Insuficiencia cardíaca como presentación de sarcoma cardíaco: caso clínico y revisión de la literatura / Cardiac sarcoma associated to heart failure: case report and review

RESUMEN: Los tumores cardíacos primarios malignos son infrecuentes en la práctica clínica. Suelen ser oligosintomáticos y de diagnóstico tardío lo que tiene como consecuencia una alta mortalidad. El caso clínico que se presenta a continuación es un ejemplo de esta patología, que debe ser especialmente considerada cuando la insuficiencia cardíaca no tiene una etiología clara. Se describen los hallazgos clínicos y de laboratorio, como también los resultados de estudios de imágenes y la histología. Se ilustra los hallazgos en la operación y la evolución clínica del paciente. Se incluye una revisión de la literatura.

ABSTRACT: Malignant primary cardiac tumors are infrequent in clinical practice. They are usually oligosymptomatic and a late diagnosis leads to a high mortality rate. The clinical case of a patient presenting with heart failure with unclear etiology is presented. Clinical and laboratory findings are described along with the results of imaging studies. Findings at the time of surgery and histopathological characteristics are illustrated. A review of the literature is included.

Malignant uterine neoplasms attended at a brazilian regional hospital: 16-years profile and time elapsed for diagnosis and treatment / Neoplasias malignas do útero atendidas em hospital regional brasileiro: Perfil em 16 Anos e tempo dispendido até diagnóstico e tratamento

Abstract Objective The present study aims to evaluate the profile of endometrial carcinomas and uterine sarcomas attended in a Brazilian cancer center in the period from 2001 to 2016 and to analyze the impact of time elapsed fromsymptoms to diagnoses or treatment in cancer stage and survival. Methods This observational study with 1,190 cases evaluated the year of diagnosis, age-group, cancer stage and histological type. A subgroup of 185 women with endometrioid histology attended in the period from 2012 to 2017 was selected to assess information about initial symptoms, diagnosticmethods, overall survival, and to evaluate the influence of the time elapsed from symptoms to diagnosis and treatment on staging and survival. The statistics used were descriptive, trend test, and the Kaplan- Meier method, with p-values < 0.05 for significance. Results A total of 1,068 (89.7%) carcinomas (77.2% endometrioid and 22.8% nonendometrioid) and 122 (10.3%) sarcomas were analyzed, with an increasing trend in the period (p < 0.05). Histologies of non-endometrioid carcinomas, G3 endometrioid, and carcinosarcomas constituted 30% of the cases. Non-endometrioid carcinomas and sarcomas weremore frequently diagnosed in patients over 70 years of age and those on stage IV (p < 0.05). The endometrioid subgroup with 185 women reported 92% of abnormal uterine bleeding and 43% diagnosis after curettage. The average time elapsed between symptoms to diagnosis was 244 days, and between symptoms to treatment was 376 days, all without association with staging (p = 0.976) and survival (p = 0.160). Only 12% of the patients started treatment up to 60 days after diagnosis. Conclusion The number of uterine carcinoma and sarcoma cases increased over the period of 2001 to 2016. Aggressive histology comprised 30% of the patients and, for endometrioid carcinomas, the time elapsed between symptoms and diagnosis or treatment was long, although without association with staging or survival.

Resumo Objetivo O presente estudo avaliou o perfil dos carcinomas endometriais e sarcomas uterinos atendidos em um centro brasileiro de câncer no período de 2001 a 2016, e avaliou o impacto do tempo decorrido entre os sintomas até o diagnóstico ou tratamento no estadiamento e sobrevida pelo câncer. Métodos Estudo observacional com 1.190 casos que analisou o ano do diagnóstico, faixa etária, estágio e tipo histológico do câncer. Um subgrupo de 185 mulheres com histologia endometrioide e atendidas no período de 2012 a 2017 foi selecionado para avaliar informações sobre sintomas iniciais, métodos de diagnóstico, sobrevida global e para analisar a relação entre o tempo decorrido a partir dos sintomas até o diagnóstico e tratamento no estadiamento e sobrevida. Foram realizadas análises estatísticas descritiva, de tendência linear e de sobrevida pelo método de Kaplan-Meier, com valores de p < 0,05 para significância. Resultados Os casos estudados de acordo com a histologia foram 1.068 (89,7%) carcinomas (77,2% endometrioides e 22,8% não endometrioides) e 122 (10,3%) sarcomas, com tendência crescente no período (p < 0,05). Histologias de carcinomas não endometrioides, G3 endometrioides e carcinossarcomas consistiram em 30% dos casos. Carcinomas não endometrioides e sarcomas forammais frequentemente diagnosticados em pacientes acima de 70 anos de idade e em estágio IV (p < 0,05). O subgrupo com185 mulheres com carcinoma endometrioide apresentou 92% de sangramento uterino anormal e 43% de diagnóstico após curetagem. O tempo médio decorrido entre os sintomas e o diagnóstico foi de 244 dias e entre os sintomas e o tratamento, 376 dias, todos sem associação com estadiamento (p = 0,976) e sobrevida (p = 0,160). Apenas 12% das pacientes iniciaram o tratamento em até 60 dias após o diagnóstico. Conclusão O número de casos de carcinomas e sarcomas uterinos aumentaram no período de 2001 a 2016. A histologia agressiva compreendeu 30% dos pacientes e, no caso dos carcinomas endometrioides, o tempo decorrido entre os sintomas e o diagnóstico ou tratamento foi longo, embora sem associação com estadiamento ou sobrevida.

Sarcomas de partes blandas en pacientes menores de 2 años. Serie de casos / Soft tissue sarcomas in infants. Case series

Los sarcomas de partes blandas en menores de 2 años son infrecuentes. Durante 2007-2017, 445 pacientes con sarcomas de partes blandas fueron tratados en nuestra Institución; 6 (el 0,5 %) eran menores de 2 años. Se analizaron los resultados clínicos y oncológicos en este grupo. La edad media de diagnóstico fue 15 meses. Cuatro eran varones y 2, mujeres. El seguimiento promedio fue 29 meses. El fibrosarcoma (n = 4) fue la variedad más frecuente. Cinco fueron tratados con cirugía de conservación del miembro; al restante se le realizó amputación. Todos realizaron tratamiento adyuvante con quimioterapia. La supervivencia a 24 meses fue del 100 %. Dos pacientes presentaron recidiva local; ambos casos, antes de los 24 meses.El tratamiento quirúrgico asociado a la quimioterapia impresiona ser la mejor opción terapéutica. La proporción de recurrencia local es alta para este grupo de pacientes luego de la cirugía de conservación del miembro.

Soft tissue sarcomas in children under 2 years of age are infrequent. During 2007-2017, a total of 445 patients diagnosed with soft tissue sarcomas were treated at our institution, 6 (0.5 %) were under 2 years. We analysed clinical and oncologic outcomes in this select group. The mean age of diagnosis was 15 months. Four patients were male and 2 female. The mean follow-up time was 29 months. Fibrosarcoma (n = 4) was the most frequent diagnosis. Five patients were treated with limb salvage surgery, and the remaining one had to undergo amputation. All patients received adjuvant treatment with chemotherapy. The 24-month survival rate was 100 %. Two patients presented a local recurrence before 24-months follow-up. Surgical treatment associated with chemotherapy seems to be the best therapeutic option. Local recurrence rate after limb salvage surgery is high for this group of patients

Adult primary liver sarcoma: systematic review / Sarcoma primário de fígado em adultos: revisão sistemática

ABSTRACT Introduction: primary liver sarcoma is a rare type of tumor, more common in children. Among adults, it represents a spectrum of neoplasms with reserved prognosis. There is no consensus on the treatment of choice of these lesions, justifying a systematic review of the literature on treatment options, prognostic factors, and survival. Material/Methods: a systematic review of articles published in Pubmed, Medline, LiLacs e SciElo, from 1966 to March/2019, presenting the keywords: primary-liver-sarcoma and primary-hepatic-sarcoma was undertaken. Studies including patients older than 18 years, and published in English, Portuguese and Spanish were included. Case reports, metastatic tumors and multiple oncologic diagnosis were excluded. The initial search listed 1,318 articles. 1,206 did not meet the inclusion criteria. After reviewing 112 eligible articles, 15 were selected (14 case series and 1 retrospective-cohort). Results: proposed treatment modalities for primary liver sarcoma included surgery and/or chemotherapy and/or radiotherapy or liver transplantation. The most common histological types were angiosarcoma (32%), leiomyosarcoma (29%), epithelioid hemangioendothelioma (15%) and embryonal sarcoma (7%). Histology, degree of differentiation and R0 resection were mentioned positive prognostic factors. Median survival ranged from two to 23 months. Five-year survival rate varied from 0% to 64%, on average 21%. Conclusion: surgical resection (R0 resection) is the main treatment for primary liver sarcomas. Development of effective systemic therapies are required to improve prognosis of patients harboring this type of tumor.

RESUMO Introdução: o sarcoma primário do fígado é uma neoplasia rara, que acomete mais frequentemente crianças. Nos adultos, representa espectro de neoplasias de prognóstico reservado. Entretanto, em nenhuma faixa etária há consenso sobre a terapêutica de escolha dessas lesões, motivando revisão sistemática com objetivo de elencar opções de tratamento, fatores prognósticos e sobrevida. Material/Método: realizamos revisão sistemática dos artigos publicados nas bases de dados Pubmed, Medline, LiLacs e Scielo, de 1966 a março/2019, contendo as palavras-chaves: primary-liver-sarcoma e primary-hepatic-sarcoma. Foram incluídos estudos que incluíram pacientes com idade a partir de 18 anos e publicados em inglês, português e espanhol. Relatos de caso, tumores metastáticos e pacientes com múltiplos diagnósticos oncológicos foram excluídos. Foram encontrados inicialmente 1.318 artigos, desses, 1.206 foram excluídos por estarem fora dos critérios de inclusão. Dos 112 artigos analisados e discutidos, 15 foram incluídos nesse artigo (14 séries de casos e 1 estudo de coorte retrospectivo). Resultado: os tratamentos propostos para o sarcoma primário do fígado em adultos incluíram cirurgia e/ou quimioterapia e/ou radioterapia ou transplante hepático. Os tipos histológicos mais frequentemente relados foram angiossarcoma (32%), leiomiossarcoma (29%), hemangioendotelioma epitelioide (15%) e sarcoma embrionário (7%). Tipo histológico, grau de diferenciação e resseção R0 foram os principais fatores de bom prognóstico. A sobrevida média total variou de dois a 23 meses. A sobrevida em cinco anos variou entre 0 e 64%, em média 21%. Conclusão: ressecção cirúrgica R0 é a base do tratamento dos sarcomas primários do fígado. Esse grupo heterogêneo de tumores requer desenvolvimento de terapias sistêmicas efetivas para melhoria do prognóstico.

Resultados del tratamiento quirúrgico de sarcomas de partes blandas en adultos / Outcomes of surgical treatment of soft-tissue sarcomas in adults

Antecedentes: los sarcomas de partes blandas son raros tumores mesenquimáticos con varios tipos histológicos y diferentes comportamientos clínicos. Objetivo: describir las características clínicas y patológicas, así como los resultados del tratamiento quirúrgico de una serie de pacientes operados por sarcomas de partes blandas. Material y métodos: se realizó una revisión retrospectiva de las historias clínicas de 2403 pacientes operados entre octubre de 2014 y abril de 2018. Veintidós de ellos (0,91%) presentaron sarcomas de partes blandas. Resultados: el promedio de edad fue 52 años (rango 19-92), 13 (59%) eran mujeres. La localización de los tumores fue: miembro inferior en 12 casos, cabeza y cuello en 5, tronco en 3 y miembro superior en 2. Catorce casos (63,6%) fueron tumores de alto grado. Los tipos patológicos fueron: sarcoma pleomórfico 7 (32%), sarcoma sinovial 4 (18%), liposarcoma 3 (14%), otros 4 (36 %). Todos fueron extirpados en forma completa y en 5 casos (35,7%) requirieron amputación: 4 de miembro inferior y uno superior. Según el tamaño y la ubicación del tumor se emplearon diferentes procedimientos reconstructivos, incluyendo 3 colgajos libres. Aquellos pacientes con lesiones de alto grado o con márgenes histológicamente positivos recibieron radioterapia posoperatoria. Durante el seguimiento (promedio 16 meses), seis pacientes presentaron recidivas locales, cuatro con metástasis pulmonares sincrónicas, todos ellos con tumores de alto grado. La sobrevida global fue del 86,4%. Conclusión: los sarcomas de partes blandas son neoplasias infrecuentes e invasivas, ampliamente distribuidas, que requieren procedimientos quirúrgicos agresivos. Es necesario tratamiento adyuvante en casos seleccionados y seguimiento periódico debido a la alta tasa de recidiva y metástasis a distancia.

Background: Soft-tissue sarcomas (STS) are rare mesenchymal tumors with several histologic subtypes and different clinical patterns. Objective: The aim of this study was to describe the clinical and pathological characteristics and surgical outcomes of a series of patients with STS. Material and methods: The clinical records of 2403 undergoing surgery between October 2014 and April 2018 were retrospectively reviewed. Twenty-two patients (0.91%) presented STS. Results: Mean age was 52 years (range: 19-92) and 13 (59%) were women. The tumors were located in the lower extremities in 12 cases, head and neck in five, trunk in three and upper extremities in two. Fourteen cases (63.6%) were high-grade tumors. Pleomorphic sarcoma was the most common histologic type (32%) followed by synovial sarcoma (18%), liposarcoma (14%), and other types (36%). All the tumors were completely resected and five patients (35.7%) required amputation, four in the lower extremity and on in the upper extremity. Different reconstructive procedures were performed according to tumor size and location, including three free flaps. Those patients with high-grade sarcomas or with positive margins received postoperative radiotherapy. After a mean follow-up of 16 months, six patients presented local recurrences and four patients had synchronous metastatic disease in the lungs; all these patients had high-grade tumors. Overall survival was 86.4%. Conclusion: STS are rare and invasive neoplasms, widely distributed, requiring aggressive and occasionally complex surgical procedures. It is necessary to consider adjuvant treatments in selected cases and to maintain regular follow-up due to the high rate of recurrences and distant metastases.

Outcomes after surgical resection of primary non-myxoma cardiac tumors

Abstract Objective: Primary cardiac tumors are rare lesions with different histological type. We reviewed our 17 years of experience in the surgical treatment and clinical results of primary non-myxoma cardiac tumors. Methods: Between July 2000 and February 2017, 21 patients with primary cardiac tumor were surgically treated in our institution. The tumors were categorized as benign non-myxomas and malignants. Data including the demographic characteristics, details of the tumor histology and grading, cardiac medical and surgical history, surgical procedure of the patients were obtained from the hospital database. Results: Eleven patients were diagnosed with benign non-myxoma tumor (male/female:7/4), ranging in age from 10 days to 74 years (mean age 30.9±26.5 years). Papillary fibroelastoma was the most frequent type (63.6%). There were two early deaths in benign group (all were rhabdomyoma), and mortality rate was 18%. The mean follow-up period was 69.3±58.7 months (range, 3 to 178 months). All survivals in benign group were free of tumor-related symptoms and tumor relapses. Ten patients were diagnosed with malignant tumor (sarcoma/lymphoma:8/2, male/female:3/7), ranging in age from 14 years to 73 years (mean age 44.7±18.9 years). Total resection could be done in only three (30%) patients. The mean follow-up period was 18.7±24.8 months (range, 0-78 months). Six patients died in the first 10 months. Conclusion: Complete resection of the cardiac tumors, whenever possible, is the main goal of surgery. Surgical resection of benign cardiac tumors is safe, usually curative and provides excellent long-term prognosis. On the contrary, malignant cardiac tumors still remain highly lethal.

Evaluation of adjuvant chemotherapy in feline injection site-associated sarcoma / Avaliação da quimioterapia no tratamento do sarcoma de aplicação felino

Feline Injection Site-Associated Sarcoma (FISS) is a neoplasm that implies in reduction of quality of life and overall survival in feline patients. A retrospective study of 13 cases of FISS was conducted to evaluate the efficacy of surgical treatment associated to chemotherapy with doxorubicin or carboplatin. Local recurrence occurred in all patients. Patients treated with surgery and chemotherapy presented a longer overall survival and disease-free interval when compared to those that solely received surgical treatment, although no statistical significance was observed (p= 0.3360 and 0.7506, respectively). Surgery remains as the main option for FISS treatment. Further prospective studies with larger samples are warranted to investigate the benefit of chemotherapy for this neoplasm.(AU)

O Sarcoma de Aplicação Felino (SAF) é uma neoplasia associada a redução na qualidade de vida e sobrevida global. O objetivo deste estudo foi avaliar a eficácia da quimioterapia associada à cirurgia no manejo do SAF. Estudo retrospectivo de 13 pacientes com SAF submetidos à cirurgia isolada ou associada a quimioterapia com carboplatina ou doxorrubicina. Recorrência local ocorreu em todos os pacientes. Pacientes tratados com cirurgia e quimioterapia apresentaram maior sobrevida global e intervalo livre de doença quando comparados àqueles que receberam apenas tratamento cirúrgico, mas não foi observada diferença estatística (p=0,3360 e 0,7506, respectivamente). A cirurgia continua sendo a principal opção para o tratamento do SAF. Estudos prospectivos são necessários para investigação do real benefício da quimioterapia para esta neoplasia.(AU)

Morcelamento elétrico de tecido uterino: qual o risco potencial de disseminação de neoplasias malignas? / Electrical uterine tissue morbidity: what is the potential risk of malignant neoplasm spread?

O Food and Drug Administration (FDA) fez um alerta de segurança em relação ao risco potencial de disseminação de neoplasias malignas pelo uso do morcelador elétrico durante procedimentos minimamente invasivos. Esse artigo visa avaliar as evidências científicas sobre morcelamento uterino em relação ao risco de disseminação de câncer, sendo abordados a incidência de sarcoma uterino entre as mulheres previamente diagnosticadas com doença uterina benigna aparente, a possibilidade de avaliação pré-operatória desse risco, o posicionamento das principais sociedades médicas em relação à utilização do morcelador elétrico e as repercussões na prática clínica após as recomendações do FDA.(AU)

The US Food and Drug Administration (FDA) released a safety communication advising the potential risk of inadvertent spread of cancer cellsafter the use of electrical morcellator in minimally invasive procedures. This article aims to evaluate the scientific evidence regarding the risk of spread of cancer after uterine morcellation, the incidence of uterine sarcoma among women diagnosed with benign uterine disease, the possibility of preoperative assessment of this risk, the main medical societies positions statements regarding theuse of electric morcellation and the FDA recommendation`s impact on clinical practice. A review of the literature and a research of the main medical societies positions statements regarding the use of electric morcellation in minimally invasive surgeries were done.(AU)

Sarcoma miofibroblástico posradioterapia / Radiation-induced myofibroblastic sarcoma

Paciente de 51 años, varón, fumador y alcohólico moderado, con síntomas de disfonía intermitente que debuta con una adenopatía cervical metastásica de carcinoma epidermoide, sin hallazgos del tumor primario. Es intervenido quirúrgicamente siéndole realizada una disección cervical unilateral y posteriormente es tratado con quimiorradioterapia concomitante. A los 4 años del final del tratamiento oncológico, sin recidiva local ni aparición del tumor primario, el paciente debuta con disnea de presentación subaguda. Tras la exploración física y pruebas diagnósticas, se constata una nueva tumoración en laringe que resulta ser un "sarcoma miofibroblástico de bajo grado". Según los criterios de Cahan, este tumor, de estirpe histológica poco frecuente en laringe, hace pensar que probablemente podría ser inducido por la radiación. Se discuten los criterios de causalidad entre radiación y oncogenesis y se resumen las características de los tumores radioinducidos.

We report a clinical case about a male patient, smoker and alcoholic, that debuts with a neck metastasis of an epidermoid carcinoma of unknown origin. He was submitted to a cervical dissection and treated with adjuvant combined chemo and radio therapy. Four years after finishing the oncologic treatment without tumor recurrence or appearence of the primary tumor, he begins with sub acute dyspnea. After physical examination and imaging, a new larynx tumor was found, "low grade myofibroblastic sarcoma". Under Cahan criteria, this tumoral kind, rare in larynx, was probably induced by the local radiotherapy. We discuss briefly the causality between radiation and oncogenesis and the characteristics of the radio induced tumors.

Reconstrução torácica com retalhos do músculo reto abdominal e grande dorsal: relato de casos / Chest wall reconstruction using abdominal rectus and latissimus dorsi muscle flaps: case report

INTRODUÇÃO: A reconstrução da parede torácica ainda permanece como um dos grandes desafios da cirurgia plástica reparadora. O presente estudo tem como objetivo apresentar o relato de pacientes submetidos à reconstrução de parede torácica com a utilização de retalhos miocutâneos, após ressecções de parede por tumores/osteomielite. MÉTODOS: Foram incluídos quatro pacientes, três deles portadores de osteomielite e o último apresentando-se com sarcoma, todas as afecções acometendo a parede torácica. Foram submetidos à ressecção de parede e reconstrução com retalhos miocutâneos do grande dorsal (dois casos) e reto abdominal (dois casos). RESULTADOS: Os retalhos utilizados foram suficientes para cobertura cutâneo-muscular e mantiveram boa vitalidade. Em apenas um caso, houve sofrimento parcial do retalho. Não houve recidiva precoce da doença. A estabilidade da caixa torácica foi preservada. CONCLUSÕES: A utilização dos retalhos citados no reparo dos defeitos torácicos mostrou-se satisfatória na intenção de prover revestimento cutâneo e músculo bem vascularizado, este fundamental no combate aos quadros infecciosos locais.

INTRODUCTION: Chest wall reconstruction remains one of the great plastic surgery repair challenges. The present work aims to report on cases of chest wall reconstruction using myocutaneous flaps after wall resection due to tumor/osteomyelitis. METHODS: Four patients were included, among which three presented with osteomyelitis and the other presented with sarcoma; both of these conditions affected the chest wall. Each patient underwent wall resection and reconstruction using myocutaneous flaps from the latissimus dorsi (two cases) and abdominal rectus (two cases). RESULTS: The flaps used were sufficient for skin-muscle covering and maintained good vitality. Partial flap injury occurred in one case. Chest cavity stability was preserved. CONCLUSIONS: The use of the above flaps to repair chest defects was satisfactory with the aim of covering the skin and providing well-vascularized muscles, the latter of which was fundamental to preventing local infection.

Sarcoma pleomórfico gigante de la mama / Giant pleomorphic breast sarcoma

Abstract It is reported the case of a female patient 50 years old who, after failed attempts at diagnosis, arrives at breast surgery service of the E.S.E. Hospital Universitario del Caribe with history of five months consistent inthe excessive growth of a mass in right breast; patient received radical mastectomy plus reconstruction withlatissimus dorsi muscle flap. Following excision of tumor mass of 6,500 g is performed immunohistochemicaldiagnosis of a giant pleomorphic sarcoma.

Resumen introducción: Los tumores derivados del tejido conectivo interlobulillar de la mama son en mayor proporción malignos y, en virtud a su ubicación, en la actualidad reciben el nombre de "sarcomas" de la mama. Caso clínico: Se reporta el caso de una paciente femenina de 50 años de edad quien, posterior a fallidosintentos de diagnóstico, acude al servicio de cirugía de mama de la E.S.E. Hospital Universitario del Caribecon un cuadro de cinco meses de evolución consistente en el crecimiento desmedido de una masa en mamaderecha; la paciente recibió mastectomía radical más reconstrucción con colgajo de músculo dorsal ancho.Posterior a exéresis de masa tumoral de 6.500 g de peso se lleva a cabo diagnóstico inmunohistoquímico deun sarcoma pleomórfico gigante de la mama.

Perfurador baseado em transposição de retalho estenocelidomastoide para defeitos da região posterior do pescoço: uma nova alternativa / Perforator based sternocleidomastoid transposition flap for nape of neck defect: a novel option

Retalhos do músculo esternocleidomastoideo têm sido descritos na literatura para reconstrução dos defeitos da cavidade oral e tratamento da síndrome de Frey. Apesar de largamente estudado, esses retalhos não são utilizados com frequência para reconstruções na região de cabeça e pescoço devido limitações como tamanho reduzido, camadas inseguras, contorno da deformidade no pescoço e questões de segurança oncológica. Relata-se uso de perfurador baseado em transposição de retalho para defeito na região da posterior do pescoço, seguido por excisão de sarcoma de partes moles. Trata-se de alternativa válida para procedimentos de reconstrução como retalhos regionais de pedículo ou transferência de tecido livre em um grupo apropriado de pacientes.

Sternocleidomastoid musculocutaneous flaps have been described in the literature for reconstruction of oral cavity defects and treatment of Frey's syndrome. Although widely studied, it is not used routinely in head and neck reconstruction due to limitations like small size, unreliable skin paddle, contour deformity in the neck and the question of oncologic safety. We report use of perforator based musculocutaneous transposition flap for defect over nape of the neck, followed by excision of a soft tissue sarcoma. This constitutes a valid alternative to other reconstructive procedures like pedicled regional flaps or free tissue transfer in a suitable group of patients.

Comparative study of planned and unplanned excisions for the treatment of soft tissue sarcoma of the extremities

OBJECTIVE: Unplanned excision of soft tissue sarcomas is common because benign soft tissue lesions are very frequent. This study evaluated the impact of unplanned resections on overall survival, local recurrence and distant metastasis in patients with soft tissue sarcomas of the extremities. METHODS: In total, 52 patients who were diagnosed with soft tissue sarcomas between May 2001 and March 2011 were analyzed in a retrospective study. Of these patients, 29 (55.8%) had not undergone previous treatment and the remaining 23 (44.2%) patients had undergone prior resection of the tumor without oncological planning. All subsequent surgical procedures were performed at the same cancer referral center. The follow-up ranged from 6 to 122 months, with a mean of 39.89 months. Age, lesion size and depth, histological grade, surgical margins, overall survival, local and distant recurrence and adjuvant therapies were compared. RESULTS: Residual disease was observed in 91.3% of the re-resected specimens in the unplanned excision group, which exhibited greater numbers of superficial lesions, low histological grades and contaminated surgical margins compared with the re-resected specimens in the planned excision group. No differences were observed in local recurrence and 5-year overall survival between the groups, but distant metastases were significantly associated with planned excision after adjustment for the variables. CONCLUSIONS: There was no difference between patients undergoing unplanned excision and planned excision regarding local recurrence and overall survival. The planned excision group had a higher risk of distant metastasis, whereas there was a high rate of residual cancer in the unplanned excision group. .

Ressecção alargada no tratamento do dermatofibrossarcoma protuberante / Extended resection for treatment of dermatofibrosarcoma protuberans

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.