Evaluation of incidence and histolopathological findings of soft tissue sarcomas in genitourinary tract: Uludag university experience

ABSTRACT Purpose: In this study we aimed to review urological soft tissue sarcomas of genitourinary tract that were diagnosed in our institution and their prognostic factors for survival. Materials and Methods: The clinical and pathological records of 31 patients who had diagnosis of soft tissue sarcomas primarily originating from the genitourinary tract between 2005-2011 were reviewed. Results: The most common site was kidney (17 cases, 54.8%), and most common diagnosis was leiomyosarcoma (11 cases, 35.4%). A total of 24 patients (77.4%) had surgical excision. The surgical margins were positive in 7 patients who presented with local recurrence after primary resection. Twelve patients developed metastatic disease. During follow-up (range 9-70 month), 26 of the 31 patients (88.9%) were alive. Significant survival differences were found according to histological type (p: 0.001), with lower survival rates for malignant fibrous histiocytoma. The tumor size, the presence of metastasis at the time of diagnosis and tumor localization were not statistically significant for overall survival. Conclusions: In our series, prostate sarcomas, paratesticular rhabdomyosarcoma and malignant fibrous histiocytoma had poor prognosis, especially in patients presenting with metastatic disease.

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.

High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Children with High-Risk or Recurrent Bone and Soft Tissue Sarcomas

Despite increasing evidence that high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) might improve the survival of patients with high-risk or recurrent solid tumors, therapy effectiveness for bone and soft tissue sarcoma treatment remains unclear. This study retrospectively investigated the feasibility and effectiveness of HDCT/auto-SCT for high-risk or recurrent bone and soft tissue sarcoma. A total of 28 patients (18 high-risk and 10 recurrent) underwent single or tandem HDCT/auto-SCT between October 2004 and September 2014. During follow-up of a median 15.3 months, 18 patients exhibited disease progression and 2 died of treatment-related toxicities (1 veno-occlusive disease and 1 sepsis). Overall, 8 patients remained alive and progression-free. The 3-year overall survival (OS) and event-free survival (EFS) rates for all 28 patients were 28.7% and 26.3%, respectively. In the subgroup analysis, OS and EFS rates were higher in patients with complete or partial remission prior to HDCT/auto-SCT than in those with worse responses (OS, 39.1% vs. 0.0%, P = 0.002; EFS, 36.8% vs. 0.0%, P < 0.001). Therefore, careful selection of patients who can benefit from HDCT/auto-SCT and maximal effort to reduce tumor burden prior to treatment will be important to achieve favorable outcomes in patients with high-risk or recurrent bone and soft tissue sarcomas.

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. .

Mortality and Embolic Potential of Cardiac Tumors / Mortalidade e Potencial Embólico dos Tumores Cardíacos

Background: Cardiac tumors are rare, mostly benign with high embolic potential. Objectives: To correlate the histological type of cardiac masses with their embolic potential, implantation site and long term follow up in patients undergoing surgery. Methods: Between January 1986 and December 2011, we retrospectively analyzed 185 consecutive patients who underwent excision of intracardiac mass (119 females, mean age 48±20 years). In 145 patients, the left atrium was the origin site. 72% were asymptomatic and prior embolization was often observed (19.8%). The diagnosis was established by echocardiography, magnetic resonance and histological examination. Results: Most tumors were located in the left side of the heart. Myxoma was the most common (72.6%), followed by fibromas (6.9%), thrombi (6.4%) and sarcomas (6.4%). Ranging from 0.6cm to 15cm (mean 4.6 ± 2.5cm) 37 (19.8%) patients had prior embolization, stroke 10.2%, coronary 4.8%, peripheral 4.3% 5.4% of hospital death, with a predominance of malignant tumors (40% p < 0.0001). The histological type was a predictor of mortality (rhabdomyomas and sarcomas p = 0.002) and embolic event (sarcoma, lipoma and fibroelastoma p = 0.006), but not recurrence. Tumor size, atrial fibrillation, cavity and valve impairment were not associated with the embolic event. During follow-up (mean 80±63 months), there were 2 deaths (1.1%) and two recurrences 1 and 11 years after the operation, to the same cavity. Conclusion: Most tumors were located in the left side of the heart. The histological type was predictor of death and preoperative embolic event, while the implantation site carries no relation with mortality or to embolic event. .

Fundamento: Os tumores do coração são infrequentes, em sua maioria benignos e com alto potencial embólico. Objetivo: Correlacionar o tipo histológico do tumor cardíaco com seu potencial embólico, com o sítio de implantação e analisar a evolução tardia destes pacientes submetidos à cirurgia. Métodos: No período de dezembro de 1986 a setembro de 2011 foram retrospectivamente analisados 186 pacientes operados (119 do sexo feminino e idade média de 48 ± 20 anos). Foram 145 tumores de átrio esquerdo (77%), 72% dos pacientes assintomáticos e 19,8% com embolização prévia. O diagnóstico foi confirmado por ecocardiograma, ressonância magnética e exame histológico. Resultados: A maioria dos tumores situava-se nas câmaras esquerdas. O mixoma foi o mais frequente (72,6%), seguido dos fibromas (6,9%), trombos (6,4%) e sarcomas (6,4%). Seus tamanhos variaram de 0,6cm a 15 cm (média de 4,6 ± 2,5cm). Houve 37 embolizações prévias à operação (10,2% AVC, 4,8% IAM e 4,3% periférica). Foram 5,4% de óbito hospitalar, com predomínio nos tumores malignos (40% p < 0,0001). O tipo histológico foi preditor de mortalidade (rabdomioma e sarcomas p = 0,002) e de evento embólico (sarcomas, fibroelastoma e lipoma p = 0,006), porém não de recidiva. O tamanho tumoral, a fibrilação atrial, a cavidade e valva acometida não apresentaram relação com o evento embólico. Durante o seguimento (média de 80 ± 63 meses), houve 2 óbitos (1,1%) e duas recidivas tumorais 1 e 11 anos após a operação, ambas para a mesma cavidade. Conclusão: O tipo histológico foi preditor de óbito e de evento embólico pré-operatório, enquanto o sítio de implantação não. .

Descriptive analysis of and overall survival after surgical treatment of lung metastases / Analise descritiva e sobrevida global do tratamento cirurgico das metastases pulmonares

OBJECTIVE: To describe demographic characteristics, surgical results, postoperative complications, and overall survival rates in surgically treated patients with lung metastases. METHODS: This was a retrospective analysis of 119 patients who underwent a total of 154 lung metastasis resections between 1997 and 2011. RESULTS: Among the 119 patients, 68 (57.1%) were male and 108 (90.8%) were White. The median age was 52 years (range, 15-75 years). In this sample, 63 patients (52.9%) presented with comorbidities, the most common being systemic arterial hypertension (69.8%) and diabetes (19.0%). Primary colorectal tumors (47.9%) and musculoskeletal tumors (21.8%) were the main sites of origin of the metastases. Approximately 24% of the patients underwent more than one resection of the lesions, and 71% had adjuvant treatment prior to metastasectomy. The rate of lung metastasis recurrence was 19.3%, and the median disease-free interval was 23 months. The main surgical access used was thoracotomy (78%), and the most common approach was wedge resection with segmentectomy (51%). The rate of postoperative complications was 22%, and perioperative mortality was 1.9%. The overall survival rates at 12, 36, 60, and 120 months were 96%, 77%, 56%, and 39%, respectively. A Cox analysis confirmed that complications within the first 30 postoperative days were associated with poor prognosis (hazard ratio = 1.81; 95% CI: 1.09-3.06; p = 0.02). CONCLUSIONS: Surgical treatment of lung metastases is safe and effective, with good overall survival, especially in patients with fewer metastases. .

OBJETIVO: Descrever características demográficas, resultados operatórios, complicações pós-operatórias e taxa de sobrevida global em pacientes com metástases pulmonares tratados cirurgicamente. MÉTODOS: Análise retrospectiva de 119 pacientes submetidos a um total de 154 cirurgias de ressecção de metástase pulmonar entre 1997 e 2011. RESULTADOS: Do total de 119 pacientes, 68 (57,1%) eram do sexo masculino, e 108 (90,8%) eram brancos. A mediana de idade foi de 52 anos (variação, 15-75 anos). Nessa amostra, 63 pacientes (52,9%) apresentaram comorbidades, sendo as mais frequentes hipertensão arterial sistêmica (69,8%) e diabetes (19,0%). Tumores primários colorretais (47,9%) e musculoesqueléticos (21,8%) foram os principais sítios de origem das metástases. Aproximadamente 24% dos pacientes foram submetidos a mais de uma ressecção das lesões, e 71% fizeram tratamento adjuvante prévio à metastasectomia. A taxa de recidiva de metástase pulmonar foi de 19,3%. A mediana do intervalo livre de doença foi de 23 meses. A principal via de acesso usada foi toracotomia (78%), e o tipo de ressecção mais frequente foi em cunha e segmentectomia (51%). O índice de complicações pós-operatórias foi de 22% e o de mortalidade perioperatória foi de 1,9%. As taxas de sobrevida global em 12, 36, 60 e 120 meses foram, respectivamente, de 96%, 77%, 56% e 39%. A análise de Cox confirmou que complicações nos primeiros 30 dias pós-operatórios associaram-se a pior prognóstico (hazard ratio = 1,81; IC95%: 1,09-3,06; p = 0,02). CONCLUSÕES: O tratamento cirúrgico das metástases pulmonares oriundas de diferentes sítios tumorais é efetivo e seguro, com boa sobrevida global, especialmente nos casos com um menor número de lesões ...

Clinical Implication of Surgical Resection for the Rare Cardiac Tumors Involving Heart and Great Vessels

This study aimed to investigate the clinical implication of surgical resection for the malignancies of heart and great vessels. Between January 2001 and May 2011, a retrospective review of the results in 12 patients was conducted. There were 6 patients with primary cardiac tumor including leiomyosarcoma, angiosarcoma, undifferentiated type sarcoma and malignant fibrous histiocytoma. The remaining 6 patients had the metastatic tumors or adjacent invasion to the heart and great vessels. Six of seven patients who underwent complete resection had no evidence of recurrence. However, four of five patients who underwent incomplete resection or biopsy showed local recurrence or distant metastasis of residual tumor, and one of them required reoperation for recurred tumor. In-hospital mortality was 8.3% and the mean survival of all patients was 22.2 +/- 6.1 months. Survival of the incomplete resection group, except for the two biopsy cases, was 25.9 +/- 7.9 months, and there was no mortality in the complete resection group. Therefore, clinical outcomes in patients who had malignancies of the heart and great vessels may be improved when the aggressive and complete resection, or possible debulking for palliation, was performed. Moreover, adjuvant multimodality therapy may be imperative to prevent recurrence or metastasis, and to provide improved survival.

Prognostic factors in patients with jaw sarcomas

The aim of this study was to identify the prognostic factors related to the survival of patients with sarcomas of the jaw treated in the Dr. Eduardo Caceres Graziani National Institute for Neoplastic Diseases, Lima, Peru. Age, gender, delay in consultation, diagnostic delay, therapeutic delay, tumor size, tumor location, facial asymmetry, pain, treatment type, and histopathological diagnosis were all evaluated as possible prognostic factors that would influence survival in those with jaw sarcomas. In the analysis, the following was used: mortality tables, Kaplan-Meier's product-limit method, log-rank, and Breslow and Tarone-Ware tests; for the prognostic factors, Cox's Regression Model was used. The overall survival rate, with the patient being free from disease at two years, was 55 percent, and that at five years was 45 percent. In the independent analysis of the prognostic factors, four variables were statistically significant in influencing survival: gender (p = 0.043), histopathologic diagnosis (p = 0.019), tumor location (p = 0.019), and treatment type (p = 0.030). According to Cox's Regression Model for the multivariate analysis, statistically significant prognostic factors were: gender (p = 0.086), tumor location (p = 0.020), and treatment type (p = 0.092). Thus, the variables of gender, tumor location, and treatment type were determined to be predictive factors for prognosis of survival.

Sarcomas de partes moles de extremidades: análise da sobrevida, funcionalidade e qualidade de vida no Rio de Janeiro / Soft tissue sarcomas of the extremities: survival analysis, functionality and quality of life in Rio de Janeiro

Os sarcomas de partes moles de extremidades compreendem um grupo deneoplasias raras com características histopatológicas variadas. Apesar dos progressos no tratamento nas últimas décadas, com aumento das cirurgias preservadoras de membros emodalidades de radioterapia, ainda existe discussão sobre a associação dos fatores prognósticos importantes para a sobrevida. No planejamento terapêutico é esperado que as cirurgias preservadoras de membros promovam melhores resultados funcionais commenor impacto no retorno às atividades da vida diária quando comparado aos pacientes amputados. Entretanto, não há um consenso sobre diferenças nos resultados funcionais entre esses dois grupos de pacientes. Este estudo teve como objetivo analisar os fatoresassociados à sobrevida e identificar o perfil epidemiológico de funcionalidade equalidade de vida de uma coorte hospitalar de referência em oncologia no Rio de Janeiro. Os resultados sugerem que o sexo, idade, tamanho do tumor, localização, grau, tipo histopatológico são os fatores que interferem significativamente na sobrevidaglobal em cinco anos. E que os índices de funcionalidade e qualidade de vidaapresentaram escores satisfatórios tanto para pacientes com cirurgia preservadora de membros quanto para amputados. Conclui-se que além do tempo de diagnóstico da evolução do tumor, fatores inerentes às características próprias do indivíduo ehistopatológicas interferem no prognósticos da doença. Novos estudos na população brasileira poderão contribuir para as questões que ainda permanecem em discussão sobre a influência na sobrevida da qualidade da margem cirúrgica e dos protocolos de radioterapia e quimioterapia, e estudos sobre a funcionalidade e qualidade de vida que mensurem as variáveis em diferentes fases do tratamento com análises multivariadas para esclarecer os principais fatores associados a esses índices.

Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida / Epithelioid sarcoma: clinical behavior, prognostic factors and survival

OBJETIVO: Descrever os aspectos epidemiológicos, clínicos, o tratamento e o prognóstico dos pacientes com sarcoma epitelióide. MÉTODOS: Revisão do prontuário de 25 pacientes matriculados no INCA com o diagnóstico de sarcoma epitelióide, no período de 05 de junho de 1987 à 15 de julho de 2005. RESULTADOS: A idade mediana foi de 33 anos, variando de 10 à 70 anos. A localização primária mais freqüente foi os membros superiores em doze casos (48 por cento). O tamanho do tumor foi descrito em 19 casos e a mediana foi de 5cm, variando de 1,5 à 15cm. A cirurgia foi realizada em dezessete pacientes com onze amputações. As margens cirúrgicas estavam livres em quinze pacientes, comprometidas em três e em sete não foram relatadas. Seis receberam tratamento com algum tipo de quimioterapia e quatorze receberam tratamento com radioterapia com dose mediana de 46,5Gy. Recidiva local ocorreu em treze casos (52 por cento). Recidiva nodal foi diagnosticada em nove pacientes (36 por cento). Metástase pulmonar foi diagnosticada em sete pacientes (28 por cento). Seis pacientes realizaram o tratamento oncológico na sua totalidade no INCA. Atualmente doze estão vivos sem doença, dois estão vivos com doença e onze pacientes foram a óbito. CONCLUSÃO: O sarcoma epitelióide é um subtipo raro de sarcoma de partes moles que apresenta alta taxa de recidiva local, regional e metástase à distância. Incide principalmente nas extremidades de pacientes jovens. O tratamento cirúrgico consiste em ressecção alargada com margens livres.

OBJECTIVE: To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas. METHODS: Careful analysis of 25 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between june 1987 and july 2005. RESULTS: Mean age at diagnosis was 33 years old, ranged from 10 to 70. The primary site of presentation was the upper extremity in twelve patients (48 percent). The size of the tumor was given in 19 cases, with the mean size of 5cm, while they ranged from 1,5 to 15cm. Surgery was made in 17 patients, with eleven amputation. Tumors margins were free in fifteen patients, positive in three and in seven were not studied. Six received any type of quimiotherapy and 14 received treatment with radiotherapy with mean dose of 46,5 Gy. Local recurrence occurred in thirteen cases (52 percent). Nodal spread was diagnosed in nine (36 percent). Pulmonary metastases were diagnosed in seven patients (28 percent). Six patients underwent cancer treatment in its entirety at the National Institute of Cancer. At present twelve patients are alive without disease, two have disease and eleven patients have died. CONCLUSION: Epithelioid sarcoma is a rare subset of soft tissue sarcoma with high rate of local recurrence, regional node and distant metastases. Occurs predominantly in young patients, mainly on the superior member extremities. Surgical treatment of epithelioid sarcoma consists of early wide local resection to negative microscopic margins. These patients require carefully follow-up to evaluate local recurrence, nodal metastases, and pulmonary metastases.

Outcome after Surgical Treatment of Pelvic Sarcomas

BACKGROUND: We present here the oncological and functional outcomes of limb salvage with or without reconstruction for primary sarcomas in the pelvic bone. METHODS: Forty-four patients who underwent pelvic resection for primary sarcomas involving the pelvis were reviewed. The average follow-up period was 39 months (range, 0 to 146 months). Chondrosarcoma (n = 17) and osteosarcoma (n = 10) were the most common diagnoses. Various clinicopathologic factors were analyzed in relation to the oncological outcomes of overall survival and local recurrence. The Musculoskeletal Tumor Society functional scores and complications were compared according to the tumor location, pelvic continuity and the type of resection. RESULTS: The overall 5-year survival rate was 40%. Metastasis at the time of diagnosis, the surgical margin and the histologic grade were the independent prognostic factors for survival. The surgical margin was an independent prognostic factor for local recurrence. An ischiopubic location of the tumor, restoration of pelvic continuity and hip joint reconstruction with total hip replacement arthroplasty were related with higher functional scores. Complications occurred in 50% of the patients and the complications varied according to the tumor location with infection being the most common complication. CONCLUSIONS: Achieving an adequate surgical margin is necessary for improving the oncological outcome of pelvic sarcomas. Restoration of the pelvic continuity and the hip joint improves the functional outcome. However, complications are common and so careful selection of the reconstruction method is needed.

Impacto da cirurgia comportamental no tratamento dos pacientes portadoresde sarcormas primários do retroperitônio

Racional: Tradicionalmente, o tratamento dos sarcomas retroperitoniais é cirúrgico e utilizando-se de uma estratégia transportada dos sarcomas de extremidade, a cirurgia compartimental, que no caso dos sarcomas retroperitoniais representa a ressecção dos órgãos em contato com o tumor mesmo sem estarem evidentemente invadidos. Esta atitude agrega morbidade e seu impacto na sobrevida destes pacientes, especialmente na população portadora de tumores com média superior a 20 cm de diâmetro é desconhecido. Objetivo: Este estudo visa determinar o impacto sobre a sobrevida e taxa de recidiva nos pacientes que realizaram a cirurgia compartimental com ressecção de órgãos em associação no tratamento dos pacientes portadores de sarcomas primários do retroperitônio operados no Instituto Nacional de Câncer. Secundariamente, foram estudados os indicadores de morbimortalidade pós-operatória e a identificação dos fatores prognósticos para sobrevida. Métodos: Foram avaliados por análise estatística retrospectiva 91 pacientes portadorees de sarcoma primário de retroperitônio, operados no INCA, no período de junho de 1992 a janeiro de 2008, divididos em cinco grupos, totalizando noventa e um pacientes: Grupo dos “Ressecados”, com todos os pacientes ressecados, Grupo “Compartimental Total”, contendoo aqules que sofreram cirurgia compartimental, Grupo “Compartimental Negativo”, com aqueles cujos órgãos ressecados não estavam invadidos, Grupo “Compartimental Positivo”, com aqueles cujos órgãos ressecados estavam invadidos e Grupo “Não Compartimental”, com os pacientes que não tiveram ressecção de órgão em associação. Determinando as diferenças e semelhanças entre eles e seu impacto na sobrevida e morbimortalidade do paciente. Seré calciulada as sobrevidas global e livre de doença para 2, 5 e 10 anos, além de uma análise uni e multivariada de Cox. Resultados: As queixas mais comuns foram massa e dor abdominal. A taxa de ressecabilidade foi de 83,5% e de radicalidade de 55,3%. Houve 6 óbitos pós-operatórios (6,6%) e 28 pacientes com complicações pós-operatórias (30,8%). Os leiomiossarcomas e os lipossarcomas foram os mais incidentes (totalizando 62,6%). O grau histológico mais freqüente foi o G3 (41,8%) e o diâmetro tumoral mediano foi de 20,5 cm. Houve 124 órgãos ressecados em associação, com somente 42 realmente invadidos na análise histológica. A sobrevida média dos ressecados estimada em 5 anos foi de 32,4% e a sobrevida livre de doença foi de 36,8%. Na analise univariada o grau de diferenciação histológica ([G1 + G2] X [G3 + GX]), a ressecção radical (R0) ou paliativa (R1+R2), a realização de hemotransfusão intraoperatória e a re-ressecção, mesmo que paliativa, nos casos de recorrência ou persistência de doença, foram significantes para sobrevida. A cirurgia compartimental NÃO teve impacto na sobrevida, mas aumentou a morbidade, tempo cirúrgico e a hemotransfusão. Conclusão: Fatores prognósticos clássicos como radicalidade, grau de diferenciação celular, hemotransfusão e re-ressecção foram validados. A cirurgia Compartimental, com ou sem invasão dos órgãos adjacentes não aumentou a sobrevida, mas a morbidade e não diminuiu a taxa de recidiva. Não encontramos vantagem em ressecar órgãos em associação por princípio devendo realizá-la por necessidade. Somente a ressecção completa de pequenos tumores, de baixo grau, evitando hemotransfusões intraoperatórias desnecessárias e realizando a re-ressecção em caso de recidiva ou persistência de doença se podererá garantir uma melhor sobrevida

Background: Retroperitoneal sarcomas (RPS) are rare tumors and radical surgical approach is still the only curative treatment. We aim to first estimate postoperative morbidity, mortality and identify significant prognostic factors for survival of patients with RPS and then secondly, to evaluate the influence of en bloc resection on survival. Methods: This was a retrospective follow-up study of 91 patients with RPS operated in the Section of Abdominopelvic Surgery of the National Cancer Institute-Brazil (INCA) between June 1992 and January 2008. Overall and 2-, 5- and 10-year disease-free survival rates were calculated and univariate and Cox multivariate analysis were performed. Results: The most common complaints were abdominal pain and mass. Resection was possible in 83.5% and curative resection in 55.3%. Six patients died within the postoperative period (mortality = 6.6%) and 28 had complications (30.8%). Leiomyosarcomas and liposarcomas predominated, as well as G3. The median tumor diameter was 20.5 cm. There were 124 organs resected in association, with only 42 proven invaded. The 5-year overall survival and disease free survival rates were 32.0% and 36.8%, respectively. Cell differentiation, curative or palliative resection, blood transfusion and re-resection were significant variables. Compartment surgery had no impact on survival, but increased the duration of surgery, the need for blood transfusion and overall morbidity. Conclusions: This study suggests that early diagnosis and curative resection can improve long-term survival. Adjacent organs with evidence of direct invasion must be removed en bloc; others should be spared

Neoadjuvant chemoradiation therapy for soft tissue sarcomas of the extremities

INTRODUCTION AND OBJECTIVE: Neoadjuvant and adjuvant therapies for soft tissue sarcomas of the extremities are still controversial. The aim of this study was to analyze the results of a protocol of neoadjuvant chemoradiation therapy for extremity sarcomas. METHODS: A retrospective analysis was carried out in a consecutive series of 49 adult patients with advanced extremity soft tissue sarcomas that could not be resected with adequate margins during the primary resection. All patients were treated with a protocol of preoperative radiation therapy at a total dose of 30 Gy, concomitant with doxorubicin (60 mg/m²) chemotherapy. The main endpoints assessed were local recurrence-free survival, metastasis-free survival and overall survival. The median follow-up time was 32.1 months. RESULTS: The five-year local recurrence-free survival, metastasis-free survival and overall survival rates were 81.5 percent, 46.7 percent and 58.3 percent, respectively. For high-grade tumors, the five-year metastasis-free and overall survival rates were only 36.3 percent and 41.2 percent, respectively. Severe wound complications were observed in 41.8 percent of the patients who underwent surgery. These complications precluded adjuvant chemotherapy in 73.7 percent (14/19) of the patients eligible to receive it. CONCLUSIONS: In this study, neoadjuvant chemoradiation therapy was associated with a good local control rate, but the distant relapse-free rate and overall survival rate were still poor. The high rate of wound complications modified the planning of adjuvant treatment in most patients.

Synovial sarcoma of the extremities: prognostic factors for 20 nonmetastatic cases and a new histologic grading system with prognostic significance

PURPOSE: To evaluate 20 cases of nonmetastatic synovial sarcoma of the extremities regarding prognostic factors, and to propose a histologic grading system with prognostic significance. METHODS: The cases of 20 patients (14 females and 6 males) with nonmetastatic synovial sarcomas of the extremities treated between 1985 and 1998, were retrospectively evaluated regarding prognostic factors. A histologic grading system with prognostic significance is proposed. RESULTS: The mean follow-up period was 48.4 months (range, 16-116 months). There was local recurrence in 3 cases (15 percent), microscopic surgical margin being the only prognostic factor identified. Seven patients (35 percent) died of the disease in a mean postoperative period of 31.7 months (range, 16-53 months), all with pulmonary or brain metastasis. The survival rate was 65 percent in 48.4 months of follow-up. CONCLUSION: The unfavorable prognostic factors identified regarding survival were high histologic grade, tumors proximal to the knee or elbow, and spontaneous tumor necrosis over 25 percent. Local recurrence did not have influence on survival in this study. The presence of mast cells appears to have a positive influence on survival, although statistical significance was not reached (P = 0.07). The oncologic and functional result was good in 6 cases (30 percent), regular in 7 (35 percent), and poor in 7 cases (35 percent).

OBJETIVO: Avaliar casos de sarcoma sinovial não-metastático das extremidades no que se refere a fatores prognósticos, e propor um sistema histológico de pontuação com significado prognóstico. MATERIAL E MÉTODO: Vinte casos (14 do sexo feminino e 6 do sexo masculino) de sarcomas sinoviais não-metastáticos das extremidades tratados entre 1985 e 1998 no departamento de Ortopedia foram avaliados retrospectivamente no que se refere a fatores prognósticos e está sendo proposto um sistema de pontuação histológico com significado prognóstico. RESULTADOS: A média dos períodos de acompanhamento foi 48,4 meses (mínimo 16 meses, máximo 116). Houve recorrência localizada em 3 casos (15 por cento), sendo a margem cirúrgica microscópica o único fator prognóstico identificado. Sete pacientes (35 por cento) morreram da doença, todos em período pós-operatório médio de 31,7 meses (mínimo 16 meses, máximo 53), todos com metástase pulmonar ou cerebral. A sobrevida foi de 65 por cento em 48,4 meses de acompanhamento. CONCLUSÃO: Os fatores prognósticos desfavoráveis identificados referentes à sobrevida foram: grau histológico alto, tumores proximais de joelho ou cotovelo e necrose espontânea de tumor acima de 25 por cento. Neste estudo, a recorrência localizada não influiu na sobrevida. Parece que a presença de mastócitos influi positivamente na sobrevida, porém não obtivemos significado estatístico (p=0,07). O resultado oncológico e funcional foi bom em seis casos (30 por cento), regular em sete (35 por cento) e insatisfatório em sete (35 por cento).

The outcomes and prognostic factors of patients with soft-tissue sarcoma.

The present study evaluates the outcomes and prognostic factors in patients with soft-tissue sarcomas of the extremities, trunk, head and neck region. A retrospective study of 104 patients who underwent treatment was conducted on 48 males and 56 females with a mean age of 44.5 years (range, 10-85 years). Seventy-eight patients had high-grade sarcomas and most tumors (89.5 percent) were located at the extremities. One hundred patients were treated by surgery and 51 patients were treated by both surgery and radiation therapy. With the median follow-up time of 24.5 months, local recurrence developed in 26 patients (25 percent) and distant metastasis developed in 29 patients (27.9 percent). The actuarial overall 3-year disease-specific survival rate were 74.2 percent. Multivariate statistical analysis revealed that positive surgical margin and occurrence of distant metastasis were significant predictors for overall survival. Positive surgical margin was the only factor that increased the risk of local recurrence and older age (> or = 60 years) was only the factor that increased the risk of distant metastasis. The results reaffirm the importance of the surgical margin where uncontrolled local disease affects the risk of local failure and disease-specific survival. Occurrence of distant metastasis is associated with older age (> or = 60 years) and decreases disease-specific survival of the patients.

1470 cases of sarcoma referring to Imam Khomeini hospital during an 11-year period

Sarcoma is a malignant neoplasm arising in mesenchymal tissue. It comprises 1% of adult malignancies and 15% of childhood neoplasms. The present study was designed to review the clinical characteristics of 1470 sarcoma cases and related predictors of out come, relapse and survival. For this retrospective study, 1470 medical files of patients with sarcoma who were referred during 1991-2002 to Imam Khomeini Hospital were studied. Sarcoma comprised 11% of all cases referring to our hospital. The mean age of patients with sarcoma was 30 years and male to female ratio was 3/2. Bone to soft tissue sarcoma ratio was 3.1 in children and 1.3 in adults. Osteosarcoma, Ewing and rhabdomyosarcoma presented 83% of children tumors, however, in adults osteosarcoma, synovial sarcoma and malignant fibrous histocytoma [MFH] were the most common subtypes. The main prognostic factors for survival were tumor size, margin of surgery, neurovascular involvement in pathological report, initial metastasis and complete response to initial therapy. Adjuvant radiotherapy, small tumor size, curative surgery with chemotherapy and free surgical margins were significantly associated with reduced recurrence. Complete response to primary therapy is the main independent variable of overall survival of patients. Earlier diagnosis and experienced team including surgical, medical and radiotherapy oncologist is needed for better response and longer survival rate

Estudo da morbimortalidade cirúrgica, da sobrevida e dos fatores prognósticos de sarcomas primários do retroperitônio

Tendo como objetivo avaliar a morbimortalidade, sobrevida e os fatores prognósticos dos sarcomas primários do retroperitônio, analisamos retrospectivamente 59 prontuários de pacientes operados na Seção de Cirurgia Abdomino-Pélvica do Instituto Nacional de Câncer no período de junho de 1992 a julho de 2003. Vinte e três pacientes eram homens e 36 mulheres; 38 brancos, 6 negros e 15 pardos, com idade mediana de 50 anos (6-72). História familiar de câncer esteve presente em 21 casos (35,59%). As queixas mais comuns foram dor abdominal (31 pac.) e massa abdominal (17 pac.). TC foi o principal exame realizado para o diagnóstico (53 pac.). A taxa de ressecabilidade foi de 74,57% (44 pac.) e a de radicalidade entre os ressecados de 48,88% (22 pac.). Houve 2 óbitos pós-operatórios (3,38%) e 12 complicações pós-operatórias (20,33%). Os leiomiossarcomas (16 pac.) e os lipossarcomas (15 pac.) foram os mais incidentes. O grau de diferenciação tumoral mais freqüente foi o G3 (23 pac. [38,98%]) e o diâmetro tumoral médio, de 20,4 cm. A re-ressecção no total de ressecados (45 pac.) aconteceu em 21 pacientes (46,66%), sendo única em 16, dupla em 3 e tripla em 2 pacientes. Em 10 dos 21 pacientes, a re-ressecção foi radical. A sobrevida global foi de 49% em 2 anos e 20% em 5 anos, e a mediana de sobrevida livre de doença foi de 23 meses. A análise univariada, o diâmetro do tumor (> ou <= 12 cm), o grau de diferenciação tumoral ([Gl + G2] X [G3 + G4]), a ressecção radical (R0) ou paliativa (R1+R2) a hemotranfusão no ato operatório e a re-ressecção, mesmo que paliativa, nos casos de recidiva ou persistência de doença (n = 52), foram significativos para sobrevida (p = 0,0267, 0,048, 0,0001, 0,022 e 0,0003, respectivamente). Na análise multivariada, não houve fator independente. Essas análises sugerem que, no momento, somente o diagnóstico precoce, a cirurgia radical R0, a ausência de hemotransfusão intra-operatória e a re-ressecção nos casos de recidiva ou persistência de doença possibilitarão a sobrevida a longo prazo

To evaluate the morbjmortality, survival and the prognostics factors of the retroperitonial sarcomas, we analyzed retrospectively 59 records of patients operated by the Abdomino-pelvic Department of Surgery at the National Cancer Institute of Brazil in the period between June 1992 and July 2003. Twenty three were men and 36 women, 38 white, 6 black and 15 mixed. Mean age was 50 years old (6-72). Family history of cancer was present in 21 cases (35.59%). The most common complaints were abdominal pain (31 pts) and abdominal mass (17 pts). TC was the main exam for diagnosis (53 pats). Resectability rate was 76.27% (45 pts), andradicality 48.88% (22pts). There were 2 postoperative deaths (3.38%). There were 8 technical complications (13.55%), besides 4 clinical complications (6.77%). Liposarcomas (16 pts) and the leiomyosarcomas (15 pts) were the most common types. The most frequent tumor grade was G3 (3 pts [38.98%J) and the tumor median diameter was 20.4 cm. Re-resection rate was (46.66%), being single in 16, double in 3 and triple in 2 patient. The overall survival rate was 49% for 2 years and 20% for 5 years and the median disease free survival was 23 months. In the univariate analysis, the diameter of the tumor (> or <= 12 em), grade of tumor differentiation ([G1 + G2] X [G3 + G4J), radical R0) or palliative resection(R1+R2), operative blood transfusion, and re-resection, even if palliative, in the cases of recurrence or persistence of disease (n=52) were significant (p = 0.0267, 0.048, 0.0001, 0.022 and 0.0003, respectively). Multivariate analysis did not show any independent factor. These analyses suggest that, in the moment, only early diagnosis, radical surgery, absence of intra-operative blood transfusion and the re-resection in cases of recurrence or persistence of disease will provide greater chances of a long term survival

Sarcomas de partes moles primários de tronco e extremidades em lactentes / Primary trunk and extremities soft sarcomas in infants

Durante o período de janeiro de 1970 a dezembro de 1991, 30 lactentes portadores de sarcoma de partes moles primário do tronco e extremidades foram admitidos no Departamento de Pediatria do Hospital do Câncer. Treze neoplasias eram originárias da parede torácica e abdominal; 13, de membros inferiores; e quatro, de membros superiores. Dez pacientes apresentavam doença localizada e 20, doença avançada. Em relaçäo ao tipo histológico, 18 tumores foram classificados como sarcomas de partes moles näo rabdomiossarcoma. Quinze crianças estäo vivas sem evidência de doença; uma, viva com segundo tumor (LLA); três foram perdidos de seguimento e 11 foram a óbito (10 por progressäo de doença e um por toxicidade). A intervençäo cirúrgica, a quimioterapia e a radioterapia utilizadas isoladamente ou combinadas na dependência do tipo histológico e extensäo da doença nos pacientes portadores de sarcomas de partes moles foram responsáveis pelo aumento da taxa de cura nesses casos. As características intrínsecas do lactente fazem com que cada vez mais esses recursos terapêuticos venham a ser aprimorados a fim de que os efeitos colaterais decorrentes do tratamento sejam evitados ou minimizados. Ao analisarmos lactentes portadores de sarcomas de partes moles, pudemos avaliar os efeitos deletérios decorrentes da terapêutica preconizada nos anos 70 e 80, atentando para o fato de que cada vez mais, cura e qualidade de vida devem caminhar paralelamente. Os avanços em biologia molecular poderÝo, num futuro próximo, selecionar pacientes de maior ou menor risco, preconizando tratamentos mais e menos agressivos na dependência do prognóstico. O fator prognóstico mais importante foi a extensäo da doença ao diagnóstico.

Sarcomas de la pared torácica. Resección y reconstrucción / Chest wall neoplasm. Resection and reconstruction

Los sarcomas de la pared torácica corresponden al 5 por ciento de este tipo de neoplasias. La resección y reconstrucción de la pared torácica se ha desarrollado mucho en los últimos años debido a la evolución de las diferentes técnicas de reconstrucción, principalmente de los colgajos miocutáneos. En este artículo presentamos la experiencia obtenida durante 1984 a 1994 en el Instituto Nacional de Cancerología. Se llevaron a cabo 59 resecciones de la pared torácica; la indicación más frecuente la representaron los sarconas óseos en 16 pacientes (27.2 por ciento) y los sarcomas de partes blandas en 15 (25.4 por ciento). La indicación quirúrgica tuvo una finalidad paliativa en 26 casos 844.1 por ciento) y curativa en 33 (54.9 por ciento). A todos los pacientes se les realizó resección ósea. El promedio de costillas resecadas fue de 3.8. Después de la resección, el defecto esquelético y la estabilidad de la pared torácica se reconstruyó en 36 sujetos 861 por ciento); de éstos, se realizó con fascia lata en 17 (47.4 por ciento) y con malla sintética en 18 (50 por ciento). La reconstrucción de partes blandas fue necesaria en 21 casos y se llevó a cabo con colgajos miocutáneos; el más utilizado fue el músculo dorsal ancho en 11 pacientes. El tiempo quirúrgico promedio fue de cuatro horas 15 minutos y el de hospitalización de 8.4 días. La morbilidad fue del 20.3 por ciento y la mortalidad operatoria del 5.0 por ciento