Sarcoma de Ewing óseo: Enfoque multidisciplinario y resultados oncológicos en 88 pacientes / Ewing sarcoma of the bone. Multidisciplinary approach and oncological results in 88 patients

El sarcoma de Ewing óseo es un tumor poco frecuente, agresivo, que afecta principalmente a niños y adultos jóvenes. Existe ausencia de registros en nuestro país respecto de la prevalencia de esta enfermedad, los esquemas de tratamiento utilizados y sus resultados. El objetivo fue analizar, en un grupo de pacientes con sarcoma de Ewing óseo tratados con quimioterapia y cirugía de conservación de miembro, las tasas de supervivencia global, de recurrencia local y los factores de riesgo oncológicos. Se incluyó a 88 pacientes. La edad media de la serie fue de 14.5 años y el seguimiento promedio de 8.8 años. La tasa de supervivencia global fue de 79.5% a los 2 años, de 69% a 5 años y de 64% a 10 años. Los factores pronósticos negativos asociados a menor supervivencia fueron: mala respuesta a la quimioterapia, edad ≥ de 16 años, localización central, y recurrencia local. En el análisis multivariable únicamente la respuesta a la quimioterapia tuvo significancia estadística. La tasa libre de recurrencia local a 2 y 5 años fue del 87%. La mala respuesta a la quimioterapia fue el único factor significativo para la recurrencia local. Consideramos que la cirugía de conservación de miembro asociada a quimioterapia pre y postoperatoria debe ser el tratamiento para el sarcoma de Ewing óseo, alcanzando de esta manera una supervivencia global a 5 años del 69%. En nuestra serie, la respuesta a la quimioterapia ha sido el factor pronóstico más relevante para supervivencia y recurrencia local.

Ewing sarcoma of the bone is a rare, highly aggressive tumor that typically affects children and young adults. In Argentina, the lack of Ewing's sarcoma registries reflects in the absence of information regarding prevalence, treatment protocols and patient´s outcome. The purpose of this study was to analyze, in a group of patients diagnosed with Ewing sarcoma of the bone, treated with chemotherapy and limb-conserving surgery, their overall survival rate, local recurrence rate, and oncological risk factors. A retrospective research was conducted between 1990 and 2017. Eighty-eight patients with Ewing sarcoma of the bone matched the inclusion criteria. Median age was 14.5 years and median follow-up was 8.8 years. Overall survival rate was 79.5%, 69% and 64% at 2, 5 and 10 years respectively. Negative prognostic factors, associated with less survival rate after univariate analysis, were: bad response to chemotherapy (tumoral necrosis 0-89%), age > 16 years-old, central tumor localization and local recurrence. Gender and tumor size were not significant prognostic factors. After multivariate analysis, response to chemotherapy remained statistical significant. Local recurrence-free survival rate at 2 and 5 years was 87%. Tumor response to chemotherapy (0-89%) was the only significant factor for local recurrence. We consider that limb-salvage surgery, with neoadjuvant and adjuvant chemotherapy, are the mainstays of treatment for Ewing's sarcoma, with an overall survival rate, at 5 years, of 69%. In this population, response to chemotherapy is the most relevant prognostic factor, being associated with both local recurrence and overall survival.

Osteosarcoma of the jaw - experience at the Medical University Vienna and comparative study with international tumor registries

OBJECTIVES: Osteosarcoma of the jaw (OSAJ) is fundamentally different in clinical practice from its peripheral counterparts. Studies are difficult to conduct due to low incidence rates. The primary aim of this study was to provide for the first time a comprehensive retrospective analysis of the treatment concepts and outcome data of OSAJ patients treated at the University Hospital Vienna and to compare these with two recently published studies on OSAJ. The clinical study was accompanied by a biomarker study investigating the prognostic relevance of melanoma-associated antigen-A (MAGE-A) in OSAJ specimens. METHOD: Eighteen patients were included, and their outcomes were compared to published data. Immunohistochemistry was performed with mouse monoclonal antibodies against MAGE-A. Survival rates were estimated by the Kaplan-Meyer method. The log-rank test was used to analyze potential prognostic parameters. Fisher's exact test was performed to define the significant differences between the survival rates of the current study and the DOESAK registry. RESULTS: Disease-specific survival was 93.8% after five and 56.3% after ten years. The development of metastases (p=0.033) or relapse (p=0.037) was associated with worsened outcomes in our group as well as in the comparative group. Despite the different treatment concepts of the study groups, survival rates were comparable. MAGE-A failed to show prognostic relevance for OSAJ patients. CONCLUSIONS: Uncertainties about the optimal treatment strategies of OSAJ patients will currently remain. Thus, prospective studies of OSAJ are needed but are only feasible in a multicenter study setting, conducted over a prolonged time period.

Incidence and mortality of bone cancer among children, adolescents and young adults of Brazil

OBJECTIVES: Bone cancers occur frequently in children, adolescents, and young adults aging 15 to 29 years. Osteosarcoma and Ewing sarcoma are the most frequent subtypes in this population. The aim of this study was to describe incidence and mortality trends of bone cancers among Brazilian children, adolescents and young adults. METHODS: Incidence information was obtained from 23 population-based cancer registries. Mortality data were extracted from the Atlas of Cancer Mortality from 1979 to 2013. Specific and adjusted rates per million were analyzed according to gender, morphology and age at diagnosis. Median rates were used as a measure of central tendency. Joinpoint regression was applied to analyze trends. RESULTS: Median incidence rates were 5.74 and 11.25 cases per million in children and young adults respectively. Osteosarcoma in the 15-19 years aged group had the highest incidence rates. Stable incidence rates were observed among five registries in 0-14 year's age group. Four registries had a decreased incidence trend among adolescents and young adults. Median mortality rates were 1.22 and 5.07 deaths per million in children and young adults respectively. Increased mortality was observed on the North and Northeast regions. Decreased mortality trends were seen in the South (children) and Southeast (adolescents and young adults). CONCLUSION: Osteosarcoma and Ewing Sarcoma are the most incident bone cancers in all Brazilian regions. Bone cancers showed incidence and mortality patterns variation within the geographic regions and across age groups, although not significant. Despite limitations, it is crucial to monitor cancer epidemiology trends across geographic Brazilian regions.

Expressão de neurotrofinas e de seus receptores no osteossarcoma primário / Expression of neurotrophins and their receptors in primary osteosarcoma

RESUMO Objetivo: determinar a expressão de neurotrofinas e seus receptores tirosina quinases em pacientes com osteossarcoma (OS) e sua correlação com desfechos clínicos. Métodos: biópsias de tumores primários de pacientes com OS tratados em uma única instituição, consecutivamente, entre 2002 e 2015, foram analisados através de imuno-histoquímica para expressão de receptores de tirosina quinase A e B (TrKA e TrKB), fator de crescimento neural (NGF) e fator neurotrófico derivado do cérebro (BDNF). De forma independente, dois patologistas classificaram os marcadores de imuno-histoquímica como negativos (negativos e focais fracos) ou positivos (moderado focal/difuso ou forte focal/difuso). Resultados: foram analisados dados de 19 pacientes (10 do sexo feminino e 9 do masculino) com mediana de idade de 12 anos (5 a 17,3 anos). Dos tumores, 83,3% estavam localizados em membros inferiores e 63,2% dos pacientes eram metastáticos ao diagnóstico. A sobrevida global em cinco anos foi de 55,3%. BDNF foi positivo em 16 pacientes (84%) e NGF em 14 pacientes (73%). TrKA e TrKB apresentaram coloração positiva em quatro (21,1%) e oito (42,1%) pacientes, respectivamente. A análise de sobrevida não demonstrou diferença significativa entre receptores TrK e neurotrofinas. Conclusão: amostras de OS primário expressam neurotrofinas e receptores TrK através de imuno-histoquímica. Estudos futuros podem auxiliar na identificação do papel das mesmas na patogênese do OS e determinar se há possível correlação prognóstica.

ABSTRACT Objective: to determine the expression of neurotrophins and their tyrosine-kinase receptors in patients with osteosarcoma (OS) and their correlation with clinical outcomes. Methods: we applied immunohistochemistry to biopsy specimens of patients consecutively treated for primary OS at a single institution between 2002 and 2015, analyzing them for expression receptors of tyrosine kinase A and B (TrKA and TrKB), neural growth factor (NGF) and brain derived neurotrophic factor (BDNF). Independently, two pathologists classified the immunohistochemical markers as negative (negative or weak focal) or positive (moderate focal/diffuse or strong focal/diffuse). Results: we analyzed data from 19 patients (10 females and 9 males), with median age of 12 years (5 to 17.3). Tumors' location were 83.3% in the lower limbs, and 63.2% of patients had metastases at diagnosis. Five-year overall survival was 55.3%. BDNF was positive in 16 patients (84%) and NGF in 14 (73%). TrKA and TrKB presented positive staining in four (21,1%) and eight (42,1%) patients, respectively. Survival analysis showed no significant difference between TrK receptors and neurotrophins. Conclusion: primary OS samples express neurotrophins and TrK receptors by immunohistochemistry. Future studies should explore their role in OS pathogenesis and determine their prognostic significance in larger cohorts.

Survival outcome among patients with Ewing's sarcoma of bones and joints: a population-based cohort study

ABSTRACT BACKGROUND: The aim here was to elucidate the current survival condition of patients diagnosed with Ewing's sarcoma of the bones and joints and determine independent risk factors associated with the prognosis. DESIGN AND SETTING: Retrospective cohort study based on the Surveillance, Epidemiology and End Results (SEER) database in the United States. METHODS: We identified 397 patients who were diagnosed with Ewing's sarcoma of the bones and joints between January 2004 and December 2013. The multivariate Cox proportional hazards model was used to determine factors associated with the risk of death by adjusting for various factors. RESULTS: The one, two and five-year disease-specific survival rates were 89.08%, 78.08% and 62.47%, respectively. The factors related to death were age (≥ 18 years versus < 18 years; hazard ratio, HR = 1.77; 95% confidence interval, CI: 1.38-2.31); tumor site (extremity versus spine and pelvis; HR = 2.03; 95% CI: 1.31-2.62); tumor size (> 10 cm versus ≤ 10 cm; HR = 1.78; 95% CI: 1.34-2.56); and type of treatment (surgery alone versus radiotherapy with surgery; HR = 0.51; 95% CI: 0.38-0.89; or radiotherapy alone versus radiotherapy with surgery; HR = 1.61; 95% CI: 1.10-2.39; or no treatment versus radiotherapy with surgery; HR = 1.86; 95% CI: 1.23, 2.58). CONCLUSIONS: Patients with Ewing's sarcoma showed poor survival in situations of age ≥ 18 years, tumor size > 10 cm, receiving radiotherapy alone and receiving no treatment. Patients undergoing surgery alone had better survival.

Outcomes of patients with bone metastases from differentiated thyroid cancer

ABSTRACT Objective Bone metastases (BM) from differentiated thyroid cancer (DTC) are associated with poor survival rates. Due to the low frequency of this entity, we performed a multicentric retrospective study that aimed to evaluate the presentation, outcome and causes of death in this population. Subjects and methods We reviewed file records from 10 databases. BM were diagnosed by: i) biopsy and/or ii) radioiodine (RAI) bone uptake + elevated thyroglobulin (Tg) levels and/or c) bone uptake of 18-FDG in the PET-CT scan + elevated Tg levels. Results Fifty-two patients with DTC were included (44% male, mean age 54 years); 58% had papillary histology. BM were synchronous with DTC diagnosis in 46% of the participating cases. BM were symptomatic in 65% of the cases. Multiple BM were present in 65% of patients, while simultaneous metastatic disease in additional sites was found in 69%. Ninety-eight percent of patients received treatment for the BM, which included RAI therapy in 42 patients; 30 of them received cumulative RAI doses that were larger than 600 mCi 131I. The mean follow-up after a BM diagnosis was 34 months. The 2- and 5-year survival rates after diagnosis of the first BM were 64% and 38%, respectively. The status on the last evaluation was DTC-related death in 52% of the patients; 26% of them died from direct complications of BM or their treatments. Conclusion BM are usually radioiodine-refractory and are associated with a short overall survival, although most of the patients died of causes not directly related to the BM.

Factores pronósticos de sobrevida de pacientes con osteosarcoma / Prognostic factors for survival of patients with osteosarcoma

Identificar los factores pronósticos de sobrevida de pacientes con osteosarcoma a través de un modelo paramétrico de sobrevida. Material y métodos: Se realizó un estudio de cohorte retrospectivo, observacional, el cual incluyó 68 pacientes con diagnóstico de osteosarcoma atendidos en el Instituto traumatológico entre el 1 de enero del 2002 al 31 de diciembre del 2012. Para el cálculo del promedio de incidencia de osteosarcoma se consideró como denominador, la población mayor e igual 15 años, en el tiempo de estudio.Los criterios de inclusión fueron: pacientes de ambos sexos, edad igual o mayor a 15 años y biopsia positiva para cualquier tipo de osteosarcoma. Se consideraron las siguientes variables predictoras: sexo, localidad del tumor, metástasis pulmonar, recidiva, tipo de osteosarcoma, grado histológico, tratamiento y porcentaje de necrosis tumoral. Resultados: La tasa de incidencia promedio de osteosarcoma en la población de 15 años o más fue de 0,98 casos por un millón de habitantes por año. El modelo multivariado contempló como mal pronóstico: sexo masculino, metástasis pulmonar durante el primer año, recidiva y necrosis tumoral menor al 90 por ciento. Discusión: Las variables predictoras de sobrevida de pacientes con osteosarcoma que coinciden con otros modelos multivariados son: sexo masculino, metástasis pulmonar y porcentaje de necrosis tumoral menor al 90%. Por otra parte, la discrepancia con otros autores se puede explicar por la gran variación en la metodología utilizada, como también por la variabilidad de factores pronósticos considerados, más que por diferencias tangibles en el manejo de estos pacientes en los diferentes centros asistenciales...

To identify prognostic factors of survival in patients with osteosarcoma, using a parametric survival model. Material and methods: A retrospective cohort study was carried out on 68 patients diagnosed with osteosarcoma and who were treated at Traumatology Institute from January 1 2002 to December 31 2012. In order to calculate the mean incidence of osteosarcoma, the population greater than or equal to 15 years old during the study period was considered as a denominator. The following were inclusion criteria: patients of either sex, age greater than or equal to 15 years, and positive biopsy for any kind of osteosarcoma. The following predictor variables were considered: sex, tumour site, lung metastasis, relapse, kind of osteosarcoma, histological grade, treatment, and percentage of tumour necrosis. Results: The mean incidence rate of osteosarcoma in the population studied was 0.98 cases per 1 million people per year. As a poor prognosis, the multivariate model showed: masculine gender, lung metastasis during the first year, relapse, and less than 90 percent of tumour necrosis. Discussion: The predictor variables of survival in patients with osteosarcoma that agree with other multivariate models are: masculine gender, lung metastasis, and less than 90% of tumour necrosis. On the other hand, the discrepancy with other authors can be explained by the wide variation in the methodology used, as well as the variability of the prognostic factors considered, rather than tangible differences in patient management in the various medical centres...

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.

Prognostic Evaluation of Nasopharyngeal Carcinoma with Bone-Only Metastasis after Therapy

PURPOSE: To evaluate the prognosis of nasopharyngeal carcinoma (NPC) patients who developed bone-only metastasis after primary treatment and the stratification of these patients into different risk groups based on independent prognostic factors. MATERIALS AND METHODS: Eighty NPC patients who developed bone-only metastasis after definitive radiotherapy from October 2005 to December 2010 were enrolled. All these patients received palliative treatment for bone metastasis, including chemotherapy and/or radiotherapy. Clinical features, treatment modality, and laboratory parameters were examined with univariate and multivariate analyses. RESULTS: The median follow-up time was 15.5 months (range, 2-67 months) for the whole cohort. The median overall metastatic survival (OMS) time and the 2-year estimate OMS rate were 26.5 months and 52%, respectively. Multivariate analysis indicated that patients with short metastases-free interval, multiple bone metastases sites, high serum lactic dehydrogenase levels, and treated with radiotherapy or chemotherapy alone had significantly worse outcomes. Patients were stratified into three different risk groups based on the number of adverse factors present. The OMS curves of the three groups were all significantly different (p<0.001). CONCLUSION: Severl prognostic factors were found to be associated with worse outcomes. According to the number of adverse factors present, bone-only metastasis patients can be stratified into three risk groups with significantly different prognoses. Such grouping may help in improving the design of clinical trials and in guiding individualized treatment for NPC patients with bone-only metastasis.

A prediction model of survival for patients with bone metastasis from uterine cervical cancer / 부인종양

OBJECTIVE: The aim of the study was to establish a predictive model of survival period after bone metastasis from cervical cancer. METHODS: A total of 54 patients with bone metastasis from cervical cancer were included in the study. Data at the time of bone metastasis diagnosis, which included presence of extraskeletal metastasis, performance status, history of any previous radiation or chemotherapy, the number of bone metastases, onset period, and treatment were collected. Survival data were analyzed using Kaplan-Meier method and Cox proportional hazards model. RESULTS: The median survival period after diagnosis of bone metastasis was 22 weeks (5 months). The 26- and 52-week survival rates after bone metastasis were 36.5% and 15.4%, respectively. Cox regression analysis showed that extraskeletal metastasis (hazard ratio [HR], 6.1; 95% CI, 2.2 to 16.6), performance status of 3 to 4 (HR, 7.8; 95% CI, 3.3 to 18.2), previous radiation or chemotherapy (HR, 3.3; 95% CI, 1.4 to 7.8), multiple bone metastases (HR, 1.9; 95% CI, 1.0 to 3.5), and a bone metastasis-free interval of <12 months (HR, 2.5; 95% CI, 1.2 to 5.3) were significantly and independently related to poor survival. A prognostic score was calculated by adding the number of each significant factor. The 26-week survival rates after diagnosis of bone metastasis were 70.1% in the group with a score ≤2, 46.7% in the group with a score of 3, and 12.5% in the group with a score ≥4 (p<0.001). CONCLUSION: This scoring system provided useful prognostic information on survival of patients with bone metastasis of cervical cancer.

The negative prognostic impact of bone metastasis with a tumor mass

OBJECTIVE: Typically, bone metastasis causes osteolytic and osteoblastic lesions resulting from the interactions of tumor cells with osteoclasts and osteoblasts. In addition to these interactions, tumor tissues may grow inside bones and cause mass lesions. In the present study, we aimed to demonstrate the negative impact of a tumor mass in a large cohort of patients with bone metastatic cancer. METHODS: Data from 335 patients with bone metastases were retrospectively reviewed. For the analysis, all patients were divided into three subgroups with respect to the type of bone metastasis: osteolytic, osteoblastic, or mixed. The patients were subsequently categorized as having bone metastasis with or without a tumor mass, and statistically significant differences in median survival and 2-year overall survival were observed between these patients (the median survival and 2-year overall survival were respectively 3 months and 16% in patients with a tumor mass and 11 months and 26% in patients without a tumor mass; p<0.001). RESULTS: According to multivariate analysis, the presence of bone metastasis with a tumor mass was found to be an independent prognostic factor (p=0.011, hazard ratio: 1.62, 95% confidence interval: 1.11–1.76). Bone metastasis with a tumor mass was more strongly associated with osteolytic lesions, other primary diseases (except for primary breast and prostate cancers), and spinal cord compression. CONCLUSION: Bone metastasis with a tumor mass is a strong and independent negative prognostic factor for survival in cancer patients. .

Serum alkaline phosphatase predicts survival outcomes in patients with skeletal metastatic nasopharyngeal carcinoma

OBJECTIVE: Bone metastasis is frequently associated with nasopharyngeal carcinoma. The diagnosis and follow-up of bone metastatic patients usually relies on skeletal X-ray and bone scintigraphy, which are time-consuming and costly. This study aimed to evaluate whether serum alkaline phosphatase offers clinical value in predicting the clinical response and survival outcome for skeletal metastatic nasopharyngeal carcinoma. METHODS: Serum alkaline phosphatase was measured at baseline and then before each cycle of treatment in 416 nasopharyngeal carcinoma patients with bone metastasis. The correlations between the pre-treatment and post-treatment alkaline phosphatase levels and the treatment efficacy were analyzed using the chi-square test. Survival was analyzed using the Kaplan–Meier method and then compared using the log-rank test. RESULTS: Patients with elevated pre-treatment alkaline phosphatase (>110 IU/L) had significantly worse progression-free survival (P<0.001) and overall survival (P<0.001) than those with a normal level of this marker (≤110 IU/L). Patients with elevated post-treatment alkaline phosphatase had worse progression-free survival (P<0.001) and overall survival (P<0.001) compared with those with a normal level. Patients with normal pre-treatment and post-treatment alkaline phosphatase showed the most favorable prognosis. The Cox multivariate analysis revealed that only the pre-treatment and post-treatment alkaline phosphatase levels were independent prognostic factors for progression-free survival (HR ϝ 1.656, P<0.001; HR ϝ 2.226, P<0.001) and for overall survival (HR ϝ 1.794, P<0.001; HR ϝ 2.657, P<0.001). CONCLUSIONS: Serum alkaline phosphatase appears to be a significant independent prognostic index in patients with skeletal metastatic nasopharyngeal carcinoma, which could reflect the short-term treatment response ...

Non-Spine Bone Metastasis as an Initial Manifestation of Cancer in Korea

Non-spine bone metastasis accounts for approximately 20% of all skeletal metastases, but little data have been published that focused on bone metastasis to the pelvis and extremities as an initial manifestation of cancer. We determined 1) clinicopathologic characteristics of patients who presented with non-spine bone metastasis of unknown primary malignancy, and 2) process by which the diagnosis of primary cancer was made. We retrospectively reviewed 84 patients with bone metastasis of unknown primary cancer site at the time of presentation. The study population consisted of 56 men and 28 women, with a mean age of 59.1 yr (17.5-85.6 yr). The average follow-up period was 20.8 months (1-120 mo). Primary cancer site was identified in 79 patients (94.0%), and was determined to be the lung (46.4%), kidney (13.1%), liver (9.5%), thyroid (8.3%), and prostate (4.8%). Five-year overall survival rate was 28.0%. Multiple bone metastases, distant organ metastasis, and multiple bone with organ metastases were the significant prognostic factors in univariate analysis. Multiple bone metastases remained significant after multivariate analysis (P = 0.008). Lung cancer is the most common site of primary cancer, and patients with multiple bone metastases have a poor prognosis, possibly due to disseminated cancer and a greater tumor burden.

Sobrevida de pacientes con cáncer de mama y metástasis ósea / Survival of patients with breast cancer and bone metastasis

Introduction: Breast cancer is an important cause of death among female cancer in Chile. It metastasizes to any part of the body, being bone the first area of dissemination in 26-50 percent of cases and being found in 75 percent of patients dead from this cause. A median survival of 18 to 24 months is reported, and a probability of surviving 5 years of 20 percent. Therefore the objective of this study is to determine the difference of median survival depending on the presence of bone metastasis. Methods: A retrospective cohort study was conducted with 822 patients diagnosed with breast cancer between 2003 and 2010. The exposed cohort had scintigraphicly diagnosed bone metastasis, and the control group had no bone metastasis. Results: 8.88 percent of 822 patients, had bone metastasis. Those with bone metastasis had a mortality of 74 percent, and in the group with no bone metastasis, 16.69 percent died (p < 0.01). The median survival of patients with no metastasis was 44.3 months (IQR 35-83), and with metastasis was of 35 (IQR 18.6-46.1) (p < 0.01). With bone metastasis, over 48 months survival was 30.6 percent; and over 84 months 14.8 percent. Without bone metastasis, above 84 months, 78.51 percent survived (p < 0.01). Discussion and Conclusions: The difference between the two groups, considering global survival, is significant and implies an important decrease in survival and quality of life; the result obtained also differs from those reported in literature and it makes us reflect on the importance to consider bone metastasis not as a terminal event...

Introducción: El cáncer de mama es una importante causa de muerte por cáncer en las mujeres chilenas. Metastatiza a cualquier parte del cuerpo, siendo hueso la primera zona de diseminación en 26-50 por ciento de los casos, encontrándose un 75 por ciento de los pacientes que fallecen por esta causa. Se reporta una sobrevida de 18-24 meses, y una supervivencia a los 5 años del 20 por ciento. El objetivo del siguiente trabajo es determinar la sobrevida ante presencia de metástasis óseas. Pacientes y Método: Se realizó un estudio de cohortes retrospectivas con 822 pacientes diagnosticadas con cáncer de mama entre los años 2003 y 2010. La cohorte expuesta tiene diagnóstico cintigráfico de metástasis ósea, y la de control no tiene metástasis óseas. Resultados: 8,88 por ciento del total de 822 pacientes, presentó metástasis ósea. Con metástasis ósea existió una mortalidad del 74 por ciento, mientras que sin metástasis fue 16,69 por ciento (p < 0,01). La mediana de sobrevida sin metástasis ósea fue 44,3 meses (RIQ 35-83), en cambio, ante metástasis ósea fue 35 meses (RIQ 18,6-46,1) (p < 0,01). A los 48 meses, la supervivencia fue del 30,6 por ciento, para los pacientes con metástasis ósea y a los 84 meses, 14,8 por ciento, mientras que en los pacientes sin metástasis, la sobrevida a los 84 meses fue de 78,51 por ciento (p < 0,01). Discusión y Conclusiones: La diferencia de sobrevida entre pacientes con y sin metástasis ósea es significativa, implica una disminución en la supervivencia y la calidad de vida; difiere poco de lo reportado en la literatura, pero no debe hacernos considerar la metástasis ósea como un evento terminal de la enfermedad...

What is the best treatment option in postmenopausal, hormone responsive breast cancer patients with isolated bone metastases.

Background: Bone is the most common metastatic site for breast cancer. Aim: To determine the effectiveness of addition of chemotherapy to hormonal therapy in postmenopausal hormone receptor-positive breast cancer patients with isolated bone metastases. Materials and Methods: Between June 2001 and January 2007, 101 patients were classified into two groups according to initial treatment modalities; patients who received hormonotherapy only (group I) and chemotherapy followed by hormonotherapy (group II). The effect of treatment choice on clinical course, time to progression, and overall survival were evaluated. Results: There were 70 patients in group I and 31 patients in group II. Bone metastases in 27 patients (26.7%) were synchronous and the remainder were metachronous. The median follow-up time was 41 months. The two groups showed similar results when patients' tumor characteristics were compared. However, 81% of synchronous cases had upfront chemotherapy following hormonotherapy, whereas this ratio was only 12% in the metachronous group. All patients received systemic antiresorptive bisphosphonates whereas only 24 patients required palliative radiotherapy at some time during the course of their disease. In groups I and II, the median time to progression was 12 and 16 months (P: 0.96) and median overall survival was 41 and 40 months (P: 0.79), respectively. In HER-2-positive patients, a trend of prolongation of overall survival was observed in group II, but it was not statistically significant (P: 0.12). Conclusions: Anti-hormonal therapy still seems to be considered as the ideal treatment of choice for postmenapousal breast cancer patients with isolated bone metastases.

Osteosarcomas de la región de cabeza y cuello / Osteosarcoma of the head and neck area: analysis of 12 patients

Background: Less than 10 percent of osteosarcomas are located the head and neck region, mainly affecting the mandible and maxillary region. Aim: To analyze the therapeutic modality, types of reconstruction, surgical complications and survival of patients treated for osteosarcoma of the head and neck. Material and Methods: Review of medical records of 12 patients aged 17 to 34 years (6 women) treated for osteosarcoma of the head and neck between September 1998 and may 2009. Results: The localization of the tumor was maxillary in eight, mandibular three and ethmoidal in one patient. According to histologic grade, seven tumors were grade 1, four were grade 2 and one was grade 3. Adjuvant and neoadjuvant chemotherapy were administered to all and seven patients, respectively. The surgical treatment for maxillary tumors was maxillectomy. A reconstruction with rectum abdomini free flap was done in four patients and with dermoepidermic graft and an obturator prosthesis in three. In one patient, the defect was covered with a dental prosthesis. Treatment for mandibular tumors was mandibular excision. A reconstruction with peroneal free flap was performed in two patients and with an iliac crest graft in one. The ethmoidal tumor was resected and covered with local flaps. There were four complications. Two patients had a cutaneous fistula, one patient had a free flap partial atrophy and one patient had surgical site infection. Two patients who had positive margins died from local recurrence. Of the 10 patients with negative margins, seven are alive without recurrence, one died due to the disease, one has disseminated disease and one died from another cause. Conclusions: Our results in the treatment of head and neck osteosarcomas are consistent with published data.

Objetivos: Analizar la modalidad terapéutica, tipos de reconstrucción, complicaciones quirúrgicas y sobrevida de un grupo de pacientes tratados por esta patología. Métodos: Revisión de fichas clínicas de 12 pacientes portadores de osteosarcoma de cabeza y cuello entre septiembre de 1998 y mayo de 2009. Resultados: Edad promedio 30 años (17-34), 6 mujeres y 6 hombres. Localización fue 8 maxilar, 3 mandibular y 1 etmoidales. 7 tumores G1, 4 G2 y 1 G3. Siete pacientes recibieron quimioterapia neoadyuvante y todos adyuvante. El tratamiento de tumores maxilares fueron maxilectomías reconstruidas 4 con colgajo libre de recto abdominal, 3 con injerto dermoepidérmico más prótesis obturadora y una con prótesis dentaria. Se realizó mandibulectomía a los tumores mandibulares, 2 reconstruidos con colgajo libre de peroné y uno con injerto de cresta ilíaca. El tratamiento del tumor etmoidal fue resección craneofacial y se reparó con colgajos locales. Hubo 4 complicaciones; 2 fístulas cutáneas, una atrofia parcial de colgajo libre y una infección de herida operatoria. Dos pacientes tuvieron bordes comprometidos, quienes murieron por recidiva local. De los 10 pacientes con bordes libres, 7 se encuentran sin evidencia de recidiva, uno con enfermedad diseminada, uno fallecido por la enfermedad y uno fallecido por otra causa. Conclusiones: Nuestros resultados son consistentes con la literatura.

Mortalidad por cáncer infantil en Colombia durante 1985 a 2008 / Child mortality from cancer in Colombia, 1985-2008

OBJETIVO: Determinar la magnitud de la mortalidad por cáncer infantil en Colombia y evaluar las tendencias en su evolución entre 1985 y 2008. MÉTODOS: Se analizó durante dicho período la mortalidad en la población colombiana de 0 a 14 años provocada por cáncer en todas las localizaciones -leucemias, tumores malignos del sistema nervioso central (SNC), linfomas no Hodgkin, linfomas Hodgkin, tumores óseos y tumores renales. Se calculó el cambio promedio en las variaciones de las tendencias de mortalidad por cáncer en dicho grupo etario. RESULTADOS: Las muertes por cáncer constituyeron el 3,5 por ciento de la mortalidad en menores de 15 años. Entre los períodos 1985-1989 y 2005-2008 las tasas de mortalidad por cáncer mostraron un descenso en ambos sexos, pasando de 54,4 muertes por millón a 44,8 muertes por millón en niños y de 40,9 muertes por millón a 37,9 muertes por millón en niñas. La mortalidad por leucemias y linfomas registró un descenso estadísticamente significativo, mientras que la mortalidad por cánceres del SNC, contrariamente, aumentó también de manera significativa. CONCLUSIONES: Pese a leves tendencias a la baja en la mortalidad por leucemias y linfomas no Hodgkin, las tasas de mortalidad por cáncer infantil en Colombia permanecen altas y requieren esfuerzos importantes en los tratamientos para obtener mayores logros.

OBJECTIVE: Determine the magnitude of child mortality from cancer in Colombia and evaluate the trends in its evolution from 1985 to 2008. METHODS: Mortality in the Colombian population aged 0-14 years from cancer in any site (e.g., leukemia, malignant tumors of the central nervous system (CNS), nonHodgkin's lymphoma, Hodgkin's lymphoma, bone tumors, kidney tumors) during this period was analyzed. The mean change in the variations of cancer mortality trends in this age group was calculated. RESULTS: Deaths from cancer accounted for 3.5 percent of mortality in children under 15 years of age. During the periods 1985-1989 and 2005-2008 there was a decrease in mortality from cancer in both sexes, with figures dropping from 54.4 deaths per million to 44.8 deaths per million in boys and from 40.9 deaths per million to 37.9 deaths per million in girls. There was a statistically significant decrease in leukemia- and lymphoma-related mortality, whereas mortality associated with cancers of the CNS increased significantly. CONCLUSIONS: In spite of slight downward trends in mortality from leukemia and non-Hodgkin's lymphoma, childhood cancer mortality rates in Colombia remain high. Significant work on treatments for childhood cancer is required to obtain greater success.

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.

Chondrosarcoma of bone: does the size of the tumor, the presence of a pathologic fracture, or prior intervention have an impact on local control and survival?

AIMS AND OBJECTIVES: We studied 45 patients with chondrosarcoma, without metastasis at diagnosis, who were surgically treated between January 2000 and December 2004 to evaluate the risk factors associated with local recurrence and metastasis. MATERIALS AND METHODS: Fourteen (31%) patients had had some major prior intervention, either in the form of an open biopsy or a curettage / unplanned excision, before presenting to us. Eight patients had pathologic fractures at presentation. None of the patients received adjuvant chemotherapy or radiotherapy. The follow-up duration ranged from 8-75 months. All survivors had a minimum follow-up of 36 months (range 36-75 months). RESULTS: There were 11 grade 1 (24.5%), 23 grade 2 (51%), and 11 grade 3 (24.5%) chondrosarcomas. Thirty-two (71%) patients had tumors that were larger than 8 cm in the greatest dimension. Margins were adequate in 31 patients. Twenty-five patients had disease relapse; there were four local failures, nine distant failures, and 12 combined failures. At the time of the last review, 12 patients had died, 11 were alive with disease, and 22 were free of disease. The cumulative event-free survival was 44% and the overall survival was 73%. CONCLUSION: Grade of tumor, size of tumor, and adequacy of resection might be important predictors of outcome. Local recurrence is a prelude to distant metastasis and portends poor ultimate survival. The presence of a pathological fracture could indicate biologically aggressive disease, and limb salvage in these cases should be advised with caution. Even in cases where there has been a prior unplanned intervention, local control can be achieved by subsequent adequate resection.

Prognostic factors in the survival of patients diagnosed with primary non-metastatic osteosarcoma with a poor response to neoadjuvant chemotherapy

INTRODUCTION: Identification of variables that determine the prognosis for osteosarcoma may enable stratification of patients into subgroups with better or worse risk of local recurrence, metastases and death due to the disease. Discovery of such prognostic factors would permit selection of a subgroup of at-risk patients, with the aim of improving the therapeutic effectiveness. OBJECTIVE: To identify prognostic factors related to local recurrence-free survival, metastasis-free survival and overall survival among patients with highly malignant primary osteosarcoma that was non-metastatic on diagnosis and had poor response to neoadjuvant chemotherapy. SAMPLE AND METHODS: Out of 45 patients admitted to a referral center in Brazil between 2000 and 2004, 24 were selected for this study. RESULTS: The adverse factors that influenced the risk of local recurrence and the overall survival in univariate analysis were histological subtype other than osteoblastic (p = 0.017) and tumor size greater than 15 cm (p = 0.048). In relation to metastasis-free survival, the non-osteoblastic subtype had a worse prognosis (p = 0.007). The association of histological subtype maintained its significance in multivariate analysis for all studied survival categories. CONCLUSIONS: Tumor size greater than 15 cm is an adverse factor for local recurrence-free survival and overall survival but did not influence metastasis-free survival. The osteosarcoma histological type is a significant independent predictor for local recurrence-free survival, metastasis-free survival and overall survival.