Reconstruction with Unconventional Endoprostheses after Resection of Primary Distal Femoral Bone Tumors: Implant Survival and Functional Outcomes / Reconstrução com endoprótese não convencional após ressecção de tumores ósseos primários de fêmur distal: sobrevida do implante e resultados funcionais

Abstract Objective To evaluate the survival time, the failure rate and its causes, and the functional results of cemented endoprostheses, with a polyethylene body, used after resection of primary bone tumors of the distal femur. Methods A retrospective study including 93 primary and 77 review procedures performed between 1987 and 2014. Survival was obtained by the Kaplan Meyer analysis, and the risk factors for implant failure were assessed through the Cox proportional risk model. The causes of endoprosthesis failure were classified according to Henderson et al. into five types: soft-tissue failure, aseptic loosening, structural fracture, infection, and tumor recurrence. The functional evaluation was performed using the functional classification system of the Musculoskeletal Tumor Society (MSTS) of bone sarcomas of the lower extremity, Brazilian version (MSTS-BR). Results Osteosarcoma was the most common diagnosis; 64.5% of the patients were younger than 20 years of age; the mean follow-up was of 124.3 months. The failure rate of the primary implant was of 54.8%, and the mean survival was of 123 months. The estimated survival of the primary implant was of 63.6%, 43.5%, 24.1%, and 14.5% in 5, 10, 15, and 20 years respectively. The most common cause of failure was type 2 (37.3%). Age ≤ 26 years and right side were risk factors for failure. The mean MSTS-BR score was of 20.7 (range: 14 to 27). Conclusion The results obtained for the failure rate and survival of the implant are in accordance with those of the literature, so the procedure herein studied is adequate and yields satisfactory functional results, even in the long term.

Resumo Objetivo Avaliar o tempo de sobrevida, a taxa de falha e suas causas, e os resultados funcionais de endopróteses cimentadas, com corpo em polietileno, empregadas após ressecção de tumores ósseos primários do fêmur distal. Métodos Estudo retrospectivo, que incluiu 93 procedimentos primários e 77 de revisão, realizados entre 1987 e 2014. A sobrevida foi obtida pela análise de Kaplan Meyer, e os fatores de risco para falha do implante foram avaliados por meio do modelo de riscos proporcionais de Cox. As causas de falha da endoprótese foram classificadas segundo Henderson et al. em cinco tipos: falha de partes moles, soltura asséptica, fratura estrutural, infecção e recorrência do tumor. A avaliação funcional foi realizada por meio do sistema de classificação funcional da Musculoskeletal Tumor Society (MSTS) para sarcomas ósseos da extremidade inferior, versão brasileira (MSTS-BR). Resultados Osteossarcoma foi o diagnóstico mais comum; 64,5% dos pacientes tinham menos de 20 anos; e o seguimento médio foi de 124,3 meses. A taxa de falha do implante primário foi de 54,8%, e a sobrevida média foi 123 meses. A estimativa de sobrevida do implante primário foi de 63,6%, 43,5%, 24,1%, 14,5% em 5, 10, 15 e 20 anos, respectivamente. A causa de falha mais comum foi a do tipo 2 (37,3%). Idade ≤ 26 anos e lado direito foram fatores de risco para falha. A pontuação média no MSTS-BR foi de 20,7 (variação: 14 a 27). Conclusão Os resultados obtidos para a taxa de falha e o tempo de sobrevida do implante estão de acordo com os da literatura, de forma que o procedimento estudado é adequado e apresenta resultados funcionais satisfatórios, inclusive em longo prazo.

Cirugías ablativas por causas oncológicas. 17 años de experiencia en una Unidad Especializada del Estado Monagas, Venezuela / Ablative surgeries for oncological causes. 17 years experience in a Monagas specialized unit

Estudio retrospectivo, sobre pacientes a quienes se les practicó cirugías ablativas por causas oncológicas: un total de 76 casos, predominando en la serie: el sexo masculino, la segunda década de la vida y como motivo de consulta: aumento de volumen con un 73,5%, el osteosarcoma fue la neoplasia más frecuente con un 34%, la fractura patológica acompañó a los tumores en el 50% de los casos, el hueso más afectado fue el fémur, los estadios según Enneking predominantes fueron los II b y III. Se practicaron más cirugías en miembros inferiores, siendo la amputación transfemoral la más frecuente. La intención de los procedimientos fue predominantemente curativa con un 69% sobre las intenciones paliativas, se practicaron más amputaciones en niveles oncológicos radicales que amplias. Se obtuvo una sobrevida global de 53,4% a los dos años de seguimiento, el miembro fantasma estuvo presente como complicación por encima del 77% de los casos operados(AU)

Retrospective study on patients who underwent ablative surgeries for oncological causes: a total of 76 cases, predominantly in the series: male sex, second decade of life and as a reason for consultation: volume increase with 73,5%. Osteosarcoma was the most frequent neoplasm with 34%, pathological fracture accompanied the tumors in 50% of cases, the most affected bone was the femur, the predominant Enneking stages were IIb and III. More surgeries were performed on the lower limbs, with transfemoral amputation being the most frequent. The intention of the procedures was predominantly curative with 69% of the palliative intentions, more amputations were performed at radical oncological levels than extensive ones. An overall survival of 53.4% was obtained at two years of follow-up, the phantom limb was present as a complication in over 77% of operated cases.

Osteosarcoma extraesquelético. Presentación de un caso / Extraskeletal osteosarcoma. Case report

El osteosarcoma extraesquelético es un tumor maligno de alto grado que representa menos del 2% de los sarcomas de partes blandas y que afecta principalmente a personas entre la quinta y sexta década de la vida. Típicamente el diagnóstico es tardío, con un período síntomas-diagnóstico extenso, >6 meses en comparación con el del osteosarcoma convencional. Comunicamos el caso de un hombre de 43 años que acudió con una tumoración en la región glútea y el muslo izquierdo de un año de evolución. Luego de estudios complementarios y una biopsia no concluyentes, se procedió a la resección quirúrgica del tumor con márgenes amplios. El estudio histológico de la pieza indicó osteosarcoma extraesquelético. El paciente tuvo una evolución muy buena, sin elementos de recidiva local ni compromiso a distancia luego de 20 meses de seguimiento. Nivel de Evidencia: IV

Extraskeletal osteosarcoma (EOS) is a high-grade malignant tumor that accounts for less than 2% of soft tissue sarcomas and mainly engages people between the fifth and sixth decade of life. It is typically of late diagnosis, with an extensive symptom-diagnosis period greater than 6 months compared to conventional osteosarcoma. We report the case of a 43-year-old patient who presented in our service with a 1-year-old tumor localized in the gluteal and left thigh region. After inconclusive paraclinical studies and biopsy, the tumor was surgically resected with wide margins and diagnosed as EOS after the histological analysis of the piece. The patient showed a very good clinical and paraclinical progression without signs of local or distant recurrence after 20 months of follow-up. Level of Evidence: IV

Spondylectomy for Primary Ewing Lumbar Sarcoma in Children / Espondilectomia para sarcoma lombar primário de Ewing em crianças

Abstract Primary Ewing sarcoma in the spine is very rare, and the treatment for it is multidisciplinary. There is no consensus regarding the optimal method of local control; however, en bloc resection is associated with an improvement in survival rates. The authors report a case of a 5-year-old girl who initially presented low back pain, and was diagnosed with Ewing sarcoma after being submitted to imaging studies by radiography, magnetic resonance and bone biopsy. A spondylectomy was performed in accordance with the Euro Ewing protocol. At the three-year follow-up, the patient had no restrictions regarding her daily activities, and there has been no evidence of recurrence to date.

Resumo O sarcoma de Ewing da coluna vertebral é raro, e seu tratamento é multidisciplinar. Não há consenso sobre o método ideal de controle local do tumor; no entanto, a ressecção em bloco com margens negativas está associada a uma melhora da sobrevida. Os autores relatam um caso de uma paciente de 5 anos do sexo feminino que inicialmente se apresentou com dor lombar, tendo sido diagnosticada com sarcoma de Ewing de acordo com o estudo imagiológico por radiografia, ressonância magnética e biópsia óssea. A paciente foi submetida a vertebrectomia após quimioterapia, de acordo com o protocolo Euro Ewing. Aos três anos de seguimento, não apresentou restrições nas atividades da vida diária, e, até o momento, não houve evidência de recidiva.

Lung metastases at the initial diagnosis of high-grade osteosarcoma: prevalence, risk factors and prognostic factors. A large population-based cohort study

ABSTRACT BACKGROUND: Osteosarcoma is the most prevalent malignant bone tumor in children and adolescents. Lung metastases are associated with poor prognosis. OBJECTIVE: The aim here was to explore the prevalence of and risk and prognostic factors for lung metastases in high-grade osteosarcoma patients. DESIGN AND SETTING: Retrospective cohort study based on the Surveillance, Epidemiology and End Results (SEER) database in the United States. METHODS: Data on 1,408 high-grade osteosarcoma patients registered in the SEER database between 2010 and 2015 were extracted. From these, all patients with high-grade osteosarcoma and initial lung metastasis were selected for analysis on risk and prognostic factors for lung metastases. Overall survival was estimated. RESULTS: There were 238 patients (16.90%) with lung metastases at diagnosis. Axial location, tumor size > 10 cm (odds ratio, OR 3.19; 95% confidence interval, CI: 1.58-6.45), higher N stage (OR 4.84; 95% CI: 1.94-12.13) and presence of bone metastases (OR 8.73; 95% CI: 4.37-17.48) or brain metastases (OR 25.63; 95% CI: 1.55-422.86) were significantly associated with lung metastases. Younger age and surgical treatment (hazard ratio, HR 0.46; 95% CI: 0.30-0.71) favored survival. Median survival was prolonged through primary tumor surgery. CONCLUSIONS: The factors revealed here may guide lung metastasis screening and prophylactic treatment for osteosarcoma patients. A primary tumor in an axial location, greater primary tumor size, higher lymph node stage and presence of bone or brain metastases were significantly correlated with lung metastases. The elderly group (≥ 60 years) showed significant correlation with poor overall survival. For improved survival among high-grade osteosarcoma patients with lung metastases, aggressive surgery on the primary tumor site should be encouraged.

Osteosarcoma "tipo osteoblastoma" de columna cervical: A propósito de un caso / Osteoblastoma-like osteosarcoma of the cervical spine. A case report

El osteosarcoma "tipo osteoblastoma", una variante menos agresiva del osteosarcoma, es una enfermedad poco frecuente y representa un desafío diagnóstico tanto clínico como histopatológico. Se han publicado escasos reportes de casos de este tumor. Presentamos a un paciente de 16 años con un osteosarcoma "tipo osteoblastoma" localizado en la columna cervical. La biopsia ósea bajo tomografía reveló una lesión con un patrón de crecimiento permeativo, con sospecha de osteosarcoma "tipo osteoblastoma". Se realizó una espondilectomía total en bloque mediante un doble abordaje. Se describen el caso clínico, la secuencia diagnóstica, la técnica quirúrgica y el seguimiento a 10 años. Nivel de Evidencia: IV

Osteoblastoma-like osteosarcoma, a less aggressive variant of osteosarcoma, is a rare tumor which presents a clinical and histopathological diagnostic challenge. We have found few reports of cases of this tumor in the literature. In this case, we describe an osteoblastoma-like osteosarcoma of the cervical spine in a 16-year-old patient. CT-guided bone biopsy showed a lesion with a permeated growth pattern, which led to the suspicion of an osteoblastoma-like osteosarcoma. A total en bloc spondylectomy was performed through a dual approach. We present the clinical case, the diagnostic sequence, the surgical technique and 10-year follow-up results. Level of Evidence: IV

Rabdomiólisis intraoperatoria en cirugía de resección de osteosarcoma de fémur: Reporte de caso y discusión / Case report: Intraoperative rhabdomyolisis after femur osteosarcoma resection

Rhabdomyolysis is a pathology that rarely has causes in the perioperative period, where it has been commonly related as a complication of malignant hyperthermia, prolonged patient positioning with intraoperative muscle compression, in the postoperative period of bariatric surgery and in children. The purpose of this review is to present the case of a 49 year-old male patient, who underwent limb salvage surgery for treatment of a left femur osteosarcoma, with reconstruction via bone transplant and joint prosthesis. During the procedure hyperkalemia and elevation of Creatine-Phosphokinase (CPK) enzyme levels where detected, without changes compatible with renal failure, which required repeated treatment to normalize and that, after ruling out other causes, it was attributed to skeletal muscle destruction during the procedure. Rhabdomyolysis is a phenomenon inherent to this sort of procedures and may manifest initially as laboratory findings and that, if not diagnosed in time, may lead to fatal arrhythmias and acute renal failure.

La rabdomiólisis es una patología que rara vez tiene origen en el período perioperatorio, donde comúnmente se le ha relacionado como complicación de la hipertermia maligna, de decúbitos prolongados con compresión muscular intraoperatoria, del posoperatorio de la cirugía bariátrica y en niños. El objetivo de este trabajo es presentar el caso de un hombre de 49 años, sometido a resección de un osteosarcoma de fémur izquierdo con reconstrucción mediante trasplante óseo y prótesis articular, durante el cual se detectan hiperpotasemia y aumento de la Creatin-Fosfokinasa (CPK), sin alteraciones compatibles con fallo renal, que requirió reiterados tratamientos para la normalización de los valores de kalemia, y que tras descartar otras causas se atribuyó a la destrucción de musculoesquelético durante el procedimiento. La rabdomiólisis es un fenómeno inherente a este tipo de procedimientos y puede manifestarse inicialmente con alteraciones analíticas que, de no ser diagnosticadas en tiempo y forma, pueden llevar a arritmias fatales y fallo renal agudo.

Tumor Phyllodes con transformación a osteosarcoma / Breast osteosarcoma originating in a phyllodes tumor: report of one case

Phyllodes tumors account for less than 1% of tumors of the mammary gland, have both epithelial and stromal components and are classified as benign, borderline and malignant. The malignant tumors are highly heterogeneous: they can differentiate to liposarcomas, fibrosarcomas, rhabdomyosarcomas, chondrosarcomas or osteosarcomas. The differentiation to osteosarcoma is extremely rare, constitutes 1.3% of cases and is very aggressive. The standard treatment of these tumors is surgical. The role of radiotherapy and chemotherapy is not clear. However, in patients in whom wide surgical margins are not achieved, adjuvant radiotherapy can be of help. We report a 63 years old female with a right breast osteosarcoma with an osteoclastic component, originating in a phyllodes tumor. The tumor was excised surgically and afterwards she was treated with 10 sessions of 50 Gy of radiotherapy in 25 fractions. She has remained free of disease for the last 10 months.

Osteosarcoma telangiectásico en un lactante / Telangiectatic osteosarcoma in an infant

Resumen: Introducción: El osteosarcoma es uno de los tipos de cáncer más común en la etapa escolar y en la adolescencia, y es el más común de los tumores óseos malignos en este grupo de edad. Frecuentemente, el osteosarcoma es hallado en los huesos largos de las extremidades. Existen muy pocos casos descritos en menores de 5 años. Hasta donde se sabe, no hay ningún caso reportado en lactantes. La variedad telangiectásica es poco común y no existen casos reportados antes de la edad preescolar. Caso clínico: Se presenta el caso clínico de una paciente lactante de 10 meses de edad que presentó un tumor lítico en la novena costilla derecha, que posterior a la resección fue clasificado como osteosarcoma telangiectásico. Conclusiones: El osteosarcoma telangiectásico en la edad pediátrica es poco común. A la fecha, la paciente ha presentado buena respuesta al tratamiento, aunque se desconoce el pronóstico y la supervivencia de este padecimiento.

Abstract: Background: Osteosarcoma is one of the most common types of cancer in childhood and adolescence and it is the most common malignant bone tumor in this group of age. Osteosarcoma is frequently found in long bones of the extremities. There are very few cases described in children under 5 years of age, and according to this review, none in infants. The telangiectatic variant is uncommon, and there are no reported cases before preschool age. Case report: A 10-month-old female infant with a lithic tumor of the ninth right rib, which was classified after resection as a telangiectatic osteosarcoma, is presented. Conclusions: Telangiectatic osteosarcoma in the pediatric age is very uncommon. To date, the patient has presented good response to treatment, although the prognosis and survival of this condition is unknown.

Osteosarcoma mandibular osteogénico / Mandibular osteogenic sarcoma

El osteosarcoma es una neoplasia maligna, poco frecuente, que se presenta de forma agresiva y sepuede originar en el hueso de la cavidad oral. Se caracteriza por la producción de osteoide tumoral(trabéculas óseas inmaduras) por parte de las células neoplásicas. Estos tumores suelen formarse en la región metafisiaria de los huesos largos de los miembros, en especial en el fémur y en la tibia. Se presenta el caso clínico de un paciente que concurre al servicio de Estomatología de la Escuela deOdontología, de la facultad de Ciencias de la Salud, Universidad Católica de Córdoba; masculino de 26 años de edad de nacionalidad peruana con diagnóstico clínico de osteosarcoma mandibular, el cual fue tratado con hemisección, quimioterapia y radioterapia en la zona mandibular sector posterior de lado izquierdo con colocación de una placa de titanio en la región.

Osteosarcoma is a malignant, rare and aggressive neoplasm that originates in the bone of the oralcavity. It is characterized by the production of tumor osteoid (immature bone trabeculae) by neoplasticcells. These tumors usually originate in metaphyseal region of long limb bones, especially in femurand tibia.Case report: 26-year-old peruvian male patient attending the Stomatology service at the School ofDentistry, Faculty of Health Sciences, Catholic University of Cordoba. The patient was clinically diagnosedwith mandibular osteosarcoma, treated with hemisection, chemotherapy, radiotherapy onmandibular zone posterior sector of left side and fixation of a titanium bone plate.

Reconstrucción en osteosarcoma humeral con peroné vascularizado y técnica modificada de tenosuspensión / Reconstruction in humeral osteosarcoma with a vasularised fibula and a modified sling procedure

INTRODUCCIÓN: El osteosarcoma es el tumor óseo maligno más común en la niñez y la adolescencia. Se desarrolla por orden de frecuencia en metáfisis distal de fémur, proximal de tibia y húmero proximal. La variedad de presentación de osteosarcoma telangectásico es rara, y afecta principalmente a niños y adolescentes. Las opciones de tratamiento quirúrgico son cirugía de salvamento y radical. Actualmente la mayoría de los tumores óseos malignos de la cintura escapular pueden seguir la primera opción. OBJETIVO: Presentar una técnica modificada de tenosuspensión para la reconstrucción de defectos humerales en el tratamiento quirúrgico del osteosarcoma de húmero. REPORTE DE CASO: Se presenta el caso de un varón de 16 años de edad con diagnóstico de osteosarcoma telangectásico de húmero tratado con cirugía de salvamento mediante técnica modificada de suspensión con peroné libre vascularizado para la reconstrucción del defecto humeral posterior a resección S345B según la Musculoskeletal Tumour Society (MSTS) y los resultados a 6 y 12 meses. RESULTADOS: A 6 meses del posquirúrgico existió consolidación del injerto sin complicaciones. A los 12 meses no se encontraron complicaciones. Los resultados funcionales fueron del 82 y 86% MSTS, respectivamente. CONCLUSIONES: La técnica modificada de suspensión de hombro utilizando peroné vascularizado y tendón del manguito rotador residual otorga resultados satisfactorios en el caso presentado, sin embargo, se requiere de mayor investigación para considerarla como modalidad de tratamiento generalizado en defectos humerales posterior a resección en bloque como tratamiento quirúrgico.

INTRODUCTION: El osteosarcoma is the most common malignant bone tumour in paediatric patients. The most frequent locations of osteosarcoma are the distal femur, the proximal tibia, and the proximal humerus. Telangiectatic osteosarcoma is rare, most commonly affecting children and adolescents. Surgical treatment options include limb sparing procedures and amputation, with most shoulder tumours currently being able to be treated without amputation. OBJECTIVE: To present a modified sling procedure to reconstruct humeral defects after bone resections in humeral osteosarcoma. CASE REPORT: A 16 year-old male patient with telangiectatic osteosarcoma of the proximal humerus was treated with limb sparing surgery. A modified sling procedure was performed using a vascularised fibular graft to reconstruct the humerus after in-block resection. Results at 6 and 12 months follow up are presented. RESULTS: Graft consolidation was observed at 6 months follow-up, and no complications were observed at 6 and 12 months follow-up. Functional results, according to the Musculoskeletal Tumour Society (MSTS), at 6 and 12 months were 82% and 86%, respectively. CONCLUSIONS: This modified sling procedure using a vascularised fibular graft and rotator cuff tendon showed satisfactory results in this case. Future studies should further evaluate its role in treating humeral defects after in-block resection.

Post-operative hemimaxillectomy rehabilitation using prostheses supported by zygoma implants and remaining natural teeth

OBJECTIVES: This study aimed to evaluate the stability of prostheses supported by zygoma implants and remaining teeth for subjects who had undergone hemi-maxillectomy. METHODS: Ten patients were included in the study. Oral rehabilitation was performed using a temporary prosthesis that was supported by remaining teeth for the first three months. Then, a zygoma implant was placed to provide support for a final prosthesis in addition to the remaining teeth. Each prosthesis was tailor-made according to biomechanical three-dimensional finite element analysis results. The patients were assessed using the prosthesis functioning scale of the Memorial Sloan-Kettering Cancer Center. In addition, retention and bite force were recorded for both the temporary prosthesis and the final prosthesis. RESULTS: The mean bite force of the prosthetic first molar was increased to 69.2 N. The mean retentive force increased to 13.5 N after zygoma implant insertion. The bite force on the prosthetic first molar was improved to 229.3 N. CONCLUSION: Bite force increased significantly with the support of a zygoma implant. The use of zygoma implants in the restoration of maxillary defects improved functional outcome and patient satisfaction.

Osteosarcoma con presentación atípica en tercio medio de diáfisis de fémur. Reporte de un caso clínico y revisión de la literatura / Atipic osteosarcoma in the femoral shaft case report and review of the literature

Resumen: El osteosarcoma es el tumor maligno primario más frecuente del hueso. Se caracteriza por la formación de osteoide por las células tumorales. Su localización más frecuente es en las metáfisis de los huesos largos; sin embargo, la presentación puramente diafisaria se reporta en 10% de los casos. Se expone el caso de paciente del género femenino de 25 años que inició padecimiento cuatro meses previos a nuestra valoración al presentar dolor y aumento de volumen en tercio medio de muslo derecho, sin causa aparente. Las radiografías del fémur revelaron un tumor en la diáfisis del mismo. La resonancia magnética mostró extensión a partes blandas, sin compromiso del paquete neurovascular. Se tomó biopsia incisional con reporte histopatológico de osteosarcoma osteoblástico y condroblástico. Se clasificó como Enneking IIB, AJCC IIB. Inició tratamiento con tres ciclos de quimioterapia neoadyuvante y posteriormente se le realizó resección intercalar de fémur más reconstrucción con clavo centromedular y espaciador diafisario. El reporte histopatológico evidenció necrosis de 95% (Huvos grado III). Se continuó con tres ciclos de quimioterapia adyuvante. A 18 meses de concluida la quimioterapia adyuvante la paciente se encuentra sin datos de actividad tumoral local o a distancia, tolerando la deambulación sin auxiliares de la marcha. A pesar de que el osteosarcoma no se presenta de forma habitual en el tercio medio de la diáfisis de los huesos largos, debe siempre tomarse en cuenta como un diagnóstico diferencial con otras patologías de localización más frecuente en dicha región. Esta localización nos permite una amplia gama de alternativas quirúrgicas con la preservación de las articulaciones adyacentes.

Abstract: Osteosarcoma is the most frequent primary malignant bone tumor. It is characterized by osteoid production by tumor cells. Its most frequent location is in the metaphyses of long bones, but a purely diaphyseal presentation is reported in 10% of cases. We report the case of a female 25 year-old patient whose symptoms of pain and swelling of the right mid thigh started four months before, without an apparent cause. Femur X-rays showed a tumor in the femoral shaft. The MRI showed extension to soft tissues with no compromise of the neurovascular bundle. The histopathologic report of the incisional biopsy was osteoblastic and chondroblastic osteosarcoma, classified as Enneking IIB, AJCC IIB. Treatment consisted of three cycles of neoadjuvant chemotherapy. Then intercalary femur resection plus reconstruction with centromedullary nailing and a diaphyseal spacer were performed. The histopathologic report was 95% necrosis (Huvos grade III). Three cycles of adjuvant chemotherapy were given and now, 18 months after completing the latter, the patient has no signs of local or distant tumor activity, and she can walk unassisted. Despite the fact that osteosarcoma does not occur usually in the mid shaft of long bones, we should always bear it in mind as part of the differential diagnosis for other conditions that occur more frequently in that region. Such location allows for a broad gamut of surgical approaches that spare the adjacent joints.

Osteosarcoma parostal de bajo grado: Evolución oncológica y clínica / Low grade parosteal osteosaroma. Clinical and oncological outcomes]

El objetivo de este estudio fue analizar una serie de pacientes con osteosarcoma parostal de bajo grado y los resultados de su tratamiento quirúrgico y reconstrucción con un trasplante óseo. Se realizó una búsqueda retrospectiva en nuestra base de datos oncológica entre 1980 y 2010 de todos los pacientes con diagnóstico de osteosarcoma parostal. Se incluyeron para el análisis únicamente los osteosarcomas parostales de bajo grado, tratados quirúrgicamente con cirugía de conservación de miembro y reconstruidos con trasplante óseo cadavérico fresco congelado. Se incluyeron 22 pacientes en el estudio. La edad media de la serie fue de 32 ± 11 años (10-59) y el seguimiento medio de 93 ± 69 meses (8-237). La supervivencia global de los pacientes analizados fue de 91% (IC95%: 79-100) a 10 años. Cuatro pacientes (18%) presentaron una recidiva local de la enfermedad, dos de éstas fueron clasificadas histológicamente como osteosarcoma parostal desdiferenciado. Dos pacientes desarrollaron enfermedad a distancia, siendo el pulmón el único sitio de localización. La tasa de supervivencia de las reconstrucciones de la cirugía de conservación de miembro a 10 años fue de 65% (IC95%: 44-86). La supervivencia a largo plazo de los pacientes con osteosarcoma parostal de bajo grado es superior al 90%. La resección quirúrgica con márgenes amplios debe ser el tratamiento de elección y la reconstrucción biológica es una alternativa válida.

The objective of the study was to analyze a group of patients with low grade parosteal osteosarcoma treated with limb salvage surgery and reconstructed with bone allograft. A retrospective review from our oncologic data base between 1980 and 2010 was done and all patients with diagnosis of low grade parosteal osteosarcoma, treated with limb salvage surgery and reconstructed with allograft were included. Twenty-two patients were included for the analysis. The mean age was 32 ± 11 years (10-59) y the mean follow-up 93 ± 69 months (8-237). Ten year overall survival of the series was 91% (95%CI: 79-100). Four patients developed local recurrence, 2 of them histological classified after the resection dedifferentiated parosteal osteosarcoma. Two patients developed distant recurrence, being the lung the only site of metastasis. Ten year limb salvage reconstruction survival was 65% (95%CI: 44-86). Long term survival rate in low grade parosteal osteosarcoma is over 90%. Surgical resection wide margin should be the elective treatment and biological limb salvage reconstruction is a good alternative.

Undifferentiated osteosarcoma of the mandible oblique line: a case report / Osteossarcoma indiferenciado da mandíbula: relato de caso clínico na região do trígono retromolar

O osteossarcoma (OS) é o tumor maligno primário mais comum. Entretanto, o osteossarcoma localizado na mandíbula (JOS) é raro, agressivo e maligno, constituindo de 5-13% dos casos de OS do corpo todo. O JOS tem predileção pelo sexo masculino com idade de 34-36 anos. O prognóstico da doença está associado com diversas variáveis tais como a localização do tumor, fase de desenvolvimento, existência ou não de metástases, sexo, idade, e resposta do organismo ao tratamento. Este artigo relata um caso incomum de osteossarcoma localizado na região do trígono retromolar mandibular, composto por osso mesenquimal primitivo, que comumente ocorre nas regiões de extremidades e ossos longos...

This article reports a case of 82 years old woman, who consulted in a private radiology dental clinic for implants planning. In a panoramic radiography, a radiopaque area with sun-ray appearance located above the external oblique line on the right side was observed. The incisional biopsy showed presence of sarcomatous stroma presenting osteoid tissue with irregularly shaped and large amount of osteoblasts, varied sizes and shapes, with prominent nuclei, intensely colored, arranged in a disorderly way around trabecular bone. Histopathological diagnosis was obtained for undifferentiated osteosarcoma. Osteosarcoma located in the jaw (JOS) is rare and aggressive, constituting 5% to 13% of all cases of skeletal osteosarcoma. JOS has a male predilection in third decade of life...

Ossificação do pedículo vascular de um retalho livre de fíbula: relato de caso / Ossification of vascular pedicle in fibula free flap: a case report

Introdução: Várias são as vantagens da utilização de retalhos fibulares para as reconstruções de defeitos craniomaxilofaciais, incluindo a baixa morbidade da área doadora, boa qualidade óssea possibilitando a realização de implantes osteointegrados quando indicados, além da possibilidade de inclusão de uma ilha de pele quando indicado. Durante a dissecção do retalho, próximo à região do pedículo vascular, normalmente inclui-se um cuff muscular e uma faixa de periósteo. O potencial osteogênico do periósteo transplantado tem sido objeto de estudo. Relato de caso: paciente de 15 anos, submetido à reconstrução microcirúrgica com um retalho fibular para um defeito mandibular pós-ressecção de um sarcoma ósseo. Evoluiu com aumento de volume, de consistência óssea na região cervical próximo à cervicotomia realizada para anastomose vascular. Exames de imagem mostravam características ósseas da massa. Foi então submetido à nova cervicotomia e exploração da massa, sendo observada uma formação de tecido ósseo no local da anastomose vascular. Exame anatomopatológico da peça mostrava formação de tecido ósseo adjacente ao retalho periostal. Discussão: Durante a dissecção do retalho fibular, a osteotomia é realizada a alguns centímetros da articulação do joelho, isto a fim de facilitar a dissecção do pedículo vascular na região do oco poplíteo. O pedículo vascular fica então envolto por uma cuff muscular e por uma tira de periósteo. Este mantém sua capacidade osteogênica, que pode ser ativada de acordo com o estímulo do local. A ossificação do periósteo do pedículo vascular de retalhos livres de fíbula permanece um evento raro, porém relatado por centros diferentes.

Introduction: The use of fibula flaps for the reconstruction of craniomaxillofacial defects has many advantages, including the low morbidity of the donor area, good bone quality for use of osseointegrated implants, and the possibility to include a skin island, when indicated. During the dissection of the flap, a muscle "cuff" and a periosteal strip are usually included near the region of the vascular pedicle. The osteogenic potential of the transplanted periosteum has been the object of studies. Case report: A 15-year-old male patient underwent microsurgical reconstruction using a fibula flap for a mandibular defect caused by the resection of a bone sarcoma. He developed increased volume and bone consistency in the cervical region next to the area where a cervicotomy was performed for vascular anastomosis. Imaging examinations showed the characteristics of the bone mass. He then underwent a new cervicotomy and mass exploratory surgery because bone tissue formation was observed at the site of vascular anastomosis. Anatomopathological examination of the specimen showed bone tissue formation next to the periosteal flap. Discussion: During fibula flap dissection, osteotomy is performed a few centimeters from the knee joint to facilitate the dissection of the vascular pedicle in the region of the popliteal fossa. Then, the vascular pedicle is surrounded by a muscle cuff and periosteal strip. This maintains its osteogenic capacity, which can be activated according to the stimulus of the area. Although periosteal ossification of the vascular pedicle in fibula free flaps is a rare event, it has been reported in different centers.

A meta-analysis of limb-salvage versus amputation in the treatment of patients with Enneking II pathologic fracture osteosarcoma.

Introduction: The aim of this meta-analysis was to further explore whether the relapse, 5-year survival and metastasis the same or not between limb-salvage and amputation in the treatment of patients with limited stage Enneking‡U pathologic fracture osteosarcoma. Materials and Methods: An electronic search of the Medline, EMBASE and CNKI was done on October 2014. The clinical studies about amputation or limb-salvage surgery in the treatment of patients with limited stage Enneking‡U pathologic fracture osteosarcoma were searched and reviewed. The effect size of relapse, 5-year survival and metastasis between the amputation and limb-salvage surgery were pooled by stata11.0 software (Stata Corporation, College Station, TX, USA, http://www.stata.com;) using random or fixed effect model. The funnel plot and Egger's line regression test were used for evaluation of publication bias. Results: A total of 89 studies were identified and seven articles with 200 cases in the limb-salvage surgery group and 84 subjects in the amputation group were finally included in the meta-analysis. The pooled data indicated that no statistical different of risk for developing relapse between limb-salvage and amputation was found relative risk (RR) =1.40, 95% confidence interval (CI): 0.71-2.79, (P = 0.33). The 5-year survival rate of patients underwent limb-salvage surgery was smaller than patients received amputation RR = 1.86, 95%CI: 1.19-2.89, (P = 0.01); the metastasis rate of patients underwent limb-salvage surgery was significant decreased compared with patients received amputation RR = 0.56, 95% CI: 0.34-0.94, (P = 0.03). No publication bias was existed in this meta-analysis. Conclusion: Limb-salvage surgery does not increased the risk of relapse compared with amputation in the treatment of patients with limited stage Enneking‡U pathologic fracture osteosarcoma.

Osteosarcoma condroblástico gigante del fémur: reporte de un caso / Giant chondroblastic osteosarcoma of the femur: a case report

El osteosarcoma es un tumor maligno compuesto de células fusiformes, que se caracteriza por la producción de tejido osteoide y hueso, su crecimiento es rápido con extensión locoregional y difusión metastásica a pulmón. Generalmente 20% de los pacientes presentan metástasis en el momento del diagnóstico. Se observa con frecuencia en los huesos largos de los adolescentes y adultos jóvenes con mayor afectación del sexo masculino. El tratamiento de los pacientes con osteosarcomas se basa en la resección amplia y completa o en una amputación del tumor primario, y en la administración de quimioterapia coadyuvante. Presentamos el caso de paciente masculino de 21 años, quien consultó por una tumoración en la rodilla izquierda de rápido crecimiento hasta alcanzar 80 cms de perímetro. La biopsia incisional reportó un condrosarcoma condroblástico. En vista de no tener respuesta al tratamiento coadyuvante, se decidió realizar una desarticulación coxofemoral. Cuando el osteosarcoma es resistente a la quimioterapia y su crecimiento continúa, se deben realizar procedimientos radicales para garantizar la sobrevida del paciente(AU)

Osteosarcoma is a malignant tumor composed of spindle cells, characterized by the production of osteoid tissue and bone is growing rapidly, with locoregional extension and metastatic spread to the lung. Generally 20% of patients have metastases at diagnosis. It most often occurs in the long bones of adolescents and young adults with greater male involvement. Treating osteosarcoma patients is based on wide and complete resection or amputation of the primary tumor and the administration of adjuvant chemotherapy. We present the case of male patient 21, who presented a tumor in his left knee rapidly growing up to 80 cm in circumference. Incisional biopsy chondroblastic reported chondrosarcoma. In view of having no adjuvant treatment response was decided to hip disarticulation. When osteosarcoma is resistant to chemotherapy and growth continues, radical procedures must be performed to ensure the survival of the patient(AU)

Is [99m]Tc-MIBI scintigraphy a predictor of response to pre-operative neoadjuvant chemotherapy in osteosarcoma?

Multidrug resistance [MDR], which may be due to the over expression of P-glycoprotein [Pgp] and/or MRP, is a major problem in neoadjuvant chemotherapy of osteosarcoma. The aim of this study was to investigate the role of Tc-99m MIBI scan for predicting the response to pre-operative chemotherapy. Twenty-five patients [12 males and 13 females, aged between 8 and 52y] with osteosarcoma were studied. Before the chemotherapy, planar [99m]Tc-MIBI anterior and posterior images were obtained 10-min [tumor-to-background ratio: [T[1]/B[1]][10min]] and 3-hr after tracer injection. After completion of chemotherapy, again [99m]Tc-MIBI scan was performed at 10-min after tracer injection. In addition to calculation of decay corrected tumor to background [T/B] ratios, using the 10-min and 3-hr images of the pre-chemotherapy scintigraphy, percent wash-out rate [WR%] of [99m]Tc-MIBI was calculated. Using the 10-min images of the pre- and post-chemotherapy scans, the percent reduction in uptake at the tumor site after treatment [Red%] was also calculated. Then after surgical resection, tumor response was assessed by percentage of necrosis. All patients showed significant [99m]Tc-MIBI uptake in early images. Only 9 patients showed good response to chemotherapy [necrosis >/= 90%] while 16 patients were considered as non-responder [necrosis <90%]. There was no statistical significant difference between non-responders and responders in [T[1]/B[1]] [10min].There was a significant negative correlation between WR% and percentage of necrosis [P=0.001]. On the other hand, there was a significant correlation between Red% and percentage of necrosis [P<0.001].There was also statistical significant difference in WR% and Red% between non-responders and responders [both P< 0.001]. Washout rate of [99m]Tc-MIBI in pre-chemotherapy scintigraphy as well as Red% using pre- and post-chemotherapy MIBI scintigraphy are useful methods for predicting response to neoadjuvant chemotherapy

Flexible resins in the rehabilitation of maxillectomy patient.

Surgical resection is an established and common method for treatment of maxillofacial tumors. Such intervening surgery in this region may involve the destruction of antral, nasal, or orbital contents and jaws. Normally, the end result is a defect which may affect speech, swallowing, mastication and facial appearance. Prosthodontic restoration of functions and facial form is an excellent method in the replacement of missing parts and restoration of contour. Pertinent research in materials, designs and methods have resulted in whole array of possible prosthess that can be provided to meet specific patient needs. This case report describes the fabrication of definitive obturator prosthesis made of flexible acrylic resin on a chrome cobalt base.