hospital ¨Dr. Mario Muñoz Monroy¨ of Colon, Matanzas. Diagnosed with dermatofibrosarcoma protuberant, she arrived to the consultation with a tumor of soft tissues located in left rib cage, recidivist on a lesion resected 8 months before. It had a multinodular and irregular surface, of around 3-4 cm diameter, with bluish and dark blue areas and a reddened surrounding area of hard consistence, not adhered to deep planes. The diagnosis was ratified by the neuropathologic result. The surgical treatment was applied again with wide free-tumors margins. Metastases are rare and few frequent, but locally they can infiltrate fat, fascia, muscles and even bone structures. When they present they occur mainly in lung and regional lymphatic ganglia; in these cases survival is scarce.
Assuntos
Humanos , Feminino , Adulto , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/patologia , Dermatofibrossarcoma/diagnóstico por imagem , Neoplasias Cutâneas/diagnóstico , Neoplasias de Tecidos Moles/diagnóstico , Recidiva Local de NeoplasiaRESUMO
RESUMEN El dermatofibrosarcoma protuberans (DFSP) es un sarcoma fusocelular de grado intermedio de malignidad con máxima incidencia en adultos entre 20 y 40 años y de localización habitualmente troncular (cabeza, cuello extremidades superiores). Se presentan tres casos de DFSP de localización excepcional a nivel vulvar. Las pacientes fueron tratadas con vulvectomía respetando márgenes de seguridad pero, debido a la idiosincrasia del tumor, presentaron recidivas locales que precisaron de una nueva cirugía. En su posterior seguimiento no presentaron recaídas y se encuentran libres de enfermedad.
ABSTRACT Dermatofibrosarcoma protuberans (DFSP) is an intermediate grade spindle-cell sarcoma with a highest incidence in adults between 20 and 40 years old and a trunk location (head, neck and arms). We introduce three case reports of vulvar DFSP considered unusual because of their location. The patients were conducted a free-margin vulvectomy but, due to the nature of the tumor, local reappearances required a second surgery. In the subsequent follow-up they did not suffer from any tumor relapse and they are currently disease-free.
Assuntos
Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/diagnóstico , Neoplasias Vulvares/cirurgia , Neoplasias Vulvares/diagnóstico , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Vulvares/patologia , Cirurgia de Mohs , Dermatofibrossarcoma/patologia , Diagnóstico Diferencial , VulvectomiaRESUMO
Introdução: O dermatofibrossarcoma protuberante (DFSP) é um tumor fibrohistiocítico de origem dérmica raro, que acomete a pele, representando 1% dos sarcomas de partes moles e menos de 0,1% de todas malignidades. A principal característica deste tipo de tumor é a sua elevada taxa de recidiva local após excisão cirúrgica. Decidir a margem cirúrgica adequada para a ressecção completa é um desafio. Relato de caso: 24 anos, apresenta lesão tumoral vegetante avermelhada no ombro esquerdo, assintomática. Possui história familiar positiva de DFSP em membro inferior. O laudo histopatológico definitivo confirmou Dermatofibrossarcoma protuberans. Discussão: A característica histológica do tumor é a presença de projeções tipo tentáculos de células neoplásicas na periferia que se estendem através do tecido subcutâneo até a fáscia muscular. Certamente, o fator de maior importância para o controle local é a obtenção de margens cirúrgicas livres. A excisão através da cirurgia micrográfica de Mohs é uma ótima opção em regiões onde a excisão ampla não é desejável, como na face. A margem padrão estabelicida em grande parte da literatura é de 3 cm com ressecção até a fáscia muscular, podendo ser diminuida para 2 cm em locais em que a excisão ampla prejudique a conformação do local, como na face. (AU)
Dermatofibrosarcoma protuberans (DFSP) is a rare dermal fibrohistiocytic tumor that affects the skin, accounting for 1% of soft tissue sarcomas and representing less than0.1% of all malignancies. The main characteristic of this type of tumor is a high rate of local recurrence after surgical excision. Deciding the proper surgical margin for complete resection is a challenge. Case report: 24 year-old, shows a reddish vegetative asymptomatic tumor on the left shoulder. She has a positive family history of DFSP in the lower limb. The definitive histopathological report confirmed Dermatofibrossarcoma protuberans. Discussion: The histological feature of the tumor is the presence of tentacle type projections of neoplastic cells in the periphery extending through the subcutaneous tissue to the muscular fascia. Of course, the most important factor for local control is the achievement of free surgical margins. Excision through Mohs micrographic surgery is a great option in regions where wide excision is not desirable, as in the face. The standard margin in most pf the literature is 3 cm resected to the muscular fascia, and it can be reduced to 2 cm in places where the extensive excision impairs the conformation of the site, as in the face. (AU)
Assuntos
Humanos , Feminino , Adulto , Neoplasias Cutâneas , Dermatofibrossarcoma , Retalho Miocutâneo , Margens de ExcisãoRESUMO
BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare low grade tumor with a locally aggressive behavior and low metastatic potential. OBJECTIVES: To evaluate the factors that are associated with relapse in DFSP. Methods Retrospective analysis of medical records from 61 patients with dermatofibrosarcoma. Fluorescence in situ hybridization was used to detect translocations. RESULTS: Of 61 patients, 6 experienced a relapse. No patient with resection margins greater than 3 cm had a recurrence. One relapse was observed in a patient treated with at least 2 cm margins and 4 relapses occurred in 16 patients whose margins were below 2 cm (P = 0.018). The frequency of translocations was 77.8%. The recurrence rate was lower in patients with translocation, but this difference was not significant. Immunohistochemical markers did not correlate with recurrence rates, but greater FasL expression was associated with recurrence in patients with margins smaller than 3 cm. CONCLUSIONS: Surgical margins smaller than than 2 cm are related to higher recurrences in dermatofibrosarcomas. In this analysis a 2 cm margin was acceptable for treatment. Between all the immunohistochemical markers analyzed, only FasL was associated with a higher recurrence rate in patients with margins smaller than 3 cm.
Assuntos
Biomarcadores Tumorais/metabolismo , Dermatofibrossarcoma/patologia , Recidiva Local de Neoplasia/patologia , Neoplasias Cutâneas/patologia , Translocação Genética , Adolescente , Adulto , Idoso , Apoptose , Proliferação de Células , Criança , Pré-Escolar , Dermatofibrossarcoma/genética , Dermatofibrossarcoma/metabolismo , Feminino , Seguimentos , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Invasividade Neoplásica , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/metabolismo , Prognóstico , Estudos Retrospectivos , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/metabolismo , Adulto JovemRESUMO
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
El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo raro, localmente agresivo, con tendencia a la recurrencia local pero que rara vez metastatiza. Constituye el sarcoma de origen cutáneo más común. Representa un desafío diagnóstico y terapéutico para los dermatólogos, debido a la alta tasa de recurrencia local que posee. En el siguiente artículo, presentamos el caso de un paciente con este diagnóstico y su posterior manejo (AU)
Dermatofibrosarcoma protuberans (DFSP) is an unusual locally aggressive cutaneous tumor, with tendency to local recurrence but rarely to metastasis. It is the most common cutaneous sarcoma, presenting a diagnostic and therapeutic challenge for dermatologists, due to the high rate of local recurrence. A male patient diagnosed with DFSP and its subsequent management is reported (AU)
Assuntos
Humanos , Masculino , Adulto , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/patologia , Neoplasias Cutâneas , Diagnóstico DiferencialRESUMO
Introducción: El Dermatofibrosarcoma Protuberans (DFSP) es un sarcoma cutáneo infrecuente y poco descrito en la literatura regional. En este trabajo se describió y analizó las características clínicas e histopatológicas de los DFSP tratados en nuestro centro. Métodos: Estudio retrospectivo de todos los casos de DFSP primarios, confirmados con biopsia entre los años 2002 y 2016 en el Servicio de Anatomía Patológica del Hospital Clínico Universidad de Chile (HCUCh). Resultados: La muestra contó con 18 casos, 67% de sexo femenino, la edad promedio de diagnóstico fue 45,5 años. El 28% de los DFSP se localizaron en tronco, 22% extremidades inferiores, 22% extremidades superiores, 22% región inguinogenital y 6% cervical. Un 22% de las biopsias fueron enviadas con diagnóstico clínico presuntivo de DFSP. En la histopatología, todos los DFSP estaban compuestos por células fusiformes, distribuidas en patrón estoriforme que infiltraban hasta el tejido adiposo subcutáneo. La inmunohistoquímica se realizó en 7 casos, siendo positivo para CD34 en todas estas muestras y negativo para el factor XIIIa. En el 50% de los casos se observó compromiso de márgenes quirúrgicos. Conclusiones: Nuestros resultados fueron similares a publicaciones internacionales. Se observó mayor frecuencia en mujeres y diagnóstico clínico más tardío. Se constató un bajo índice de sospecha clínica de DFSP, confundiéndose con patologías benignas, lo que podría incidir en la frecuencia de márgenes positivos del estudio. Este trabajo constituye uno de los reportes de series de casos más extensos desarrollado a nivel nacional.
Background: Dermatofibrosarcoma protuberans (DFSP) is a rare skin sarcoma barely described in regional literature. This paper described and analyzed the clinical and histopathologic features of DFSP treated in our center. Methods: Retrospective study of all cases of primary DFSP, confirmed with biopsy between 2002 and 2016 in the Department of Pathology of the University of Chile Clinical Hospital (HCUCh). Results: The sample had 18 cases, 67% female, the average age of diagnosis was 45.5 years. The 28% of DFSP were located on the trunk, 22% lower limbs, 22% upper limbs, 22% inguino-genital region and 6% on the cervical region. A 22% of the biopsies were sent with presumptive clinical diagnosis of DFSP. On histopathology, all DFSP were composed of spindle- shaped cells distributed in storiform pattern, infiltrating to the subcutaneous adipose tissue. Immunohistochemistry was performed in 7 cases, being positive for CD34 in all of these samples and negative for factor XIIIa. In 50% of cases surgical margins were positive. Conclusions: Our results were similar to international publications. Higher frequency was observed in females and a delayed clinical diagnosis. There was a low index of clinical suspicion of DFSP, often confused with benign conditions, which could have influenced the high frequency of positive margins found in the study. This paper is one of the most extensive reports of case series developed nationwide.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/patologia , Neoplasias Cutâneas/epidemiologia , Biópsia , Imuno-Histoquímica , Chile , Epidemiologia Descritiva , Estudos Retrospectivos , Dermatofibrossarcoma/epidemiologia , Distribuição por Idade e SexoRESUMO
Dermatofibrosarcoma protuberans (DFSP) is a locally invasive neoplasia with a pattern of infiltrative growth that leads to extended resections. To avoid unnecessary resections and spare tissues, its treatment requires an adequate assessment of the margins. We present a case where artificial dermis (Matriderm®) was used followed by skin graft for reconstruction. We present a 50-year-old woman with a DFSP in the occipital region. She was referred to us after a first surgery with positive margins. A wide local excision with a 2-cm margin was performed and periosteal tissue was also removed, which led to exposure of the skull. Matriderm was placed on the bone surface and dressings were changed every other day. Meanwhile, margins were evaluated by the complete circumferential and peripheral deep margin assessment (CCPDMA) and were positive for DFSP in the superior margin. After 4 weeks the area was completely covered by granulation tissue and a new resection followed by reconstruction with a skin graft was performed. With regard to the difficulties in the margin assessment in DFSP, we present artificial dermis (Matriderm) as an option for reconstructive surgery in these patients, especially when a skin graft cannot be performed as a first option.
Assuntos
Colágeno , Dermatofibrossarcoma/cirurgia , Elastina , Neoplasias de Cabeça e Pescoço/cirurgia , Couro Cabeludo , Neoplasias Cutâneas/cirurgia , Transplante de Pele , Dermatofibrossarcoma/patologia , Feminino , Neoplasias de Cabeça e Pescoço/patologia , Humanos , Pessoa de Meia-Idade , Neoplasias Cutâneas/patologia , Pele ArtificialRESUMO
INTRODUÇÃO: Dermatofibrossarcoma Protuberante (DFSP) é um tumor de pele raro e de malignidade intermediária, com baixo potencial metastático, mas alta taxa de recorrência após tratamento cirúrgico. O tratamento clássico é a ressecção alargada, com margens variáveis. Muitos trabalhos descreveram os resultados da cirurgia micrográfica de Mohs no tratamento desta afecção. O objetivo deste estudo retrospectivo é verificar se a ressecção alargada constitui um método confiável no tratamento do DFSP. MÉTODO: Entre agosto de 1968 e setembro de 2013, 31 lesões foram ressecadas em 30 pacientes com DFSP. Todos os pacientes foram submetidos à excisão cirúrgica radical, com remoção de 3 cm de tecido sadio nas margens laterais e com a margem profunda incluindo uma estrutura anatômica não infiltrada pelo tumor. Os seguintes aspectos foram estudados: gênero, idade, local da lesão, tratamento prévio e características peculiares da proservação. RESULTADOS: Dezenove (63,3%) pacientes eram do sexo masculino e 11 (37,7%), do feminino. A média de idade da apresentação foi de 40,9 anos. As lesões estavam localizadas em tronco (61,3%), cabeça (22,6%), membros superiores (6,4%), membros inferiores (6,4%) e pescoço (3,3%). Tratamento prévio não foi observado em 58,1% dos pacientes. Um paciente (3,3%) evoluiu com recidivas e óbito, em decorrência do tratamento cirúrgico; três (10,0%) faleceram por outras causas. CONCLUSÕES: A ressecção alargada com margens de 3 cm, com remoção de estrutura anatômica sadia, constitui método eficiente no tratamento do DFSP.
INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor with intermediate malignancy and low metastatic potential, but a high recurrence rate after surgical treatment. The classical treatment is extended resection with varying margins. Many studies have described Mohs micrographic surgery for treatment of this disease. This retrospective study was to verify if extended resection is a reliable DFSP treatment method. METHOD: A total of 31 lesions were resected in 30 patients with DFSP between August 1968 and September 2013. All patients underwent radical surgical excision, with removal of 3 cm of healthy tissue on the lateral margins and with deep margin including an anatomical structure without tumor infiltration. Analyzed patient characteristics included sex, age, tumor site, previous treatment, and peculiar characteristics observed during the follow-up period. RESULTS: Nineteen (63.3%) patients were male and 11 (37.7%) female. Their average age at tumor presentation was 40.9 years. The tumors were located on the trunk (61.3%), head (226%), upper limbs (6.4%), lower limbs (6.4%), and neck (3.3%). No previous treatment was reported in 58.1% of the patients. One patient (3.3%) developed recurrence and died due to the surgical treatment; three patients (10.0%) died from other causes. CONCLUSIONS: Extended resection with 3-cm margins and removal of healthy anatomical structures is an effective treatment for DFSP.
Assuntos
Humanos , Masculino , Feminino , Adulto , História do Século XXI , Sarcoma , Neoplasias Cutâneas , Ferimentos e Lesões , Coleta de Dados , Estudos Retrospectivos , Dermatofibrossarcoma , Estudo de Avaliação , Tumores de Células Gigantes , Sarcoma/cirurgia , Sarcoma/patologia , Neoplasias Cutâneas/cirurgia , Ferimentos e Lesões/cirurgia , Ferimentos e Lesões/patologia , Coleta de Dados/métodos , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/patologia , Tumores de Células Gigantes/cirurgia , Tumores de Células Gigantes/patologiaRESUMO
We present a typical case of dermatofibrosarcoma protuberans with local recurrence 2 months after surgery and, motivated by this patient, make a review of the most important aspects. This is a rare tumor and we call special attention to the fact that its recurrence is extremely frequent, so there is absolute need to observe these patients periodically after surgery.
RESUMO
El dermatofibrosarcoma protuberans (DFSP) atrófico congénito es un tumor extremadamente poco frecuente, con contados reportes en la literatura mundial. Habitualmente el diagnóstico se demora años y se confunde con diversas marcas de nacimiento o con otras lesiones que cursan con atrofia cutánea. El comportamiento biológico sería, sin embargo, similar al de las otras formas de DFSP que se presentan en el niño y en el adulto, con alto riesgo de recurrencia tras la resección tumoral, por lo cual es importante conocer el aspecto para sospechar el diagnóstico. El estudio histopatológico se debe complementar con la inmunotinción con CD34, un marcador de utilidad no solo para aclarar el diagnóstico, sino también para guiar el tratamiento en la Cirugía de Mohs, que es en la actualidad el tratamiento de elección. La patogenia del DFSP se relacionaría con una mutación genética que provocaría una sobreproducción del factor de crecimiento derivado de las plaquetas que llevaría a un crecimiento celular maligno estimulado autocrinamente. El principal diagnóstico diferencial del DFSP atrófico congénito, tanto clínico como histopatológico, es el hamartoma dendrocítico dérmico tipo medallón, tumor congénito benigno descrito el año 2004. Presentamos el caso de un niño a quien se le diagnosticó un DFSP atrófico congénito a los 13 años de edad, después de haber sido reiteradamente interpretado como una lesión sin importancia. En este caso, se realizó resección con márgenes amplios, porque la ubicación anatómica lo permitía. En el control a los 18 meses después del tratamiento, el paciente no ha presentado recurrencias.
Congenital atrophic dermatofibrosarcoma protuberans is an extremely rare tumor, with few reports in world literature. Usually the diagnosis take a lot of years and get confused with many birthmarks or other lesions that present with cutaneous atrophy. However, the biological behavior would be similar to other forms of dermatofibrosarcoma protuberans (DFSP) shown in children and adults, with high risk of recurrence after surgical resection, that is why is so important to know the aspect to suspect the diagnosis. The histopathological study is complemented by the CD34 inmuno staining, used to clarify the diagnosis and to guide the treatment in Mohs micrographic surgery, which is currently the treatment of choice. The pathogenesis of DFSP would be related to a genetic mutation that induce an overproduction of platelet-derived growth factor that would lead to autocrine-stimulated malignant cell growth. The main differential diagnosis, clinical and histological, is the medallion-like dermal dendrocyte hamartoma, a congenital tumor first described in 2004. We report the case of a child who was diagnosed with a congenital atrophic dermatofibrosarcoma protuberans at the age of 13, after been repeatedly interpreted as a lesion without any importance. In this case, a resection with wide margins was made, because the anatomical location allowed it. The patient has not shown any recurrence of the tumor after 18 months of treatment.
Assuntos
Humanos , Masculino , Adolescente , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/diagnóstico , Cirurgia de Mohs , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/diagnóstico , Diagnóstico Diferencial , Dermatofibrossarcoma/congênito , Neoplasias Cutâneas/congênito , 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
El Tumor de Bednard es una rara forma de presentación del Dermatofibrosarcoma Protuberans, clasificado como un sarcoma de bajo grado con alta agresividad local y tendencia a la recurrencia cuando no presenta márgenes quirúrgicos adecuados. Presenta una difícil aproximación inicial y notoria demora en el correcto diagnóstico, lo que muchas veces influye negativamente en su pronóstico. Presentamos en el siguiente reporte un paciente de 24 años con diagnóstico de Tumor de Bednard, que se presentó como un tumor sólido subcutáneo de larga data.
Bednard tumor is a rare form of presentation of dermatofibrosarcoma protuberans. It is classified as a low-grade sarcoma with high local aggressiveness and relapse tendency when inadequate surgical margins are present after surgery. Diagnosis is difficult and shows a marked delay in the correct diagnosis, affecting the prognosis. We present a case-report of a patient with the diagnosis of Bednard tumor presented as a subcutaneous long standing solid tumor.
Assuntos
Humanos , Masculino , Adulto Jovem , Dermatofibrossarcoma/cirurgia , Dermatofibrossarcoma/diagnóstico , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/diagnósticoRESUMO
Se presenta un caso clínico atendido en el servicio de Cirugía General del Centro de Diagnóstico Integral La Victoria, municipio Caroní, Estado Bolívar, Venezuela. La paciente acudió a consulta con un tumor de partes blandas, localizado en pared abdominal, recidivante, de tres años de evolución, irregular, de aproximadamente 8 cm de diámetro, indoloro, con zonas rojizas. El diagnóstico definitivo se obtiene a través del informe histopatológico, de la lesión. El único tratamiento eficaz, es el quirúrgico. Siendo la cirugía micrográfica de Mohs la técnica de elección (AU)
It is presented a clinical case assisted at the General Surgery Service of La Victoria Integrated Diagnostic Centers, Caroni municipality, Bolivar State, Venezuela. The patient went to consultation presenting a soft-tissue tumor, located in the abdominal wall, recurrent, of three years of evolution, irregular, approximately 8 cm in diameter, painless, with reddish areas. The definitive diagnosis is obtained through the histopathological report of the lesion. The only effective treatment is surgical, being Mohs Micrographic Surgery the technique of choice (AU)