Surgical Outcome and Oncological Survival of Osteofibrous Dysplasia-Like and Classic Adamantinomas: An International Multicenter Study of 318 Cases.

BACKGROUND: Osteofibrous dysplasia-like adamantinoma (OFD-AD) and classic adamantinoma (AD) are rare, neoplastic diseases with only limited data supporting current treatment protocols. We believe that our retrospective multicenter cohort study is the largest analysis of patients with adamantinoma to date. The primary purpose of this study was to describe the disease characteristics and evaluate the oncological outcomes. The secondary purpose was to identify risk factors for local recurrence after surgical treatment and propose treatment guidelines. METHODS: Three hundred and eighteen confirmed cases of OFD-AD and AD for which primary treatment was carried out between 1985 and 2015 were submitted by 22 tertiary bone tumor centers. Proposed clinical risk factors for local recurrence such as size, type, and margins were analyzed using univariable and multivariate Cox regression analysis. RESULTS: Of the 318 cases, 128 were OFD-AD and 190 were AD. The mean age at diagnosis was 17 years (median, 14.5 years) for OFD-AD and 32 years (median, 28 years) for AD; 53% of the patients were female. The mean tumor size in the OFD-AD and AD groups combined was 7.8 cm, measured histologically. Sixteen percent of the patients sustained a pathological fracture prior to treatment. Local recurrence was recorded in 22% of the OFD-AD cases and 24% of the AD cases. None of the recurrences in the OFD-AD group progressed to AD. Metastatic disease was found in 18% of the AD cases and fatal disease, in 11% of the AD cases. No metastatic or fatal disease was reported in the OFD-AD group. Multivariate Cox regression analysis demonstrated that uncontaminated resection margins (hazard ratio [HR] = 0.164, 95% confidence interval [CI] = 0.092 to 0.290, p < 0.001), pathological fracture (HR = 1.968, 95% CI = 1.076 to 3.600, p = 0.028), and sex (female versus male: HR = 0.535, 95% CI = 0.300 to 0.952, p = 0.033) impacted the risk of local recurrence. CONCLUSIONS: OFD-AD and AD are parts of a disease spectrum but should be regarded as different entities. Our results support reclassification of OFD-AD into the intermediate locally aggressive category, based on the local recurrence rate of 22% and absence of metastases. In our study, metastatic disease was restricted to the AD group (an 18% rate). We advocate wide resection with uncontaminated margins including bone and involved periosteum for both OFD-AD and AD. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Factores de riesgo para artrosis precoz en el tumor de células gigantes tenosinovial / Risk factors for early osteoarthritis in tenosynovial giant cell tumour

OBJETIVO: El tumor de células gigantes tenosinovial (TCGT) es una entidad localmente agresiva, que afecta a pacientes jóvenes (en torno a la 4.ª década de vida) y puede producir destrucción articular. Presenta 2 variedades, nodular y difusa, similares histológica y genéticamente, pero con diferente pronóstico. El objetivo de este estudio es identificar los factores predisponentes para el desarrollo de artrosis precoz y de recidiva local en estos pacientes. MATERIAL Y MÉTODOS: Hemos realizado un estudio retrospectivo de 35 pacientes con diagnóstico anatomopatológico de TCGT en nuestra institución desde 1991 hasta 2017. Se recogieron variables demográficas, características del tumor primario y de su evolución para identificar posibles factores de riesgo. El tiempo medio de seguimiento fue 8.5 años (rango: 1,1-19,8). RESULTADOS: La variante difusa presenta más riesgo de artrosis que la nodular (p = 0,01), y mayor tasa de recidiva local (p = 0,015). La artrosis fue más frecuente en la cadera y el tobillo (p = 0,03) que en la rodilla. Hemos observado una diferencia de 16 meses en la duración de los síntomas previos al diagnóstico entre los que desarrollaron artrosis y los que no (p = 0,05). CONCLUSIONES: La variante difusa es más agresiva que la nodular, y se asocia con mayor riesgo de artrosis y de recidiva local. La cadera y el tobillo presentan mayor riesgo de artrosis que otras articulaciones. El tiempo de evolución de los síntomas antes del diagnóstico y del tratamiento adecuado, puede influir de forma negativa en el desarrollo de artrosis

OBJECTIVE: Tenosynovial giant cell tumour (TGCT) is locally aggressive entity affecting young people (around 4th decade of life) and can cause joint destruction. It could be nodular or diffuse. These two varieties are histological and genetically similar, but present a different prognosis. The aim of this study is to identify risk factors for local recurrence and predisposing factors for the development of early osteoarthritis in patients with TGCT. MATERIAL AND METHODS: We conducted a retrospective study of 35 patients with an anatomopathological diagnosis of TGCT in our Institution from 1991 to 2017. The mean follow-up was 8.2 years. Demographic variables, characteristics of the primary tumor and its evolution were collected to assess the risk factors for local recurrence and early osteoarthritis. RESULTS: The diffuse type was identified as a risk factor for the development of osteoarthritis (p = 0.01) and for local recurrence (p = 0.015). Osteoarthritis was more frequent in the hip and ankle than in the knee (p = 0.03). A difference of 16 months in the duration of symptoms prior to diagnosis between those who developed osteoarthritis and those who did not was observed (p = 0.05). CONCLUSIONS: The diffuse type is more aggressive than the nodular type; it is associated with a higher risk of osteoarthritis and local recurrence. The hip and ankle present a higher risk of osteoarthritis than other joints. The time of evolution of the symptoms before diagnosis and adequate treatment, negatively influences the development of osteoarthritis

Risk factors for early osteoarthritis in tenosynovial giant cell tumour. / Factores de riesgo para artrosis precoz en el tumor de células gigantes tenosinovial.

OBJECTIVE: Tenosynovial giant cell tumour (TGCT) is locally aggressive entity affecting young people (around 4th decade of life) and can cause joint destruction. It could be nodular or diffuse. These two varieties are histological and genetically similar, but present a different prognosis. The aim of this study is to identify risk factors for local recurrence and predisposing factors for the development of early osteoarthritis in patients with TGCT. MATERIAL AND METHODS: We conducted a retrospective study of 35 patients with an anatomopathological diagnosis of TGCT in our Institution from 1991 to 2017. The mean follow-up was 8.2 years. Demographic variables, characteristics of the primary tumor and its evolution were collected to assess the risk factors for local recurrence and early osteoarthritis. RESULTS: The diffuse type was identified as a risk factor for the development of osteoarthritis (p=0.01) and for local recurrence (p=0.015). Osteoarthritis was more frequent in the hip and ankle than in the knee (p=0.03). A difference of 16 months in the duration of symptoms prior to diagnosis between those who developed osteoarthritis and those who did not was observed (p=0.05). CONCLUSIONS: The diffuse type is more aggressive than the nodular type; it is associated with a higher risk of osteoarthritis and local recurrence. The hip and ankle present a higher risk of osteoarthritis than other joints. The time of evolution of the symptoms before diagnosis and adequate treatment, negatively influences the development of osteoarthritis.

Pronóstico y tratamiento de las acrometástasis: estudio observacional de 35 casos tratados en un único centro / Prognosis and treatment of acrometastases: Observational study of 35 cases treated in a single institution

Objetivo: Existe cierta contradicción en la literatura acerca del tumor primario que se asocia con más frecuencia a las acrometástasis, así como acerca de su pronóstico y tratamiento. El objetivo de nuestro trabajo es determinar la localización más frecuente de las acrometástasis, el tumor primario más frecuente según su localización y evaluar las opciones quirúrgicas en función de la supervivencia estimada. Métodos: Hemos realizado un estudio retrospectivo de los pacientes diagnosticados de acrometástasis en nuestra institución. Se recogieron variables epidemiológicas, así como información acerca de tumor primario y número, localización, síntomas y tratamiento de las acrometástasis. Resultados: Se revisaron 35 acrometástasis en 21 pacientes: 4 en miembros superiores y 31 en miembros inferiores, siendo la tibia el hueso más frecuentemente afectado (n=13). El tumor primario que con más frecuencia produce acrometástasis en miembros inferiores es el hipernefroma (19%); en los miembros superiores, sin embargo, el pulmón fue más frecuente (25%). Once pacientes fallecieron (52%), de los cuales 10 tenían metástasis en múltiples localizaciones. La media de supervivencia fue de 42 meses desde el diagnóstico de las acrometástasis. Conclusión: Las acrometástasis se observan con más frecuencia en las extremidades inferiores, y el tumor primario más frecuente es el hipernefroma, seguido del de pulmón. El tratamiento de las acrometástasis depende del pronóstico del paciente, por lo que es esencial un abordaje multidisciplinar. La media de supervivencia en nuestra serie no implica un peor pronóstico de las acrometástasis, especialmente si son únicas, por lo que es factible considerar opciones quirúrgicas como la resección amplia y reconstrucción

Objective: There is a degree of contradiction in the literature about the primary tumour that is most frequently associated with acrometastases, as well as their prognosis and treatment. The aim of this study is to determine the most frequent location of the acrometastases, the most frequent primary tumour according to its location, and to evaluate the surgical options according to the estimated survival. Methods: A retrospective study on patients diagnosed with acrometastases. The primary tumour, as well as the number, location, symptoms and treatment of the acrometastases and survival rate were collected. Results: 35 acrometastases in 21 patients were reviewed: 4 in the upper limbs and 31 in the lower limbs; the tibia was the most frequently affected bone (N=13). The primary tumour that most frequently produced acrometastases in the lower limb was hypernephroma (19%). In the upper limb lung tumour was more frequent (25%). Eleven patients died (52%); 10 of them had concomitant multiple metastases. The mean survival was 42 months from the diagnosis of acrometastases. Conclusion: Acrometastases are more frequently seen in the lower extremities and the most frequent tumour was hypernephroma followed by lung. The treatment of acrometastases depends on the prognosis of the primary tumour, so a multidisciplinary approach is essential. The mean survival in our series did not imply a poorer prognosis for acrometastases, so it is feasible to consider surgical options such as wide resection and reconstruction. A unique acrometastases is a good prognosis signal

Prognosis and treatment of acrometastases: Observational study of 35 cases treated in a single institution. / Pronóstico y tratamiento de las acrometástasis: estudio observacional de 35 casos tratados en un único centro.

OBJECTIVE: There is a degree of contradiction in the literature about the primary tumour that is most frequently associated with acrometastases, as well as their prognosis and treatment. The aim of this study is to determine the most frequent location of the acrometastases, the most frequent primary tumour according to its location, and to evaluate the surgical options according to the estimated survival. METHODS: A retrospective study on patients diagnosed with acrometastases. The primary tumour, as well as the number, location, symptoms and treatment of the acrometastases and survival rate were collected. RESULTS: 35 acrometastases in 21 patients were reviewed: 4 in the upper limbs and 31 in the lower limbs; the tibia was the most frequently affected bone (N=13). The primary tumour that most frequently produced acrometastases in the lower limb was hypernephroma (19%). In the upper limb lung tumour was more frequent (25%). Eleven patients died (52%); 10 of them had concomitant multiple metastases. The mean survival was 42 months from the diagnosis of acrometastases. CONCLUSION: Acrometastases are more frequently seen in the lower extremities and the most frequent tumour was hypernephroma followed by lung. The treatment of acrometastases depends on the prognosis of the primary tumour, so a multidisciplinary approach is essential. The mean survival in our series did not imply a poorer prognosis for acrometastases, so it is feasible to consider surgical options such as wide resection and reconstruction. A unique acrometastases is a good prognosis signal.

Serum and gene expression levels of CT-1, IL-6, and TNF-α after a lifestyle intervention in obese children.

BACKGROUND: Inflammation related molecules such as tumor necrosis factor-α (TNF-α), interleukin 6 (IL-6), and cardiotrophin-1 (CT-1) are highly expressed in obese individuals and could partly explain some comorbidities associated to obesity. In obese children, lifestyle interventions are able to lower inflammation and reduce cardiovascular risk factors associated with obesity. The aim of the present work was to study changes in inflammation-related molecules serum and peripheral blood mononuclear cells (PBMC) transcript levels after a 10-week lifestyle intervention in obese children and asses their potential association with glucose metabolism. METHODS: Twenty-three obese children (mean age 11.5 years; 48% males) underwent a 10-week lifestyle not controlled intervention trial. Anthropometric and biochemical measurements were analyzed. Transcript analysis for CT-1, IL-6, and TNF-α in PBMC were performed by RT-PCR. Serum cytokine levels were also measured at baseline and after 10-weeks. RESULTS: Participants achieved a significant reduction in body adiposity (0.34 decrease in body mass index-standard deviation), total cholesterol, and glucose levels after 10-weeks. A Significant decrease in serum TNF-α and C reactive protein (CRP) were observed. CT-1 transcript levels were significantly reduced (P = .005) after lifestyle intervention, and these changes were significantly correlated with changes in serum CT-1 levels (r = 0.451; P = .031). In multiple regression analysis baseline CT-1 transcript levels were positively associated with final insulin (R2 = 0.506; P = .035) and HOMA-IR values (R2 = 0.473; P = .034). CONCLUSIONS: We reported that serum CRP, TNF-α, as well as PBMC CT-1 transcript levels were reduced after lifestyle intervention in obese children. More studies are needed to clarify the role of inflammation-related molecules in glucose metabolism.

Factores de riesgo para la recidiva local de la fibromatosis / Risk factors for local recurrence of fibromatosis

Objetivo. Valorar los factores clínicos, radiológicos e histológicos que pueden predecir la recidiva local de fibromatosis. Métodos. Hemos realizado un estudio retrospectivo de 51 pacientes con diagnóstico de fibromatosis en nuestra institución desde 1983 hasta 2014. La media de seguimiento es de 83 meses. Hemos estudiado parámetros clínicos, localización, profundidad, tamaño, márgenes quirúrgicos e índice de proliferación (Ki-67) del tumor. Asimismo, hemos valorado el riesgo de recidiva en función del tratamiento adyuvante y la relación del tratamiento con la funcionalidad del paciente. Resultados. Hemos observado diferencias estadísticamente significativas en cuanto a la profundidad (p=0,003) y la localización (p<0,001) como factores de riesgo de recidiva local. No existen diferencias estadísticamente significativas en cuanto a edad, sexo, tamaño, márgenes quirúrgicos ni tratamientos adyuvantes, ni en el Musculoskeletal Tumor Society Score en función del tratamiento recibido. El Ki-67 tiene una media de 1,9% (rango 1-4) y su valor no se asocia con el riesgo de recidiva. Discusión. Los tumores profundos a la fascia y los localizados en extremidades son más agresivos que los localizados superficialmente y en el tronco. El Ki-67 no tiene valor predictivo en las recidivas de la fibromatosis. La radioterapia, la quimioterapia u otros tratamientos adyuvantes como el tamoxifeno no han sido eficaces en el control local de la enfermedad. La gran cantidad de recidivas, incluso en pacientes con resecciones adecuadas, induce a plantear la posibilidad de una actitud expectante en tumores asintomáticos o en enfermedad estable (AU)

Objective. To evaluate the clinical, radiological and histological factors that can predict local recurrence of fibromatosis. Methods. A retrospective study was conducted on 51 patients diagnosed with fibromatosis in this hospital from 1983 to 2014. The mean follow-up was 83 months. A study was made of the clinical parameters, location, depth, size, surgical margins, and proliferation index (Ki-67). An evaluation was also made of the risk of recurrence depending on the adjuvant treatment and the relationship between treatment and patient functionality. Results. Tumour location and depth were identified as risk factors for local recurrence, showing statistically significant differences (P<.001 and P=.003, respectively). There were no statistically significant differences in age, gender, size, surgical margins, or adjuvant treatments, or in the Musculoskeletal Tumour Society Score according to the treatment received. The mean Ki-67 was 1.9% (range 1-4), and its value was not associated with the risk of recurrence. Discussion. Deep fibromatosis fascia tumours, and those located in extremities are more aggressive than superficial tumours and those located in trunk. The Ki-67 has no predictive value in local recurrence of fibromatosis. Radiotherapy, chemotherapy, or other adjuvant treatments such as tamoxifen have not been effective in local control of the disease. Given the high recurrence rate, even with adequate margins, a wait and see attitude should be considered in asymptomatic patients and/or stable disease (AU)

Risk factors for local recurrence of fibromatosis. / Factores de riesgo para la recidiva local de la fibromatosis.

OBJECTIVE: To evaluate the clinical, radiological and histological factors that can predict local recurrence of fibromatosis. METHODS: A retrospective study was conducted on 51 patients diagnosed with fibromatosis in this hospital from 1983 to 2014. The mean follow-up was 83 months. A study was made of the clinical parameters, location, depth, size, surgical margins, and proliferation index (Ki-67). An evaluation was also made of the risk of recurrence depending on the adjuvant treatment and the relationship between treatment and patient functionality. RESULTS: Tumour location and depth were identified as risk factors for local recurrence, showing statistically significant differences (P<.001 and P=.003, respectively). There were no statistically significant differences in age, gender, size, surgical margins, or adjuvant treatments, or in the Musculoskeletal Tumour Society Score according to the treatment received. The mean Ki-67 was 1.9% (range 1-4), and its value was not associated with the risk of recurrence. DISCUSSION: Deep fibromatosis fascia tumours, and those located in extremities are more aggressive than superficial tumours and those located in trunk. The Ki-67 has no predictive value in local recurrence of fibromatosis. Radiotherapy, chemotherapy, or other adjuvant treatments such as tamoxifen have not been effective in local control of the disease. Given the high recurrence rate, even with adequate margins, a wait and see attitude should be considered in asymptomatic patients and/or stable disease.

Behavioral predictors of attrition in adolescents participating in a multidisciplinary obesity treatment program: EVASYON study.

The aims of this study were to identify the cognitive and behavioral predictors of dropping out and to estimate the attrition rate during different phases of an intervention program to treat overweight and obesity in adolescents. Overweight/obese adolescents (n=156, aged: 13-16 years; 71 male and 85 female subjects) were included in a multicomponent (diet, physical activity and psychological support) family-based group treatment program. At baseline and after 2 months (intensive phase) and 13 months (extensive phase) of follow-up, we measured adolescents' cognitive and behavioral dimensions, together with the parents' perception of their child's behavior. Of the 156 adolescents selected, 112 completed the full program (drop-out rate of 28.2%). The risk of dropping out during the extensive phase increased by 20% for each unit increase in the adolescent's social insecurity score (odds ratio=1.20, 95% confidence interval=1.07-1.34, P=0.002). The adolescents who had a high interoceptive awareness showed a significant decrease of 13.0% in the probability of dropping out (odds ratio=0.87, 95% confidence interval=0.77-0.99, P=0.040). Adolescents' social insecurity was the main predictor of drop-out in a multicomponent family-group-based obesity treatment program. To reduce attrition rates in these programs, the individual's social insecurity level needs to be reduced, whereas the family's awareness of eating-related behavior needs adjustment.

Dietary fatty acid distribution modifies obesity risk linked to the rs9939609 polymorphism of the fat mass and obesity-associated gene in a Spanish case-control study of children.

The rs9939609 polymorphism of the fat mass and obesity-associated (FTO) gene has been widely associated with childhood obesity in several European cohorts. This association appears to be dependent on dietary macronutrients. Therefore, the aim of the present study was to evaluate whether dietary fatty acid intake distribution could interact with this FTO genetic variation and obesity in a Spanish case-control study of children and adolescents. A total of 354 Spanish children and adolescents aged 6-18 years (49 % males) were genotyped for the rs9939609 variant of the FTO gene. Anthropometric parameters were taken and energy intake was measured. We observed an interaction between the consumption of SFA (percentage of total energy) and PUFA:SFA ratio and obesity risk linked to the rs9939609 SNP of the FTO gene. In the study population of the present study, the risk allele carriers consuming more than 12·6 % SFA (of total energy) had an increased obesity risk compared with TT carriers. In a similar way, A allele carriers with an intake ratio lower than 0·43 PUFA:SFA presented a higher obesity risk than TT subjects. In summary, the present study reports for the first time the influence of dietary fatty acid distribution on the effect of the rs9939609 polymorphism of the FTO gene on children and adolescents' obesity risk.

Enfermedad de Ollier: tumores benignos con riesgo de malignización. Revisión de 17 casos / Ollier disease: benign tumours with risk of malignant transformation. A review of 17 cases

Objetivo: Analizar una larga serie de esta rara entidad para entenderla mejor y valorar el riesgo de malignización y comprender esta enfermedad infrecuente para el correcto diagnóstico y tratamiento. Material y métodos: Diecisiete casos de encondromatosis múltiple tratados desde el año 1976 hasta el año 2006. Las variables estudiadas son: edad al diagnóstico y malignización, localización de la lesión, asociación familiar con otros tumores, hallazgos radiológicos, tratamientos aplicados, evolución en el tiempo, metástasis y supervivencia. Resultados: Se incluyen 16 casos de Ollier y un caso de síndrome de Maffucci. La gammagrafía fue el método de imagen que nos llevó al diagnóstico. La mediana de edad al diagnóstico de las malignizaciones es de 45 años, mientras que la mediana de edad al diagnóstico de los que no sufrieron malignización fue de 11 años. Fracturas patológicas, dismetrías y deformidades conducen al diagnóstico en la gente joven. Las lesiones benignas fueron tratadas mediante cirugía menor. Hubo 5 malignizaciones en 4 pacientes (23,5%). El fémur distal y la pelvis son los lugares de mayor frecuencia de malignización, todos ellos hacia condrosarcoma de grado I. La cirugía es el único tratamiento en todos ellos. Conclusiones: La enfermedad de Ollier es una enfermedad benigna con alto riesgo de malignización a partir de los 40 años. La gammagrafía ósea es la mejor prueba diagnostica para detectar el crecimiento del tumor y la posible malinización de las múltiples lesiones. La cirugía amplia es la única herramienta para lograr la supervivencia en estos pacientes; por eso el diagnóstico precoz es esencial (AU)

Aim: To review Ollier disease, an uncommon disease, in order to understand the clinical symptoms, diagnosis, correct treatment, and risk factors in order to prevent malignant transformation. Materials and methods: Seventeen cases of Ollier disease were treated between 1976 and 2006. The variables studied included: age at diagnosis and onset of malignant transformation, location of the lesion, family association with other tumours, radiological findings, treatment for the different lesions, metastatic lesions and survival. We excluded patients with Chondrosarcoma without previous diagnosis of Ollier disease. All malignant transformations were assessed by histopathology studies and radiological images. Results: We include 16 cases of Ollier's and 1 of Maffucci's syndrome. The median age at diagnosis for patients having malignant transformation was 45 years, whereas the average age at diagnosis for patient without progression to malignancy was 11 years. Pathological fractures, dysmetria and deformities led to the diagnosis in young people. Benign lesions were treated with minor surgery. There were five malignancy transformations in four patients (23.5%), with the distal femur being the most frequent location for transformation to grade I chondrosarcoma. Surgery was the treatment in all of them. We found no family association with other tumours. Conclusions: Multiple enchondromatosis is a benign disease with a high risk of malignant transformation above 40 years old. Bone scintigraphy is the best tool for detecting multiple lesions and malignant transformation. Radical surgery is the only way to improve survival, and this is the reason why early diagnosis is essential (AU)

[Ollier disease: benign tumours with risk of malignant transformation. A review of 17 cases]. / Enfermedad de Ollier: tumores benignos con riesgo de malignización. Revisión de 17 casos.

AIM: To review Ollier disease, an uncommon disease, in order to understand the clinical symptoms, diagnosis, correct treatment, and risk factors in order to prevent malignant transformation. MATERIALS AND METHODS: Seventeen cases of Ollier disease were treated between 1976 and 2006. The variables studied included: age at diagnosis and onset of malignant transformation, location of the lesion, family association with other tumours, radiological findings, treatment for the different lesions, metastatic lesions and survival. We excluded patients with Chondrosarcoma without previous diagnosis of Ollier disease. All malignant transformations were assessed by histopathology studies and radiological images. RESULTS: We include 16 cases of Ollier's and 1 of Maffucci's syndrome. The median age at diagnosis for patients having malignant transformation was 45 years, whereas the average age at diagnosis for patient without progression to malignancy was 11 years. Pathological fractures, dysmetria and deformities led to the diagnosis in young people. Benign lesions were treated with minor surgery. There were five malignancy transformations in four patients (23.5%), with the distal femur being the most frequent location for transformation to grade I chondrosarcoma. Surgery was the treatment in all of them. We found no family association with other tumours. CONCLUSIONS: Multiple enchondromatosis is a benign disease with a high risk of malignant transformation above 40 years old. Bone scintigraphy is the best tool for detecting multiple lesions and malignant transformation. Radical surgery is the only way to improve survival, and this is the reason why early diagnosis is essential.

EULAR/EFORT recommendations for the diagnosis and initial management of patients with acute or recent onset swelling of the knee.

OBJECTIVES: The European League Against Rheumatism (EULAR) and the European Federation of National Associations of Orthopaedics and Traumatology (EFORT) have recognised the importance of variation in diagnostic and therapeutic performance across disciplines, have found consensus in starting task forces aiming at achieving diagnostic and therapeutic uniformity, and have identified medical conditions with which representatives of both organisations will frequently be confronted in common clinical practice. The aim of the present work was to establish recommendations for the diagnosis and initial management of patients presenting with acute or recent onset swelling of the knee. METHODS: The EULAR standard operating procedures for the elaboration and implementation of evidence-based recommendations were followed. RESULTS: In all, 11 rheumatologists from 11 countries and 12 orthopaedic surgeons from 7 countries met twice under the leadership of 2 conveners, a clinical epidemiologist and a research fellow. After carefully defining the content and procedures of the task force, research questions were developed, a comprehensive literature search was performed and the results were presented to the entire committee. Subsequently, a set of 10 recommendations was formulated based on evidence from the literature if available, and after discussion and consensus building. CONCLUSIONS: This is the first combined interdisciplinary project of rheumatologists and orthopaedic surgeons, successfully aiming at achieving consensus in the diagnosis and initial management of patients presenting with acute or recent onset swelling of the knee.

Sarcoma de partes blandas: ¿existe posibilidad de rescate cuando la primera cirugía no fue resolutiva? / Soft tissue sarcoma: can a rescue procedure be performed when the first surgery was unsuccessful?

Objetivo. El objetivo de este trabajo es revisar la experiencia de nuestro centro en el tratamiento de pacientes diagnosticados de sarcoma de partes blandas (SPB) en una extremidad que consultan tras resecciones quirúrgicas inadecuadas o recidiva local. Material y método. Se trata de un estudio retrospectivo de 64 pacientes remitidos tras el tratamiento de un SPB en otro centro, divididos en 2 grupos: el grupo A, compuesto por 27 pacientes a quienes se realizó una escisión inadecuada inicial (whoops procedure) y el grupo B, con 37 pacientes afectos de una recidiva local de un SPB. Se calculó la tasa de supervivencia libre de enfermedad y la tasa de supervivencia acumulada (Kaplan-Meier). Resultados. Grupo A: la totalidad de los 27 pacientes fueron reintervenidos y en 12 casos se detectó enfermedad tumoral residual (un 44%). Veintitrés pacientes recibieron radioterapia asociada (intraoperatoria, braquiterapia y/o externa). El seguimiento medio ha sido de 67 meses (24-216) Tres pacientes presentaron recidiva local, uno de los cuales precisó amputación. El 11% de los pacientes habían fallecido en el momento de la revisión. La tasa de supervivencia libre de enfermedad a los 216 meses ha sido del 85%. Grupo B: 35 de los 37 pacientes fueron reintervenidos (94%). En 21 casos se asoció quimioterapia y en 4 perfusión aislada de la extremidad con factor de necrosis tumoral (TNF) y melfalan (10,8%). Veintisiete pacientes recibieron radioterapia (externa, intraoperatoria y/o braquiterapia) (72%), 19 de ellos habían recibido ya radioterapia después de la primera (70%). En 20 casos (10%) se asoció quimio-terapia y radioterapia. La media de seguimiento ha sido de 80 meses (12-264). Dieciséis pacientes presentaron metástasis después del tratamiento y diecinueve tuvieron complicaciones mayores. El 43% de los pacientes había fallecido en el momento de la revisión. La tasa de supervivencia libre de enfermedad a los 264 meses ha sido del 16%. Conclusiones. Después de una escisión inadecuada inicial se puede obtener una alta tasa de supervivencia libre de enfermedad en pacientes remitidos inmediatamente a centros especializados. Sin embargo, cuando aparece la recidiva local, las posibilidades de supervivencia libre de enfermedad disminuyen drásticamente

Purpose. The purpose of this paper is to review the experience of our hospital in treating patients diagnosed with a soft-tissue sarcoma (STS) in one of their limbs who sought consultation further to inappropriate surgical resections or a local relapse. Materials and methods. This is a retrospective study of 64 patients treated for STS in another hospital; the patients were divided into 2 groups: group A, comprised 27 patients where the initial excision proved to be inappropriate («whoops procedure»); group B was made up of 37 patients that had a local recurrence of a STS. The disease-free and accumulated (Kaplan-Meier) survivorship rates were calculated. Results. Group A: all 27 patients were reoperated and in 12 cases a residual tumoral disease was detected (44%). Twenty-three patients received associated radiotherapy (intraoperatively, brachytherapy and/or external beam radiotherapy). Mean follow up was 67 months (24-216) Three had a local recurrence, two of them requiring amputation. Eleven percent of patients had died at the time of examination. The disease-free survivorship rate at 216 months was 85%. Group B: 35 of the 37 patients were reoperated (94%). Chemotherapy was used in 21 cases and in four cases isolated limb perfusion was used with TNF and melphalan (10.8%). Twenty-seven patients received radiotherapy (external beam, intraoperative and/or brachytherapy) (72%), 19 of them had received radiotherapy after the first one (70%). In 20 cases (10%) both chemotherapy and radiotherapy were used. Mean follow-up was 80 months (range: 12-264). Sixteen patients had metastasis further to treatment and nineteen had major complications. Forty-three percent of patients had died at the time of this review. Disease-free survivorship at 264 months was 16%. Conclusions. After a «whoops procedure» it is possible to obtain a high disease-free survivorship rate in patients referred immediately to specialized units. Nevertheless, when local recurrence occurs, the disease-free survivorship rate decreases sharply

[Bone transplant]. / Trasplante óseo.

We describe the methodology of the Bone and Soft Tissue Bank, from extraction and storage until use. Since the year 1986, with the creation of the Bone Bank in the University Clinic of Navarra, more than 3,000 grafts have been used for very different types of surgery. Bone grafts can be classified into cortical and spongy; the former are principally used in surgery to save tumour patients, in large post-traumatic reconstructions and in replacement surgery where there are massive bone defects and a structural support is required. The spongy grafts are the most used due to their numerous indications; they are especially useful in filling cavities that require a significant quantity of graft when the autograft is insufficient, or as a complement. They are also of special help in treating fractures when there is bone loss and in the treatment of delays in consolidation and pseudoarthrosis in little vascularized and atrophic zones. They are also used in prosthetic surgery against the presence of cavity type defects. Allografts of soft tissues are specially recognised in multiple ligament injuries that require reconstructions. Nowadays, the most utilised are those employed in surgery of the anterior cruciate ligament although they can be used for filling any ligament or tendon defect. The principal difficulties of the cortical allografts are in the consolidation of the ends with the bone itself and in tumour surgery, given that these are patients immunodepressed by the treatment, the incidence of infection is increased with respect to spongy grafts and soft tissues, which is irrelevant. In short, the increasingly widespread use of allografts is an essential therapeutic weapon in orthopaedic surgery and traumatology. It must be used by expert hands.

Trasplante óseo / Bone transplant

Describimos la metodología del Banco de Huesos y Tejidos blandos desde su extracción y su almacenamiento hasta su utilización. Desde el año 1986 con la creación del Banco de Huesos en la Clínica Universitaria de Navarra, se han utilizado más de 3.000 injertos para cirugías muy diversas. Los injertos óseos se pueden clasificar en corticales y esponjosos; los primeros se emplean principalmente en cirugía de salvamento en pacientes tumorales, en grandes reconstrucciones postraumáticas y en cirugía de reemplazo en el que los defectos óseos son masivos y requieren un soporte estructural. Los injertos esponjosos son los más utilizados por sus numerosas indicaciones, de especial utilidad en el relleno de cavidades que precisan una cantidad importante de injerto cuando el autoinjerto es insuficiente o como complemento; también son de especial ayuda en el tratamiento de las fracturas cuando existen pérdidas óseas y en el tratamiento de los retrasos de consolidación y seudoartrosis en zonas poco vascularizadas y atróficas. Se usan también en cirugía protésica ante la presencia de defectos de tipo cavitario. Los aloinjertos de tejidos blandos están especialmente reconocidos en lesiones ligamentosas múltiples que requieren reconstrucciones. Hoy en día, los más utilizados son los empleados en la cirugía del ligamento cruzado anterior aunque pueden ser utilizados para reemplazar cualquier defecto ligamentoso o tendinoso. Las principales dificultades de los aloinjertos corticales están en la consolidación de los extremos con el hueso propio y en cirugía tumoral, dado que son enfermos inmunodeprimidos por el tratamiento, la incidencia de infección se encuentra aumentada con respecto a los injertos esponjosos y de tejidos blandos que es irrelevante. En definitiva, el uso de aloinjertos cada vez más extendido es un arma terapéutica imprescindible en cirugía ortopédica y traumatología, que debe ser usada por manos expertas

We describe the methodology of the Bone and Soft Tissue Bank, from extraction and storage until use. Since the year 1986, with the creation of the Bone Bank in the University Clinic of Navarra, more than 3,000 grafts have been used for very different types of surgery. Bone grafts can be classified into cortical and spongy; the former are principally used in surgery to save tumour patients, in large post-traumatic reconstructions and in replacement surgery where there are massive bone defects and a structural support is required. The spongy grafts are the most used due to their numerous indications; they are especially useful in filling cavities that require a significant quantity of graft when the autograft is insufficient, or as a complement. They are also of special help in treating fractures when there is bone loss and in the treatment of delays in consolidation and pseudoarthrosis in little vascularized and atrophic zones. They are also used in prosthetic surgery against the presence of cavity type defects. Allografts of soft tissues are specially recognised in multiple ligament injuries that require reconstructions. Nowadays, the most utilised are those employed in surgery of the anterior cruciate ligament although they can be used for filling any ligament or tendon defect. The principal difficulties of the cortical allografts are in the consolidation of the ends with the bone itself and in tumour surgery, given that these are patients immunodepressed by the treatment, the incidence of infection is increased with respect to spongy grafts and soft tissues, which is irrelevant. In short, the increasingly widespread use of allografts is an essential therapeutic weapon in orthopaedic surgery and traumatology. It must be used by expert hands

Frozen cancellous bone allografts: positive cultures of implanted grafts in posterior fusions of the spine.

We have carried out a study on the behaviour pattern of implanted allografts initially stored in perfect conditions (aseptically processed, culture-negative and stored at -80 degrees C) but which presented positive cultures at the implantation stage. There is no information available on how to deal with this type of situation, so our aim was to set guidelines on the course of action which would be required in such a case. This was a retrospective study of 112 patients who underwent a spinal arthrodesis and in whom a total of 189 allograft pieces were used. All previous bone and blood cultures and tests for hepatitis B and C, syphilis and HIV (via PCR techniques) were negative. The allografts were stored by freezing them at -80 degrees C. A sample of the allograft was taken for culture in the operating theatre just before its implantation in all cases. The results of the cultures were obtained 3-5 days after the operation. There were 22 allografts with positive culture results (12%) after implantation. These allografts were implanted in 16 patients (14%). Cultures were positive for staphylococci coagulase negative (ECN) in 10 grafts (46%), Pseudomonas stutzeri in two grafts (9%), Corynebacterium jeikeium in two grafts (9%), staphylococci coagulase positive in two grafts (9%) and for each of the following organisms in one case each (4%): Corynebacterium spp., Actinomyces odontolyticus, Streptococcus mitis, Peptostreptococcus spp., Rhodococcus equi and Bacillus spp. No clinical infection was seen in any of these patients. Positive cultures could be caused by non-detected contamination at harvesting, storing or during manipulation before implantation. The lack of clinical signs of infection during the follow-up of our patients may indicate that no specific treatment different from our antibiotic protocol is required in the case of positive culture results of a graft piece after implantation.

Bone metastases from osteosarcoma.

Bony metastases in patients with osteosarcoma are unusual and normally appear late in the course of the disease. We report our experience with eight such patients, four with solitary and four with multiple metastases. Those with solitary metastases were treated as new tumours with neoadjuvant chemotherapy and surgery. Three remain alive with no evidence of disease at 5, 7 and 8 years follow-up respectively. Histology and response to neoadjuvant chemotherapy was similar in both the primary and metastatic lesions and is a predictive factor of outcome. Those with multiple metastases were treated by palliative measures, and none survived. We conclude that resection of solitary metastases from osteosarcoma after neoadjuvant chemotherapy can be curative.

Osteosarcoma over the age of forty.

The European Musculo Skeletal Oncology Society (EMSOS) has carried out a retrospective review of patients over the age of 40 years with osteosarcoma. 481 patients from 12 centres or multicentric groups were included. 42 patients had osteosarcoma arising in Paget's disease, median survival was 9 months. Patients with axial or metastatic tumours also did badly whilst 41 patients with radiation-induced osteosarcoma had a prognosis paralleling conventional osteosarcoma matched for patient age and site of the tumour. 238 patients had high grade non-metastatic osteosarcoma and had a survival of 46% at 5 years. Older patients had less chemotherapy and fared worse. Osteosarcoma in the elderly is a curable condition and warrants intensive treatment with chemotherapy and surgical resection.

Atypical evolution of a calcified lipid cyst presenting spontaneously as a suspicious cluster of microcalcifications.

We report an infrequent mammographic case in which a gross-calcified lipid cyst evolved spontaneously to form a cluster of pleomorphic microcalcifications which were indistinguishable from those seen in breast cancer. The biopsy performed was consistent with fat necrosis. Ultrasonography of the specimen showed a nodule with mixed echogenicity surrounded by a hypoechoic rim. To our knowledge, this behavior is extremely rare, with only one similar case reported in the literature.