Resultados oncológicos y factores pronósticos en pacientes con sarcoma sinovial tratados quirúrgicamente / Oncological outcomes and prognostic factors in surgically treated patients with synovial sarcoma

Resumen Introducción : El sarcoma sinovial es un tumor raro (incidencia de 1-3 casos por millón). Es más frecuente en adolescentes y adultos menores de 30 años. Se desarrolla en cualquier parte del cuerpo, siendo, las extremidades el lugar más frecuente de aparición (80% extremidades y 20% otras localizaciones: 8% tronco, retroperitoneal/ abdominal 7%, cabeza y cuello 5%). Los resultados on cológicos de los pacientes con sarcoma sinovial son disímiles. La tasa de supervivencia libre de recurrencia local, la supervivencia libre de eventos y la superviven cia global varían entre las series publicadas. Lo mismo sucede con los factores pronósticos de la enfermedad. Métodos : El objetivo fue analizar un grupo de 43 pacientes con diagnóstico de sarcoma sinovial de las extremidades tratados quirúrgicamente, y determinar (1) tasa de supervivencia global, (2) tasa de superviven cia libre de eventos, (3) tasa de recurrencia local y (4) factores de riesgo oncológicos. Resultados : La supervivencia global a los 2 años fue 90% (IC95%: 76-96%), y 67% (IC95%: 49-80%) a los 5 años. La supervivencia libre de eventos a los 2 años fue 68% (IC95% 51-80%) y a los 5 años 48% (IC95% 32-52%). El riesgo de recurrencia local a 2 años fue 9% (IC95% 3-25%) y a los 5 años 25% (IC95% 13-46%). Los factores de mal pronóstico oncológico fueron el grado histológico y la presencia de metástasis. Discusión : Podemos concluir que nuestros resulta dos oncológicos se asemejan a las series publicadas y que en nuestra serie hubo dos factores de mal pro nóstico.

Abstract Introduction : Synovial sarcoma is an unusual tumor with an incidence of 1-3 cases per million. It is more frequent in teenagers and young adults under 30. It develops anywhere, but the extremities are the most frequent place of appearance (80% extremities, 20% other locations: 8% trunk, 7% retroperitoneal/abdominal, 5% head and neck). Oncological results are different depending on the study. Survival rate free of local recur rence, survival rate free of events and global survival rate vary upon published studies. The same happens with the disease's prognostic factors. Methods : The objective was to analyze a group of 43 patients with diagnosis of synovial sarcoma of the extremities treated surgically and determine (1) global survival rate, (2) survival rate free of events, (3) local recurrence rate and (4) oncological risk factors. Results : The global survival rate at 2 years was 90% (IC95%: 76 - 96%) and 67% (IC95%: 49-80%) at 5 years. The survival rate free of events at 2 years was 68% (IC95% 51-80%) and 48% (IC95% 32-52%) at 5 years. The recurrence rate at 2 years was 9% (IC95% 3-25%) and 25% (IC95% 13-46%) at 5 years. The histological grade and metastatic presence were bad prognostic factors. Discussion : We can conclude that our oncological results are in line with those published in previous series and that there were two factors associated with poor prognosis.

[Oncological outcomes and prognostic factors in surgically treated patients with synovial sarcoma]. / Resultados oncológicos y factores pronósticos en pacientes con sarcoma sinovial tratados quirúrgicamente.

INTRODUCTION: Synovial sarcoma is an unusual tumor with an incidence of 1-3 cases per million. It is more frequent in teenagers and young adults under 30. It develops anywhere, but the extremities are the most frequent place of appearance (80% extremities, 20% other locations: 8% trunk, 7% retroperitoneal/abdominal, 5% head and neck). Oncological results are different depending on the study. Survival rate free of local recurrence, survival rate free of events and global survival rate vary upon published studies. The same happens with the disease's prognostic factors. METHODS: The objective was to analyze a group of 43 patients with diagnosis of synovial sarcoma of the extremities treated surgically and determine (1) global survival rate, (2) survival rate free of events, (3) local recurrence rate and (4) oncological risk factors. RESULTS: The global survival rate at 2 years was 90% (IC95%: 76 - 96%) and 67% (IC95%: 49-80%) at 5 years. The survival rate free of events at 2 years was 68% (IC95% 51-80%) and 48% (IC95% 32-52%) at 5 years. The recurrence rate at 2 years was 9% (IC95% 3-25%) and 25% (IC95% 13-46%) at 5 years. The histological grade and metastatic presence were bad prognostic factors. DISCUSSION: We can conclude that our oncological results are in line with those published in previous series and that there were two factors associated with poor prognosis.

Introducción: El sarcoma sinovial es un tumor raro (incidencia de 1-3 casos por millón). Es más frecuente en adolescentes y adultos menores de 30 años. Se desarrolla en cualquier parte del cuerpo, siendo, las extremidades el lugar más frecuente de aparición (80% extremidades y 20% otras localizaciones: 8% tronco, retroperitoneal/ abdominal 7%, cabeza y cuello 5%). Los resultados oncológicos de los pacientes con sarcoma sinovial son disímiles. La tasa de supervivencia libre de recurrencia local, la supervivencia libre de eventos y la supervivencia global varían entre las series publicadas. Lo mismo sucede con los factores pronósticos de la enfermedad. Métodos: El objetivo fue analizar un grupo de 43 pacientes con diagnóstico de sarcoma sinovial de las extremidades tratados quirúrgicamente, y determinar (1) tasa de supervivencia global, (2) tasa de supervivencia libre de eventos, (3) tasa de recurrencia local y (4) factores de riesgo oncológicos. Resultados: La supervivencia global a los 2 años fue 90% (IC95%: 76-96%), y 67% (IC95%: 49-80%) a los 5 años. La supervivencia libre de eventos a los 2 años fue 68% (IC95% 51-80%) y a los 5 años 48% (IC95% 32-52%). El riesgo de recurrencia local a 2 años fue 9% (IC95% 3-25%) y a los 5 años 25% (IC95% 13-46%). Los factores de mal pronóstico oncológico fueron el grado histológico y la presencia de metástasis. Discusión: Podemos concluir que nuestros resultados oncológicos se asemejan a las series publicadas y que en nuestra serie hubo dos factores de mal pronóstico.

Chondrosarcoma of the Proximal Humerus: Does the Margin Affect Survival?

Chondrosarcoma (CS) is the second most common primary malignant bone tumour and, in the absence of reliable chemotherapy and radiotherapy, is effectively a surgical disease. Overall disease specific survival (DSS) is affected by tumour grade, whilst resection margin contributes to local recurrence free survival (LRFS). The aim of this study was to investigate factors that affect the local and systemic prognoses for conventional central CSs arising from the proximal humerus. A multi-centre, retrospective study from three international collaborative sarcoma centres identified 110 patients between 1995 and 2020 undergoing treatment for a conventional central CS of the proximal humerus; 58 patients (53%) had a grade 1 tumour, 36 (33%) had a grade 2 tumour, and 16 patients (13%) had a grade 3 CS. The mean age of patients was 50 years (range 10-85). The incidence of local recurrence (LR) was 9/110 (8.2%), and the disease specific mortality was 6/110 (5.5%). The grade was a statistically significant factor for LRFS (p < 0.001). None of the grade 1 tumours developed LR. The DSS was affected by the grade (p < 0.001) but not by the LR (p = 0.4). Only one patient with a grade 2 tumour died from the disease. The proximal humeral grade 1 CS behaved as a benign tumour, having no cases of LR nor death due to disease. Grade 2 CSs of the proximal humerus behaved in a more indolent way when compared with comparable grade tumours elsewhere in the appendicular skeleton, being locally aggressive with a higher LR rate than grade 1 CSs but still having very low mortality and a high rate of DSS. The LR in grade 2 CSs did not affect the DSS; therefore, surgical management in proximal humeral grade 2 CSs should have a greater emphasis on preserving function whilst maintaining an adequate margin for resection. The proximal humeral grade 3 CS was, as elsewhere in the skeleton, an aggressive, high-grade tumour. Therefore, surgical management should include en bloc resection with clear margins to avoid LR.

Complete tumor necrosis after neoadjuvant chemotherapy defines good responders in patients with Ewing sarcoma.

BACKGROUND: Survival in patients who have Ewing sarcoma is correlated with postchemotherapy response (tumor necrosis). This treatment response has been categorized as the response rate, similar to what has been used in osteosarcoma. There is controversy regarding whether this is appropriate or whether it should be a dichotomy of complete versus incomplete response, given how important a complete response is for in overall survival of patients with Ewing sarcoma. The purpose of this study was to evaluate the impact that the amount of chemotherapy-induced necrosis has on (1) overall survival, (2) local recurrence-free survival, (3) metastasis-free survival, and (4) event-free survival in patients with Ewing sarcoma. METHODS: In total, 427 patients who had Ewing sarcoma or tumors in the Ewing sarcoma family and received treatment with preoperative chemotherapy and surgery at 10 international institutions were included. Multivariate Cox proportional-hazards analyses were used to assess the associations between tumor necrosis and all four outcomes while controlling for clinical factors identified in bivariate analysis, including age, tumor volume, location, surgical margins, metastatic disease at presentation, and preoperative radiotherapy. RESULTS: Patients who had a complete (100%) tumor response to chemotherapy had increased overall survival (hazard ratio [HR], 0.26; 95% CI, 0.14-0.48; p < .01), recurrence-free survival (HR, 0.40; 95% CI, 0.20-0.82; p = .01), metastasis-free survival (HR, 0.27; 95% CI, 0.15-0.46; p ≤ .01), and event-free survival (HR, 0.26; 95% CI, 0.16-0.41; p ≤ .01) compared with patients who had a partial (0%-99%) response. CONCLUSIONS: Complete tumor necrosis should be the index parameter to grade response to treatment as satisfactory in patients with Ewing sarcoma. Any viable tumor in these patients after neoadjuvant treatment should be of oncologic concern. These findings can affect the design of new clinical trials and the risk-stratified application of conventional or novel treatments.

Comportamiento del condrosarcoma óseo en pacientes menores de 40 años / Chondrosarcoma of bone in young patients

Resumen El condrosarcoma ocupa el segundo lugar en frecuencia, entre los tumores malignos primarios óseos, en pacientes adultos. Típicamente afecta a adultos de entre 40 y 70 años, siendo la mayoría de las series publicadas de este grupo etario. El objetivo de este estudio fue comunicar una serie de pacientes adultos jóvenes (16 a 40 años) con diagnóstico de condrosarcoma óseo, que buscó describir las particularida des en la forma de presentación en esta población. De un total de 37 pacientes de 16 a 40 años operados por condrosarcoma en nuestro centro, entre los años 2008 y 2019, 18 presentaron tumores de bajo grado (G1/tumor cartilaginoso atípico), 16 de grado intermedio (G2), quedando una minoría con tumores de alto grado (2 con condrosarcoma G3 y 1 con un condrosarcoma desdiferenciado). La tasa de supervivencia global fue del 97% (IC 95% 82%-99%) a 2 años y 90.5% (IC 95% 73%-96%) a 5 años y la tasa libre de recurrencia local fue de 92% (IC 95% 77%-97.5%) a los 12 meses y de 89% a los 2 y 5 años (IC 95% 73%-96%). El grado histológico fue el factor pronóstico más relevante. La concordancia del grado histológico entre el diagnóstico preoperatorio de la biopsia y el material de resección quirúrgica fue del 81% (30/37).

Abstract Chondrosarcoma is the second most frequent primary malignant bone tumor. It is typically described in adults between 40 and 70 years of age, being the majority of the series published in this age group. The objective of this study was to report a series of young adults (16-40 years old) with a diagnosis of chondrosarcoma of bone in order to describe the particularities of this tumor in this population. From a total of 37 patients between 16 and 40 years old, surgically treated for chondrosar coma in our institution, 18 had low-grade tumors (G1/atypical cartilaginous tumor), 16 had an intermediate-grade (G2), and a minority had high-grade tumors (2 with a G3 and 1 with a dedifferentiated chondrosarcoma). The overall survival rate was 97% (95% CI 82%-99%) at 2 years of follow-up and 90.5% (95% CI 73-96) at 5 years of follow-up. The local recurrence free rate was 92% (95% CI % 77%-97.5%) at 1 year of follow-up and 89% at 2 and 5 years of follow-up (95% CI 73%-96%). Histological grade was the most relevant prognostic factor. The concordance of the histological grade between the preoperative diagnosis of the biopsy and the surgical resection material was 81% (30/37).

[Chondrosarcoma of bone in young patients]. / Comportamiento del condrosarcoma óseo en pacientes menores de 40 años.

Chondrosarcoma is the second most frequent primary malignant bone tumor. It is typically described in adults between 40 and 70 years of age, being the majority of the series published in this age group. The objective of this study was to report a series of young adults (16-40 years old) with a diagnosis of chondrosarcoma of bone in order to describe the particularities of this tumor in this population. From a total of 37 patients between 16 and 40 years old, surgically treated for chondrosarcoma in our institution, 18 had low-grade tumors (G1/atypical cartilaginous tumor), 16 had an intermediate-grade (G2), and a minority had high-grade tumors (2 with a G3 and 1 with a dedifferentiated chondrosarcoma). The overall survival rate was 97% (95% CI 82%-99%) at 2 years of follow-up and 90.5% (95% CI 73-96) at 5 years of follow-up. The local recurrence free rate was 92% (95% CI % 77%-97.5%) at 1 year of follow-up and 89% at 2 and 5 years of follow-up (95% CI 73%-96%). Histological grade was the most relevant prognostic factor. The concordance of the histological grade between the preoperative diagnosis of the biopsy and the surgical resection material was 81% (30/37).

El condrosarcoma ocupa el segundo lugar en frecuencia, entre los tumores malignos primarios óseos, en pacientes adultos. Típicamente afecta a adultos de entre 40 y 70 años, siendo la mayoría de las series publicadas de este grupo etario. El objetivo de este estudio fue comunicar una serie de pacientes adultos jóvenes (16 a 40 años) con diagnóstico de condrosarcoma óseo, que buscó describir las particularidades en la forma de presentación en esta población. De un total de 37 pacientes de 16 a 40 años operados por condrosarcoma en nuestro centro, entre los años 2008 y 2019, 18 presentaron tumores de bajo grado (G1/tumor cartilaginoso atípico), 16 de grado intermedio (G2), quedando una minoría con tumores de alto grado (2 con condrosarcoma G3 y 1 con un condrosarcoma desdiferenciado). La tasa de supervivencia global fue del 97% (IC 95% 82%-99%) a 2 años y 90.5% (IC 95% 73%-96%) a 5 años y la tasa libre de recurrencia local fue de 92% (IC 95% 77%-97.5%) a los 12 meses y de 89% a los 2 y 5 años (IC 95% 73%-96%). El grado histológico fue el factor pronóstico más relevante. La concordancia del grado histológico entre el diagnóstico preoperatorio de la biopsia y el material de resección quirúrgica fue del 81% (30/37).

Navigated multiplanar osteotomies for spinal primary bone tumors.

BACKGROUND: Establishing the proper diagnosis and rendering appropriate treatment of spinal primary bone tumors (SPBT) can result in definitive cures. Notably, malignant, or benign SPBT (i.e., with aggressive local behavior) generally require en bloc resection. Osteotomies of the vertebral body in more than 1 plane may avoid critical structures, preserve nerve functions, and reduce the volume of healthy bone resected. Here, our objective was to report how we planned and performed navigated multiplanar osteotomies for en bloc resection of 14 SPBT. METHODS: We performed a retrospective analysis of 14 patients with malignant or locally aggressive benign SPBT operated on consecutively between 2014 and 2019 utilizing preoperative 3D planning/navigation. Tumors were resected in an en bloc fashion utilizing multiplanar osteotomies. Patients were followed for a minimum of 12 postoperative months. RESULTS: Diagnoses included three benign but locally aggressive bone tumors (i.e., all osteoblastomas) and 11 primary sarcomas (i.e., six chordomas and five chondrosarcomas). Eleven tumors involved the sacrum and the other three, the thoracic spine. In 12 patients, the en bloc margins were classified as marginal (<1 cm), and in two patients, as wide (>1 cm). Intraoperative navigation facilitated the performance of 40 osteotomies in 14 patients (median = 2.9, range = 2-6). CONCLUSION: Navigated multiplanar osteotomies increased the precision and safety of en bloc resections for 14 primary spinal bone tumors SPBT that included 11 malignant and three benign/locally aggressive lesions.

[Denosumab as a treatment for giant cell tumor of bone. Indications, results and side effects]. / Denosumab como tratamiento neoadyuvante del tumor de células gigantes del hueso. Indicaciones, resultados y efectos adversos.

Giant cell tumor of bone is an intermediate, locally aggressive and rarely metastasizing, primary bone neoplasia. In recent years denosumab emerged as a treatment alternative for this pathology. The objective of this work was to analyze its indications as well as the clinical outcomes, side effects and local recurrence rates in patients diagnosed with giant cell tumor of bone, who received denosumab as neoadjuvant treatment. Between 2010 and 2018, 80 patients with giant cell tumor were analyzed, of whom 14 received denosumab as a neoadjuvant treatment. The minimum follow-up was 12 months. In 8 patients it was a primary tumor, while 6 showed tumor recurrence. In all cases, clinical improvement was evident. Thirteen patients presented radiographic changes, and 11 showed complete histological response. A local recurrence was evidenced in 6 of 14 patients, and at least one adverse effect related to denosumab (including tumor malignancy) was identified in 7. Despite being a useful tool for treating giant cell tumor, the use of denosumab is associated with a higher rate of local recurrences and is not free of adverse effects.

El tumor de células gigantes óseo es una neoplasia de agresividad local intermedia, que raramente metastatiza. En los últimos años el denosumab, anticuerpo monoclonal humano, surgió como una alternativa de tratamiento para esta enfermedad, al bloquear el comportamiento lítico tumoral. El objetivo de este trabajo fue determinar sus indicaciones y efectos adversos, analizando también los resultados oncológicos, y las tasas de recurrencia local en pacientes con diagnóstico de tumor de células gigantes óseo que recibieron denosumab como tratamiento neoadyuvante. Entre 2010 y 2018 se analizaron 80 pacientes con tumor de células gigantes, de los cuales 14 recibieron denosumab como tratamiento neoadyuvante. El seguimiento mínimo fue 12 meses. En 8 pacientes se trató de un tumor primario, mientras que 6 fueron pacientes con recidiva tumoral. En todos los casos se evidenció una mejoría clínica. Trece presentaron cambios radiográficos, y 11 respuesta histológica completa. En 6 de 14 pacientes se evidenció una recurrencia local y en 7 se identificó al menos un efecto adverso relacionado con el denosumab (incluyendo una malignización tumoral). A pesar de ser una herramienta útil para el tratamiento del tumor de células gigantes, el uso de denosumab está asociado a mayor tasa de recurrencias locales y no está exento de efectos adversos.

Denosumab como tratamiento neoadyuvante del tumor de células gigantes del hueso. Indicaciones, resultados y efectos adversos / Denosumab as a neoadjuvant treatment for giant cell tumor of bone. Indications, results and side effects

Resumen El tumor de células gigantes óseo es una neoplasia de agresividad local intermedia, que raramente metastatiza. En los últimos años el denosumab, anticuerpo monoclonal humano, surgió como una alternativa de tratamiento para esta enfermedad, al bloquear el comportamiento lítico tumoral. El objetivo de este trabajo fue determinar sus indicaciones y efectos adversos, analizando también los resultados oncológicos, y las tasas de recurrencia local en pacientes con diagnóstico de tumor de células gigantes óseo que recibieron denosumab como tratamiento neoadyuvante. Entre 2010 y 2018 se analizaron 80 pacientes con tumor de células gigantes, de los cuales 14 recibieron denosumab como tratamiento neoadyuvante. El seguimiento mínimo fue 12 meses. En 8 pacientes se trató de un tumor primario, mientras que 6 fueron pacientes con recidiva tumoral. En todos los casos se evidenció una mejoría clínica. Trece presentaron cambios radiográficos, y 11 respuesta histológica completa. En 6 de 14 pacientes se evidenció una recurrencia local y en 7 se identificó al menos un efecto adverso relacionado con el denosumab (incluyendo una malignización tumoral). A pesar de ser una herramienta útil para el tratamiento del tumor de células gigantes, el uso de denosumab está asociado a mayor tasa de recurrencias locales y no está exento de efectos adversos.

Abstract Giant cell tumor of bone is an intermediate, locally aggressive and rarely metastasiz ing, primary bone neoplasia. In recent years denosumab emerged as a treatment alternative for this pathology. The objective of this work was to analyze its indications as well as the clinical outcomes, side effects and local recurrence rates in patients diagnosed with giant cell tumor of bone, who received denosumab as neoadjuvant treatment. Between 2010 and 2018, 80 patients with giant cell tumor were analyzed, of whom 14 received deno sumab as a neoadjuvant treatment. The minimum follow-up was 12 months. In 8 patients it was a primary tumor, while 6 showed tumor recurrence. In all cases, clinical improvement was evident. Thirteen patients presented radiographic changes, and 11 showed complete histological response. A local recurrence was evidenced in 6 of 14 patients, and at least one adverse effect related to denosumab (including tumor malignancy) was identified in 7. Despite being a useful tool for treating giant cell tumor, the use of denosumab is associated with a higher rate of local recurrences and is not free of adverse effects.

Biology and technology in the surgical treatment of malignant bone tumours in children and adolescents, with a special note on the very young.

PURPOSE: The main challenge in reconstruction after malignant bone tumour resection in young children remains how and when growth-plates can be preserved and which options remain if impossible. METHODS: We describe different strategies to assure best possible long-term function for young children undergoing resection of malignant bone tumours. RESULTS: Different resources are available to treat children with malignant bones tumours: a) preoperative planning simulates scenarios for tumour resection and limb reconstruction, facilitating decision-making for surgical and reconstructive techniques in individual patients; b) allograft reconstruction offers bone-stock preservation for future needs. Most allografts are intact at long-term follow-up, but limb-length inequalities and corrective/revision surgery are common in young patients; c) free vascularized fibula can be used as stand-alone reconstruction, vascularized augmentation of structural allograft or devitalized autograft. Longitudinal growth and joint remodelling potential can be preserved, if transferred with vascularized proximal physis; d) epiphysiolysis before resection with continuous physeal distraction provides safe resection margins and maintains growth-plate and epiphysis; e) 3D printing may facilitate joint salvage by reconstruction with patient-specific instruments. Very short stems can be created for fixation in (epi-)metaphysis, preserving native joints; f) growing endoprosthesis can provide for remaining growth after resection of epi-metaphyseal tumours. At ten-year follow-up, limb survival was 89%, but multiple surgeries are often required; g) rotationplasty and amputation should be considered if limb salvage is impossible and/or would result in decreased function and quality of life. CONCLUSION: Several biological and technological reconstruction options must be merged and used to yield best outcomes when treating young children with malignant bone tumours. LEVEL OF EVIDENCE: Level V Expert opinion.

Computer-assisted surgery (CAS) in orthopedic oncology. Which were the indications, problems and results in our first consecutive 203 patients?

AIMS: to review a group of patients with primary bone tumors treated with intraoperative navigation and analyze: (1) The technical problems; (2) Indications for Computer Assisted Surgery (CAS); (3) Oncological results; (4) Non oncological complications. MATERIALS AND METHODS: All patients from a single institution who had preoperative virtual planned for an oncological primary bone resection assisted with navigation between May 2010 and July 2017 were enrolled in the study (203 patients). The use of computer-assisted surgery (CAS) was classified according to the oncologic procedure performed: (1) intralesional resections, (2) en-block resections, and (3) en-block resections + navigated allograft reconstructions. RESULTS: Four patients (4/203, 2%) of the series presented technical problems which came from 2 software and 2 hardware crashes. Eight (4%) procedures were intralesional resections and no local recurrences or complications were reported in this group. Ninety-eight surgeries (49%) were pure en block resection. The pelvis and sacrum were the main location in this group (57%). All bone margins were defined negative but 2 patients presented a positive resection in the soft tissues. Infection was the most prevalent complication (16/23). Ninety-three procedures were done for en block resections + allograft reconstruction (all extremities tumor). All margins were free of tumor and non oncological rate for this group was 28%. CONCLUSION: The main indications for CAS were malignant bone tumors resection. The technical failures precluded navigation use in 2%. CAS for pure en-block resections were mainly indicated in pelvic and sacrum tumors while en-block resection + allograft reconstruction assisted with navigation were only indicated in extremities tumors. LEVEL OF EVIDENCE: IV.

Pediatric Chondroblastoma and the Need for Lung Staging at Presentation.

PURPOSE: Chondroblastoma is a benign, but potentially locally aggressive, bone tumor with predilection for the epiphysis of long bones in growing children. Historically, there is a reported 2% risk of lung metastasis, however these cases are mostly in the form of isolated single reports and the vast majority in adults. The purpose of this study was to identify the "true" risk of lung metastases at presentation in skeletally immature patients with a benign chondroblastoma, and therefore revisit the need for routine chest staging. METHODS: This was a multi-institution, international retrospective study of children and adolescents diagnosed and treated for a benign chondroblastoma. We focused on the screening and diagnosis of lung metastasis, type of staging utilized and the incidence of local recurrence. Detailed review of the available literature was also performed for comparison. RESULTS: The final studied cohort included 130 children with an average age of 14.5 years (range: 6 to 18 y). There were 94 boys and 36 girls. Lesions more often involved the proximal humerus (32/130), proximal tibia (30/130), and proximal femur (28/130). At an average follow-up of 50 months, there were 15 local recurrences (11% rate) and no cases of lung metastasis. All patients underwent chest imaging at presentation. The overall reported lung metastases rate in the pulled literature review (larger series only) was 0.4% (7/1625), all patients were skeletally mature. CONCLUSIONS: This is the largest cohort of pediatric-exclusive chondroblastoma in the literature. Despite minor differences in management between the centers included, the recurrence rate was similar and there was no evidence of lung metastasis (0 in 130). The incidence of distant involvement in a true benign chondroblastoma in children is much lower than the 2% previously reported in the literature, and the need for routine chest staging should be revisited. LEVEL OF EVIDENCE: Level III.

Does Osteoarticular Allograft Reconstruction Achieve Long-term Survivorship after En Bloc Resection of Grade 3 Giant Cell Tumor of Bone?

BACKGROUND: En bloc resection of benign tumors is only indicated in aggressive lesions with substantial destruction of the affected bone. Few reports have evaluated the long-term outcome of Grade 3 giant cell tumor of bone (GCTB; defined as severe bone destruction and soft tissue extension) treated with en bloc resection and reconstruction with a massive allograft. We recently reported that patients with benign tumors achieved better allograft reconstruction survivorship compared with those treated for a malignant bone tumor. In light of that finding, we wondered whether osteoarticular allografts would be a viable long-term alternative for Grade 3 GCTB, which could be important in some countries because of greater availability and lower costs compared with endoprostheses. QUESTIONS/PURPOSES: We analyzed a group of patients with Grade 3 GCTBs treated with en bloc resection and osteoarticular allograft reconstruction in terms of (1) survivorship free from allograft removal at 10 years; (2) survivorship free from reoperation for any reason at 10 years, (3) functional results as measured by the Musculoskeletal Tumor Society (MSTS) score, (4) assessment of arthrosis at the knee. METHODS: We retrospectively analyzed all patients with a Grade 3 GCTB treated between 1980 and 2007. Only patients treated with en bloc resection and reconstruction with massive osteoarticular allografts were included in the analysis. The indication for osteoarticular reconstruction during that time included severe bone destruction with intraarticular compromise of the tumor, intraarticular fracture because of tumor growth, the presence of inadequate remaining subchondral bone to resist normal loading (for the distal femur or proximal tibia), and the preservation of a soft-tissue component (ligaments or meniscus) for articular stability. During the period, 75 patients were treated with en bloc resection. Patients treated with intralesional curettage (n = 7), reconstruction with an endoprosthesis (n = 2), intercalary arthrodesis (n = 13), or unicondylar reconstruction (n = 14) were excluded. Of the original 75 treated with en bloc resection, 52% (39) were treated with osteoarticular allograft reconstruction, and no patient was lost to follow-up before 2 years or had substantial missing data. However, of the 39 patients, another 21% (8) have not been seen in the last 5 years, but these were included here because they reached the 10-year minimum surveillance period before being lost. Twenty-three of those 39 patients were previously reported by our group and 16 new patients (treated between 1980-1985) were included in this series (eight distal radius, six distal femur, two proximal tibias), extending the follow-up period and including more patients for analysis. The median (range) follow-up duration was 26 years (10 to 34). We assessed survivorship using a Kaplan-Meier analysis, we drew MSTS scores retrospectively from patients´ medical records, and we graded arthrosis using the Ahlbäck scale for the knee (which was by far the most common joint involved, n = 31, and so it was the joint we assessed for the presence of arthrosis). RESULTS: The survivorship free from allograft removal was 85% at 10 years (95% CI 74 to 96). The allograft survivorship free from reoperation for any reason at 10 years was 72% (95% CI 59 to 87). The median (range) MSTS score was 28 points (19 to 30). The grade of arthrosis in the knee at last follow-up was analyzed in 20 patients and classified in nine as Ahlbäck Type 4, in six as Type 3, in three as Type 2 and in two as Type 5. CONCLUSIONS: Osteoarticular allograft reconstruction after a Grade 3 GCTB en bloc resection showed excellent long-term survivorship. We believe these results compare favorably with other studies on endoprosthetic reconstruction and head-to-head studies of these approaches should be performed; these would need to be multicenter trials. In the meantime, in locations where endoprostheses are unavailable or too expensive, we believe our results support the use of osteoarticular allografts. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Do Massive Allograft Reconstructions for Tumors of the Femur and Tibia Survive 10 or More Years after Implantation?

BACKGROUND: Massive bone allografts have been used for limb salvage in patients undergoing bone tumor resections as an alternative to endoprostheses. Although several studies on massive allograft reconstructions for bone tumors reported that most complications occur in the first 3 years after surgery, there are no long-term reports on complications to substantiate this contention. We believe such information is important so that surgeons and patients can make more informed decisions when choosing a reconstructive method after tumor resection. QUESTIONS/PURPOSES: (1) What is the survival of allografts free from removal, amputation, or joint replacement in patients treated for bone tumors in the lower limb with a minimum of 10 years of followup? (2) What complications occur after 10 or more years of followup? (3) Are there factors associated with allograft survival, such as age, sex, the affected bone, reconstruction type (intercalary or osteoarticular allograft), tumor type (malignant or benign), failure type, and chemotherapy use? METHODS: We retrospectively analyzed the records of 398 patients treated in one center with benign or malignant bone tumors in the femur or tibia between 1986 and 2007. During the period in question, our general indications for using allografts (354 patients) included patients with benign or low-grade sarcomas and patients with high-grade sarcomas with clinical and imaging response to neoadjuvant chemotherapy. Other approaches such as endoprostheses (44 patients) were indicated if the patient received radiotherapy, in patients with high-grade sarcomas without clinical and imaging response to neoadjuvant chemotherapy, or with neurovascular tumor involvement. We excluded from the analysis 53 patients treated with allograft-prosthetic composites, 46 with hemicondylar osteoarticular allografts, and 57 with intercalary hemicylindrical allografts. The study was thus performed in 198 patients treated with segmental massive allografts in the long bones of the lower extremity (132 femurs and 66 tibias) after resection of a primary bone tumor, including 120 patients treated with osteoarticular and 78 with segmental intercalary allografts. A total of 32 (16%) of the 198 patients died before 10 years, and graft status was known in all of those patients; these patients were included (mean followup, 192 months; range, 1-370 months). All remaining 166 patients who were not known to have died before 10 years were accounted for at least 10 years after the allograft procedure (mean, 222 months; range, 120-370 months). No patient was lost to followup. The mean age was 22 years (range, 2-55 years); 105 patients were male (53%) and 93 were female. The predominant diagnoses were osteosarcoma (n = 125, 63%), giant cell tumor of bone (n = 27, 14%), and Ewing's sarcoma (n = 19, 10%). In all, 146 patients (74%) underwent chemotherapy. Selected variables were analyzed using multivariate logistic regression analyses to identify risk factors of allograft removal, joint replacement, or amputation. We performed competitive risk analysis with allograft removal, joint replacement, or amputation as the endpoint at 5, 10, and 20 years. Patient function was evaluated using the Musculoskeletal Tumor Society (MSTS)-93 scoring system. RESULTS: The risk of allograft removal, joint replacement, or amputation was 36% at 5 years (95% CI, 30-43), 40% at 10 years (95% CI, 33-47), and 44% at 20 years (95% CI, 37-51). Fractures occurred in 15% (29 patients), infection in 14% (27 patients), nonunion in 12% (23 patients) and tumor recurrence in 7% (13 patients). Thirty-two patients died of disease before 10 years; nine of these patients had a second surgery before death, eight had an amputation, and one underwent graft removal. Of the 166 patients who were still alive 10 years after the allograft procedure, 36 underwent allograft removal, six patients underwent joint replacement, and four had an amputation; however, after 10 years, six more allografts were removed (four due to fractures, one due to infection, and one due to instability), and another patient was amputated due to a second malignancy. After controlling for potentially confounding variables including death, we found that the risk of allograft removal, joint replacement, or amputation in osteoarticular tibial grafts (58% [95% CI, 43-73] at 5, 10, and 20 years) was higher than that of osteoarticular femur allografts (29% [95% CI, 18-39] at 5 years, 30% [95% CI, 19-40] at 10 years, 37% [95% CI, 25-48] at 20 years; p = 0.010) and tibia intercalary allografts (26% [95% CI, 7-45] at 5, 10 and 20 years; p = 0.020). Fractures occurred more frequently in the femur (18% [95% CI, 11-25]) than in the tibia (5% [95% CI, 0-10]; p < 0.010), and infections occurred more frequently in the tibia (24% [95% CI, 14-35]) than in the femur (4% [95% CI, 0-8]; p < 0.001). With the number of patients we had, we found no difference in the proportion of local recurrence in the tibia (12% [95% CI, 4-20]) compared with the femur (5% [95% CI, 1-9]; p < 0.053). CONCLUSIONS: Infections were the most common complications associated with allograft removal in the first 2 to 3 years after reconstruction and were more frequently associated with tibial allograft removal. Fractures were more commonly associated with graft removal with longer term followup and were more frequently associated with femoral allograft removal. Although we cannot directly compare our results with other types of reconstructions, we believe that allografts still have a role in the reconstruction of patients with a benign or low-grade bone tumor. Future studies in femoral allograft with longer followup should be performed to analyze factors that may explain why some grafts fail, such as the percent of the length of the bone resected, type and number of plates and screws used and type of fixation (rods versus plates). There was a higher incidence of graft removal in patients with proximal tibia osteoarticular allografts, which has led us to use this type of reconstruction only in pediatric patients over the last 15 years. LEVEL OF EVIDENCE: Level III, therapeutic study.

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

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.

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.

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.

Face to Face Appointment vs. Telemedicine in First Time Appointment Orthopedic Oncology Patients: A Cost Analysis.

Medicine has evolved considerably in recent decades in part thanks to information and communication technologies in health (ICTs). However, face-to-face consultations continue to be the predominant model, since alternatives such as telemedicine are still the subject of debate. On the other hand, in some very specific specialties, centralization is relevant, mainly due to the low frequency and prevalence of diseases, as well as the need to have highly specialized professionals, causing problems in terms of accessibility and costs for the health system. In this study we have analyzed the first consultations to an orthopedics oncology service at a tertiary institution and performed an analysis of economic costs was carried out between 2 possible scenarios: face-to-face consultations versus telemedicine. Analyzing the 2 scenarios, there would be a cost-benefit in the use of telemedicine leading to a decrease in healthcare cost between 12.2% and 72%.

Aggressive granulomatosis of the hip: a forgotten mode of aseptic failure.

PURPOSE: It has been acknowledged that implant wear correlates with the risk for periprosthetic osteolysis, being aggressive granulomatosis the worst expression of bone resorption. We sought to determine the clinical, radiological, and histological features of aggressive granulomatosis after primary total hip arthroplasty (THA). METHODS: We included nine cases with aggressive granulomatosis of the hip around cemented stems. Indications for revision THA consisted of progressive signs of extensive bone resorption or implant loosening. Mean follow-up since revision THA was 143 months (SD ± 59.4). We analysed clinical outcomes, component loosening and gross as well as histological characteristics of the granulomatous lesions. RESULTS: Overall mean time between primary THA and revision surgery was 81 months (SD ± 20.8). All of the cases evidenced multiple ovoid tumour-like lesions around the stem with extensive bone loss. Only one case reported thigh pain before revision surgery, with radiological evidence of stem loosening; the remaining cases were asymptomatic with well-fixed implants. Gross anatomy findings revealed metallosis in the femoral canal and inside the cystic lesions. Pathology analysis showed monocyte-macrophage-dominated adverse foreign-body-type tissue reaction with fibroblastic reactive zones and granulomatous inflammation. CONCLUSIONS: We found a prevalence of 1% of this aseptic mode of implant failure. Since most of the retrieved stems were not loose, we did not find any alarming clinical symptoms anticipating implant failure. In this scenario, surgeons should be aware of the rapidly progressive nature of this entity and propose a revision THA in a timely fashion.

Is Navigation-guided En Bloc Resection Advantageous Compared With Intralesional Curettage for Locally Aggressive Bone Tumors?

BACKGROUND: The treatment of locally aggressive bone tumors is a balance between achieving local tumor control and surgical morbidity. Wide resection decreases the likelihood of local recurrence, although wide resection may result in more complications than would happen after curettage. Navigation-assisted surgery may allow more precise resection, perhaps making it possible to expand the procedure's indications and decrease the likelihood of recurrence; however, to our knowledge, comparative studies have not been performed. QUESTIONS/PURPOSES: The purpose of this study was to compare curettage plus phenol as a local adjuvant with navigation-guided en bloc resection in terms of (1) local recurrence; (2) nononcologic complications; and (3) function as measured by revised Musculoskeletal Tumor Society (MSTS) scores. METHODS: Patients with a metaphyseal and/or epiphyseal locally aggressive primary bone tumor treated by curettage and adjuvant therapy or en bloc resection assisted by navigation between 2010 and 2014 were considered for this retrospective study. Patients with a histologic diagnosis of a primary aggressive benign bone tumor or low-grade chondrosarcoma were included. During this time period, we treated 45 patients with curettage of whom 43 (95%) were available for followup at a minimum of 24 months (mean, 37 months; range, 24-61 months), and we treated 26 patients with navigation-guided en bloc resection, of whom all (100%) were available for study. During this period, we generally performed curettage with phenol when the lesion was in contact with subchondral bone. We treated tumors that were at least 5 mm from the subchondral bone, such that en bloc resection was considered possible with computer-assisted block resection. There were no differences in terms of age, gender, tumor type, or tumor location between the groups. Outcomes, including allograft healing, nonunion, tumor recurrence, fracture, hardware failure, infection, and revised MSTS score, were recorded. Bone consolidation was defined as complete periosteal and endosteal bridging visible between the allograft-host junctions in at least two different radiographic views and the absence of pain and instability in the union site. All study data were obtained from our longitudinally maintained oncology database. RESULTS: In the curettage group, two patients developed a local recurrence, and no local recurrences were recorded in patients treated with en bloc resection. All patients who underwent navigation-guided resection achieved tumor-free margins. Intraoperative navigation was performed successfully in all patients and there were no failures in registration. Postoperative complications did not differ between the groups: in patients undergoing curettage, 7% (three of 43) and in patients undergoing navigation, 4% (one of 26) had a complication. There was no difference in functional scores: mean MSTS score for patients undergoing curettage was 28 points (range, 27-30 points) and for patients undergoing navigation, 29 (range, 27-30 points; p = 0.10). CONCLUSIONS: In this small comparative series, navigation-assisted resection techniques allowed conservative en bloc resection of locally aggressive primary bone tumors with no local recurrence. Nevertheless, with the numbers available, we saw no difference between the groups in terms of local recurrence risk, complications, or function. Until or unless studies demonstrate an advantage to navigation-guided en bloc resection, we cannot recommend wide use of this novel technique because it adds surgical time and expense. LEVEL OF EVIDENCE: Level III, therapeutic study.