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BACKGROUND: This study describes time-trends on epidemiology, subtypes and histopathological entities of osteosarcoma (OS) in a nationwide and unselected cohort of OS patients in Norway between 1975 and 2009. Few nationwide studies are published, and we still have particularly limited knowledge regarding patients not included in clinical trials comprising about half of the OS population. METHOD: Histologically verified skeletal OS for all subgroups were included, resulting in 473 eligible cases from a total of 702 evaluated patients. To ensure completeness, the present cohort was based on all cases reported to the Norwegian Cancer Registry, complemented with data from all Norwegian hospitals involved in sarcoma management. Survival analyses were performed with overall and sarcoma-specific survival as endpoints. RESULTS: Mean annual age-standard incidence amounted to about 3.8 per million in male and 2.8 per million in female with no clear time-trends. The male to female ratio was 1.4. Peak incidence was observed in the second decade for both genders. Conventional OS comprised 71.2% of all cases, while low grade OS represented 10.4% and telangiectatic OS only 1.3%. The most common primary site of OS was femur and tibia, respectively. The axial to appendicular ratio increased with the age. The overall 10-year survival did increase from about 30% during the late 1970s to around 50% 20 years later, with no subsequent improvement during the last two decades. Axial tumours, age above 40 years and overt metastatic disease at time of diagnosis were all negative prognostic factors. CONCLUSION: No improvement in the overall survival for OS since the 1990s was documented. The survival rates are still poor for elderly people, patients with axial disease and in the primary metastatic setting. The average incidence rate of skeletal OS in Norway was in line with international figures.
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Neoplasias Óseas/epidemiología , Osteosarcoma/epidemiología , Adolescente , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Neoplasias Óseas/mortalidad , Neoplasias Óseas/patología , Niño , Preescolar , Femenino , Humanos , Incidencia , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Clasificación del Tumor , Noruega/epidemiología , Osteosarcoma/mortalidad , Osteosarcoma/patología , Pronóstico , Factores de Riesgo , Distribución por Sexo , Tasa de Supervivencia/tendencias , Factores de Tiempo , Adulto JovenAsunto(s)
Neoplasias Óseas , Osteosarcoma , Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , NoruegaAsunto(s)
Neoplasias Óseas/epidemiología , Osteosarcoma/epidemiología , Adolescente , Adulto , Neoplasias Óseas/patología , Femenino , Humanos , Masculino , Noruega/epidemiología , Osteosarcoma/patología , Sistema de Registros/normas , Reproducibilidad de los Resultados , Suecia/epidemiología , Adulto JovenRESUMEN
Purpose: We aimed to evaluate the diagnostic potential of non-erectile multi-parametric magnetic resonance imaging (mpMRI) for preoperative assessment of primary penile squamous cell carcinoma (SCC). Method: Twenty-five patients who underwent surgery for penile SCC were included. Preoperative mpMRI without artificial erection was performed in all patients. The preoperative MRI protocol consisted of high-resolution morphological and functional sequences (diffusion-weighted imaging and dynamic contrast-enhanced MRI perfusion) covering the penis and lower pelvis. T and N staging, according to the 8th edition of the Union for International Cancer Control TNM classification, as well as the largest diameter and thickness/infiltration depth of the primary lesions were determined in all patients. Imaging data were retrospectively collected and compared with the final histopathology reports. Results: Very good agreement was observed between MRI and histopathology for the involvement of corpus spongiosum (p = 0.002) and good agreement was observed for the involvement of penile urethra and tunica albuginea/corpus cavernosum (p < 0.001 and p = 0.007, respectively). Good agreement was observed between MRI and histopathology for overall T staging and weaker, but still good agreement was observed for N staging (p < 0.001 and p = 0.002, respectively). A strong and significant correlation was observed between MRI and histopathology for the largest diameter and thickness/infiltration depth of the primary lesions (p < 0.001). Conclusions: Good concordance was observed between MRI and histopathological findings. Our initial findings indicate that non-erectile mpMRI is useful in preoperative assessment of primary penile SCC.
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BACKGROUND/AIM: This study aimed to identify the prognostic factors and outcomes of osteosarcoma (OS) located in proximal versus distal extremity long bones. PATIENTS AND METHODS: A nationwide cohort comprising all Norwegian high-grade OSs in extremity long bones between 1982 and 2009 was investigated. RESULTS: The univariate analysis results identified no significant differences in survival between patients with OS in proximal long bones (101 cases) as a group in comparison to patients with OS in the distal part of these bones (120 cases). However, proximal femur and primary metastasis were both independent adverse prognostic factors for sarcoma-specific survival in multivariate analyses, while elevated LDH and secondary OS were inferior prognostic factors for event-free survival. Adequate surgery and chemotherapy had a positive impact on survival. CONCLUSION: OS of the proximal femur had an unfavorable outcome in comparison to OS in other anatomical locations in extremity long bones.
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Neoplasias Óseas/epidemiología , Neoplasias Óseas/fisiopatología , Extremidades/fisiopatología , Osteosarcoma/fisiopatología , Pronóstico , Adolescente , Adulto , Estudios de Cohortes , Supervivencia sin Enfermedad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Noruega , Osteosarcoma/epidemiología , Adulto JovenRESUMEN
PURPOSE: To describe epidemiological and clinical characteristics, as well as long-term treatment outcomes of spindle cell non-osteogenic bone sarcomas (SCS), comprising leiomyosarcoma, fibrosarcoma and undifferentiated pleomorphic sarcoma in bone. METHOD: We have analysed a nationwide cohort of 104 patients with histologically verified SCS diagnosed between 1975 and 2009, based on registry sources supplemented with clinical records from Norwegian hospitals involved in sarcoma management. RESULTS: In this unselected cohort, a stable annual incidence for SCS patients of slightly below 0.6 per million was observed, with a dominant peak among elderly patients. SCS is mostly a high-grade malignancy (92%) with a male to female ratio of 1.6 for all patients. The axial to appendicular ratio was 0.7, seemingly independent of age. More than one fourth of the patients (29%) had primary metastatic disease. Another 32 patients (46%) developed metastases during follow-up and 12 (17%) experienced local relapses. The five-year sarcoma-specific survival rate was 37%, with no documented improvement over time. Primary metastatic disease was an adverse prognostic factor for survival. Predisposing factors were documented in 19 patients (18%). Negative prognostic factors for overall survival were tumour size >9 cm, age > 40 years, axial tumour localization, FS as subtype and pathologic fracture at time of diagnoses. As expected, patients who received both surgery and chemotherapy as their primary treatment for high-grade SCS (25%) significantly had best sarcoma specific five years survival (62%). CONCLUSION: We confirm SCS as a rare high-grade bone sarcoma entity, mostly among elderly patients and with a poor overall outcome. The combined treatment of surgery and chemotherapy is essential to achieve optimal long-term survival of SCS.
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Purpose. To describe epidemiological, clinical characteristics and treatment outcomes of low-grade osteosarcoma (LGOS), including dedifferentiated osteosarcoma (DLGOS). Method. We analysed a nationwide cohort comprised of patients with histologically verified LGOS and DLGOS between 1975 and 2009, based on registry sources supplemented with clinical records from hospitals involved in sarcoma management. Results. Fifty-four patients were identified, 12 of whom had DLGOS. The annual incidence for all patients was 0.3 per million, with the peak incidence in the third decade of the life. Fifteen patients experienced local relapses during follow-up and ten developed metastatic diseases, including three at primary diagnosis. Patients with DLGOS dominated the metastatic relapse group. The five-year sarcoma-specific survival rate was 91%, with no documented improvement over time. Free margin following surgical resection of the primary tumour had a positive impact on survival. As expected, both local relapse and metastasis during follow-up were associated with an unfavourable outcome. Radiotherapy predicted poor survival due to the selection of high-risk patients in need of such treatment. Neither higher age nor axial tumour localisation was adverse prognostic factors. Conclusion. LGOS has an excellent prognosis when surgically resected with a free margin; however, LGOS has the potential to dedifferentiate and metastasize with a poor outcome.
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AIM: To evaluate the clinicopathological features of extraskeletal osteosarcoma (ESOS) and its response to multimodal therapy. PATIENTS AND METHODS: A nationwide cohort comprising all Norwegian histologically verified ESOS patients between 1975 and 2009 supplemented with clinical reports from all hospitals involved in sarcoma management. RESULTS: Thirty-seven patients were classified as ESOS, mostly elderly people. Seventy-six % had an axial tumour, including nine patients with radiation-induced ESOS. The gender balance was equal. The 5-year sarcoma-specific survival (SSS) was 16 %. Adequate surgical remission had a positive impact on SSS, in contrast to chemotherapy and radiotherapy. Primary metastatic disease, elevated tumour size and elevated serum alkaline phosphatase, serum lactate dehydrogenase and Ki67, respectively, all predicted poor outcome. CONCLUSION: The relatively poor prognosis of ESOS may relate to both primary chemotherapy resistance and different biologic characteristics of these tumours as compared to conventional osteosarcoma. Hence, new predictive molecular markers and therapeutic approaches for treatment of ESOS are needed.
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Neoplasias Óseas/terapia , Osteosarcoma/terapia , Pronóstico , Anciano , Neoplasias Óseas/epidemiología , Neoplasias Óseas/patología , Proliferación Celular/genética , Terapia Combinada , Transición Epitelial-Mesenquimal/genética , Femenino , Humanos , Masculino , Noruega , Osteosarcoma/epidemiología , Osteosarcoma/genética , Osteosarcoma/patología , Resultado del TratamientoRESUMEN
Purpose. A retrospective study of prognostic factors and treatment outcome of osteosarcoma (OS) during modern chemotherapy era with focus on patients with primary metastatic disease, nonextremity localisation, or age >40 years (nonclassical OS). Methods. A nationwide cohort, comprising 424 high-grade Norwegian bone OS patients, was based on registry sources supplemented with clinical records from hospitals involved in sarcoma management between 1975 and 2009. Results. Only 48% were younger patients with tumour in the extremities and without metastasis at diagnosis (classical OS). A considerable discrepancy in survival between classical and nonclassical OS was observed: 61% versus 26% 10-year sarcoma specific survival. Twice as many of the former received both adequate surgery and chemotherapy compared to the latter. This could only partly explain the differences in survival due to inherent chemoresistance in primary metastatic disease and a higher rate of local relapse among patients with axial tumours. Metastasis at diagnosis, increased lactate dehydrogenase, age > 40 years, and tumour size above median value were all adverse prognostic factors for overall survival. Conclusion. We confirm a dramatic difference in outcome between classical and nonclassical high-grade OS patients, but treatment variables could only partly explain the dismal outcome of the latter.