RESUMO

Small cell osteosarcoma (SCOS) is a rare variant of conventional osteosarcoma, characterized by tumor cells of small size and uniform morphology, which can lead to diagnostic confusion with other small cell tumors, requiring a detailed diagnostic approach. The manifestation in a child adds a degree of complexity, as the management of malignant tumours in paediatric patients requires specific considerations to minimize the long-term side effects of oncological treatment and preserve the structural and functional development of the orofacial region. This report concerns an 8-year-old female patient referred to the Oral and Maxillofacial Surgery outpatient clinic with progressive swelling in the right maxillofacial region, initially asymptomatic, but progressing to pain and difficulty chewing. A cone beam computed tomography scan was requested and an incisional biopsy was carried out for histopathological and immunohistochemical analysis, which confirmed the pathological entity. The lesion was then completely resected with a safety margin and the affected area removed to restore functionality and aesthetics. The surgical specimen was sent for further histopathological analysis, which confirmed the diagnosis of SCOS. Detailed immunohistochemical analysis was crucial to the diagnosis, and a comprehensive surgical approach was indicated given the aggressive behavior of the lesion. This report emphasizes the importance of an integrated multidisciplinary approach, combining oncology, pathology and oral and maxillofacial surgery.

RESUMO

Background: Primary malignant bone tumors are rare however, have a high global mortality rate. Osteosarcoma and chondrosarcoma are the most common bone sarcomas in the pelvis. The surgical management of primary bone tumors in the pelvis is challenging and depends on several factors. Internal hemipelvectomy with extremity preservation has become more popular compared to external hemipelectomy. Objective: Identify and describe characteristics of the surgical management of bone tumors and soft tissues of the pelvis by means of hemipelvectomy. Material and methods: Observational study of consecutive cases, in which patients with bone and soft tissue tumors undergoing hemipelvectomy were identified from January 2010 to December 2020 at the Oncology Surgery Department, using the Musculoskeletal Tumor Society (MSTS) scale. Results: 17 patients with pelvic tumors were included, 10 (58.8%) primary bone sarcomas, 3 (17.6%) soft tissue sarcomas, 3 (17.6%) benign bone tumors, 1 (5.8%) neoplasia of hematological origin. Average follow-up of 30 ± 14. Most frequent surgical complication, surgical wound seroma, 6 patients (37.5%). Conclusions: A multidisciplinary team is required to individualize treatment with the best sequence of options, giving improvement in oncological and functional results.

Introducción: os tumores óseos malignos primarios son raros, pero tienen una alta mortalidad global. El osteosarcoma y el condrosarcoma son los sarcomas óseos más comunes en la pelvis. El manejo quirúrgico de los tumores óseos primarios en la pelvis es desafiante y depende de varios factores. La hemipelvectomía interna con preservación de la extremidad se ha vuelto más popular en comparación con la hemipelvectomía externa. Objetivo: identificar y describir características del manejo quirúrgico de tumores óseos y tejidos blandos de pelvis mediante hemipelvectomía. Material y métodos: estudio observacional de casos consecutivos, en el que se identificaron pacientes con tumores óseos y tejidos blandos sometidos a hemipelvectomía en período de enero del 2010 a diciembre del 2020 en el Servicio de Cirugía Oncología, utilizando la escala MSTS (Musculoskeletal Tumor Society). Resultados: se incluyeron 17 pacientes con tumores pélvicos, 10 (58.8%) sarcomas óseos primarios, 3 (17.6%) sarcomas de tejido blando, 3 (17.6%) tumores óseos benignos, 1 (5.8%) neoplasia de origen hematológico. Seguimiento promedio de 30 ± 14. La complicación quirúrgica más frecuente fue el seroma de herida quirúrgica, presente en 6 pacientes (37.5%). Conclusiones: se requiere equipo multidisciplinario para individualizar tratamiento con la mejor secuencia de opciones, dando mejoría de resultado oncológico y funcional.

RESUMO

INTRODUCTION: surface sarcomas are a rare entity that need correct diagnosis to differentiate parosteal (cPOS), periosteal and the high grade surface osteosarcomas (HGSO). HGSO has malignant behavior similarities with osteosarcomas and wide resection is the key to a successful treatment.1 The Capanna and Hemi-Capanna reconstruction techniques have being developed in order to avoid amputation after an oncological resection, allowing structural support from an allograft and biological advantages from a vascularised autograft. CASE PRESENTATION: 46 years old male presenting with knee pain and 4 × 3 cm soft tissue tumor on the right tibial surface diagnosed of High Grade Surface Osteosarcoma (HGSO). Was treated by oncological resection followed by reconstruction with allograft and ipsilateral fibula autograft following the "Hemi-Capanna" technique and pedicled medial gastrocnemius flap. CONCLUSION: sufficient evidence supports the use of the Capanna technique in major musculoskeletal reconstructions. The new "Hemi-Capanna" technique has less evidence but proves to be an easier surgical technique with good functional results and little complications.

INTRODUCCIÓN: los sarcomas de superficie son una entidad rara que necesita un diagnóstico correcto para diferenciar el parostio (cPOS), el perióstico y el osteosarcoma de superficie de alto grado (HGSO). HGSO tiene similitudes de comportamiento maligno con los osteosarcomas y la resección amplia es la clave para un tratamiento exitoso. Las técnicas de reconsrucción Capanna y Hemi-Capanna han sido desarrolladas para evitar la amputación después de una resección por cáncer, permitiendo el soporte estructural de un aloinjerto y las ventajas biológicas de un autoinjerto vascularizado. PRESENTACIÓN DEL CASO: varón de 46 años que presenta dolor en rodilla y tumor de tejido blando de 4 × 3 cm en superficie tibial derecha diagnosticado con osteosarcoma de superficie de alto grado (OSAG). Se trató mediante resección oncológica seguida de reconstrucción con aloinjerto y autoinjerto de peroné ipsilateral siguiendo la técnica de "Hemi-Capanna" y colgajo pediculado de gastrocnemio medial. CONCLUSIÓN: existe evidencia suficiente que respalda el uso de la técnica de Capanna en reconstrucciones musculoesqueléticas mayores. La nueva técnica "Hemi-Capanna" tiene menos evidencia, pero demuestra ser una técnica quirúrgica más sencilla, con buenos resultados funcionales y pocas complicaciones.

RESUMO

Pain, a prevalent and debilitating symptom in cancer patients, significantly diminishes the quality of life for both individuals and their families. Addressing this critical issue, our study presents the case of a 15-year-old diagnosed with synchronous multifocal multicentric osteosarcoma. We utilized radiofrequency ablation of bilateral splanchnic nerves, a strategy of multimodal pain and palliative care. This approach not only proved to be safe and effective but also markedly improved the patient's quality of life. Our findings shine a light of hope, emphasizing the paramount importance of innovative pain management in pediatric oncology, especially in the final stages of life. This case report highlights the unwavering dedication to excellence in relieving suffering, offering hope for patients grappling with cancer.

Pain is a common and serious problem for cancer patients, osteosarcoma is a type of bone cancer that often affects children. making life hard for them and their families. We used a therapy called radiofrequency ablation on specific nerves to manage the pain. In the case of the patient's abdominal pain, this therapy was safe, worked well, and greatly improved the patient's quality of life. Our findings show the importance of new pain management methods in helping children with cancer, helping them reduce pain, using fewer strong pain medications and helping children in this case in the final stage of life.

RESUMO

BACKGROUND: Bone radiation-induced sarcomas (B-RIS) are secondary neoplasms with reportedly worse overall survival than de novo bone sarcoma. Treatment strategy for these neoplasms remains uncertain. Our systematic review sought to assess overall survival based on histology and surgical intervention. METHODS: A systemic review was conducted following Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines and registered in PROSPERO (438415). Studies describing oncologic outcomes of patients with B-RIS in the appendicular and axial skeleton were included. The Strengthening the Reporting of Observational Studies in Epidemiology checklist was used for quality assessment. Survival analysis by histologic subtype and surgery type was performed in a subset of 234 patients from 11 articles with individualized data. A total of 20 articles with a total of 566 patients were included. The most frequent location was the pelvis (27.7%), and the main histological types were osteosarcoma (69.4%), undifferentiated pleomorphic sarcoma (14.1%), and fibrosarcoma (9.2%). Limb-salvage and amputation were performed in 68.5% and 31.5% of cases, respectively. RESULTS: Local recurrence was 13%, without difference between limb-salvage surgery and amputation (p = 0.51). The metastasis rate was 42.3%. Five-year OS was 43.7% (95% confidence interval [CI], 33.3%-53.5%) for osteosarcoma, 31.5% (95% CI, 11.3%-54.2%) for UPS, and 28.1% (95% CI, 10.6%-48.8%) for fibrosarcoma. Five-year OS was 49.2% (95% CI, 35.3%-61.6%) for limb-salvage and 46.9% (95% CI, 29.1%-62.9%) for amputation. There was no difference in 5-year OS between histologic subtypes (p = 0.18) or treatment type (p = 0.86). CONCLUSION: B-RIS demonstrated poor OS at 5 years after initial management regardless of histology. Limb-salvage surgery was not associated with lower 5-year OS compared with amputation. Future studies should compare both groups while controlling for confounders. LEVEL OF EVIDENCE: Level III. See Instructions for Authors for a complete description of levels of evidence.

RESUMO

Abstract The present study describes the case of a male adult with an osteosarcoma in the proximal tibia, treated with limb salvage with endoprosthesis and chemotherapy. The patient developed an unusual metastatic pattern compromising the liver, bone, and inguinal lymph nodes, without local recurrence in the tibia or pulmonary metastases. Osteosarcoma (OS) is the second most frequent primary bone tumor after multiple myeloma in adults. Frequent sites of metastases in case of disease progression are the lungs and bone. Extrapulmonary metastases are rare. The development of new schemes of chemotherapy have improved life expectancy in osteosarcoma patients but have also altered the usual patterns of metastases, resulting in unusual metastatic locations.

Resumo Homem adulto com osteossarcoma na tíbia proximal, tratado com cirurgia de salvamento de membro com endoprótese e quimioterapia. Ele desenvolveu um padrão metastático incomum, comprometendo fígado, ossos e linfonodos inguinais, sem recorrência local na tíbia nem metástases pulmonares. O osteossarcoma (OS) é o segundo tumor ósseo primário mais frequente, depois do mieloma múltiplo em adultos. Os locais frequentes das metástases, em caso de progressão da doença são os pulmões e os ossos. As metástases extrapulmonares são raras. O desenvolvimento de novos esquemas de quimioterapia melhorou a expectativa de vida dos pacientes com osteossarcoma, porém, alterou também os padrões usuais de metástases, resultando em localizações metastáticas incomuns.

RESUMO

INTRODUCTION: parosteal osteosarcoma is an extramedullary malignant bone tumor in which cells produce osteoid, represents less than 5% of all osteosarcomas, it occurs predominantly in women between the second and fourth decade of life. It is often located in the distal region of the femur and proximal tibia. Clinically it presents with increased volume and thigh or knee pain. Due to its low incidence and clinical features, a clinical case of femoral parosteal osteosarcoma is presented, with description of the surgical technique performed. CASE REPORT: a 14-year-old female presented with a 6-month history of increased volume and right thigh pain. Radiological studies revealed a bone lesion with malignant characteristics, for which she was sent to third-level hospital where oncology study protocol was set up; consisting in two percutaneous biopsies of the lesion with Jamshidi needle, which were histopathology reported as negative for malignant cells. The pulmonary high-resolution computed tomography showed metastasis and a Tc-99m MDP bone scintigraphy showed increased osteoblastic activity in the right femoral shaft. Given the results, is confirmed the need of en-bloc resection and intercalary prosthesis implantation with adjuvant chemotherapy. CONCLUSION: the intercalary prosthesis is a suitable therapeutic option in limb-salvage surgery for patients with femoral parosteal osteosarcoma.

INTRODUCCIÓN: el osteosarcoma parostal es un tumor óseo maligno extramedular en el cual las células tumorales producen osteoide. Representa menos de 5% de los osteosarcomas. Se presenta predominantemente en la mujer, entre la segunda y cuarta década de la vida. Su localización más frecuente es la región distal del fémur y proximal de la tibia. Clínicamente, se manifiesta con aumento de volumen y dolor en muslo o rodilla. Debido a su baja incidencia y características clínicas, se presenta un caso clínico de osteosarcoma parostal femoral con descripción de la técnica quirúrgica realizada. CASO CLÍNICO: femenino de 14 años edad con cuadro clínico caracterizado por aumento de volumen y dolor en muslo derecho de seis meses de evolución. Se realizaron radiografías de fémur derecho, encontrando lesión ósea con características compatibles de malignidad, por lo que es enviada a unidad de tercer nivel para iniciar protocolo oncológico; se realizan dos biopsias percutáneas con aguja de Jamshidi, ambas con reporte histológico negativo para células malignas. En tomografía pulmonar de alta resolución se observó presencia de metástasis y la gammagrafía ósea con Tc99 reportó actividad osteoblástica en fémur derecho. Se decide tratamiento con resección en bloque y colocación de prótesis intercalar más quimioterapia adyuvante. CONCLUSIÓN: la prótesis intercalar resulta una opción terapéutica adecuada en la cirugía de salvamento de extremidad para pacientes con diagnóstico de osteosarcoma parostal femoral.

RESUMO

Introdução: O osteossarcoma consiste na neoplasia maligna mais comum do tecido ósseo. Possui seu pico de incidência em pacientes jovens, na segunda década de vida, sendo pouco frequentes os casos em pacientes mais idosos. O tumor se caracteriza por ser produtor de matriz óssea e derivado de células-tronco mesenquimais. A histopatologia da lesão é complementada pela imuno-histoquímica, através da expressão de marcadores como SATB2 e Ki-67. Objetivo: Relatar um caso clínico de paciente em idade atípica de acometimento por osteossarcoma e correlacionar com os dados préexistentes na literatura sobre o tema. Material e Método: Trata-se de relato de caso baseado em levantamento de prontuário de paciente com osteossarcoma. Resultados: Paciente do sexo masculino, 63 anos, apresentando dor e massa palpável em região de joelho direito. Realizados exames de imagem que evidenciaram lesão agressiva, com componentes líticos e escleróticos. Biópsia da lesão mostrou-se sugestiva de osteossarcoma. Após diagnóstico, foi realizada desarticulação coxofemoral de membro inferior direito. O estudo anatomopatológico revelou lesão, medindo 13,0 x 8,0 cm, compatível com osteossarcoma de alto grau, apresentando invasão linfovascular e estadiamento patológico pT3. Conclusão: Osteossarcomas em pacientes idosos configuram uma rara apresentação da neoplasia, sendo de fundamental importância a divulgação de casos na literatura para estabelecimento de condutas entre equipes. (AU)

Introduction: Osteosarcoma is the most common malignant neoplasm of bone tissue. It has its peak incidence in young patients in the second decade of life, and cases in older patients are infrequent. The tumor is characterized by being a producer of bone matrix and derived from mesenchymal stem cells. The histopathology of the lesion is complemented by immunohistochemistry, through the expression of markers such as SATB2 and Ki-67. Objective: To report a clinical case of a patient at atypical age of involvement by osteosarcoma and correlate with pre-existing data in the literature on the subject. Material and Method: This is a case report based on medical records of patients with osteosarcoma. Results: Male patient, 63 years old, presenting pain and palpable mass in the right knee region. Imaging tests were performed that showed aggressive lesion, with lytic and sclerotic components. Biopsy of the lesion was shown to be suggestive of osteosarcoma. After diagnosis, hip dysfunction of the right lower limb was performed. The anatomopathological study revealed lesion, measuring 13.0 x 8.0 cm, compatible with highgrade osteosarcoma, presenting lymphovascular invasion and pathological staging pT3. Conclusion: Osteosarcomas in elderly patients constitute a rare presentation of the neoplasm, being of fundamental importance the disclosure of cases in the literature to establish conducts between teams. (AU)

Introducción: El osteosarcoma es la neoplasia maligna más frecuente del tejido óseo. Tiene su máxima incidencia en pacientes jóvenes en la segunda década de la vida, con casos poco frecuentes en pacientes de mayor edad. El tumor se caracteriza por ser productor de matriz ósea y derivar de células madre mesenquimales. La histopatología de la lesión se complementa con la inmunohistoquímica, mediante la expresión de marcadores como SATB2 y Ki-67. Objetivo: Relatar un caso clínico de paciente en edad atípica de acometimiento por osteosarcoma y correlacionarlo con los datos preexistentes en la literatura sobre el tema. Material y Método: Se trata de un informe de caso basado en la historia clínica de un paciente con osteosarcoma. Resultados: Paciente varón de 63 años que presenta dolor y masa palpable en la región de la rodilla derecha. Los exámenes de imagen mostraron una lesión agresiva con componentes líticos y escleróticos. La biopsia de la lesión fue sugestiva de osteosarcoma. Tras el diagnóstico, se realizó una desarticulación coxofemoral del miembro inferior derecho. El estudio anatomopatológico reveló una lesión de 13,0 x 8,0 cm, compatible con un osteosarcoma de alto grado, que presentaba invasión linfovascular y una estadificación patológica pT3. Conclusión: Los osteosarcomas en pacientes idosos configuran una rara presentación de la neoplasia, siendo de fundamental importancia la divulgación de casos en la literatura para el establecimiento de conductas entre equipos. (AU)

RESUMO

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.

RESUMO

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.

RESUMO

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

RESUMO

In Uruguay, after cardiovascular diseases, cáncer ranks second as a cause of death. According to official data from the Ministry of Public Health in 2017, 24.6% of deaths were due to this disease. In our specialty, there is great concern about the evolutionary consequences of neoplastic disease related to the surgical anesthetic act, conceptualized from the length of the perioperative period. We consider necessary the inclusion of protocols for neoplastic surgery that favor recovery and evolution in the short and long term. This implies establishing anesthesiological strategies aimed at preserving immunity, by reducing psychological and surgical stress, adequate pharmacological selection, management of the internal environment and analgesia. For these reasons, it is important to establish the implementation of perioperative plans that reduce psychosomatic aggression, which in addition to medical stability, provide quality analgesia, with postoperative well-being that allow rest and recovery. Great advances have been made in tumor resection surgical techniques such as neoadjuvant and adjuvant polychemotherapy techniques, however, tumor recurrence rates have not been reduced as would be desired. Beyond the natural malignancy of the underlying pathology, it is inferred that surgical stress with a long and torpid perioperative period favors tumor recurrence. Hence our special interest in these communications related to patients in the search for solutions that improve their vital prognosis. Based on current evidence, it can be affirmed that the decrease in opiate consumption, especially the decrease in the need for Morphine, may favor better prognoses; maintaining the antimetastatic immune response in a period of special protumoral susceptibility such as the perioperative period. We therefore propose the use of analgesic / anesthetic techniques supported on the basis of regional anesthetic blocks prior to surgical aggression, complemented with the administration of Propofol, NSAIDs and p-blockers during the intraoperative period, through the presentation of 2 clinical cases of patients with osteosarcoma of knee.

En Uruguay, luego de las enfermedades cardiovasculares, el cáncer ocupa el segundo lugar como causa de muerte. Según datos oficiales del Ministerio de Salud Pública en el año 2017, el 24,6% de los fallecimientos fue por esta enfermedad. En nuestra especialidad, existe una gran preocupación por las consecuencias evolutivas de la enfermedad neoplásica relacionadas al acto anestésico quirurgico, conceptuado desde la amplitud del período perioperatorio. Consideramos necesaria la inclusión de protocolos para la cirugía neoplásica que favorezcan la recuperación y evolución tanto a corto como a largo plazo. Ello implica establecer estrategias anestésiologicas orientadas a conservar la inmunidad, mediante la disminución del estrés tanto psicológico como quirúrgico, la adecuada selección farmacológica, el manejo del medio interno y de la analgesia. Por estos motivos, es importante establecer la implementación de planes perioperatorios que dismimuyan la agresión psicosomática que, además de la estabilidad médica, aporten analgesia de calidad, con bienestar posoperatorio que permitan el descanso y la recuperación. Se han logrado grandes avances en las técnicas quirúrgicas de resección tumoral como en las técnicas de poliquimioterapia neoadyuvante y adyuvante, sin embargo, las tasas de recurrencia tumoral no se han reducido como se desearía. Más allá de la natural malignidad de la patología de base, se infiere que el estrés quirúrgico con un perioperatorio sufrido y tórpido favorece la recurrencia tumoral. De ahí nuestro especial interés de estas comunicaciones relativas a los pacientes en la búsqueda de soluciones que mejoren su pronóstico vital. En base a evidencia actual, se puede afirmar que la disminución del consumo de opiáceos, especialmente la disminución de la necesidad de morfina, puede resultar favorecedor en mejores pronósticos; manteniendo la respuesta inmune antimetastásica en un período de especial susceptibilidad protumoral como es el perioperatorio. Proponemos entonces la utilización de técnicas analgésicas/anestésicas soportadas en base a bloqueos anestésicos regionales previos a la agresión quirúrgica, complementados con la administración de Propofol, AINES y p bloqueantes durante el intraoperatorio, a través de la presentación de 2 casos clínicos de pacientes con osteosarcoma de rodilla.

RESUMO

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.

RESUMO

Although osteosarcoma is a rare disease, with a global incidence rate estimated at 5.0/million/year, it is the most frequent primary bone sarcoma in children and adolescents. In translational research, the patient-derived xenograft (PDX) model is considered an authentic in vivo model for several types of cancer, as tumorgrafts faithfully retain the biological characteristics of the primary tumors. Our goal was to investigate the association between PDX formation and clinical findings of osteosarcoma patients and the ability of the model to preserve in immunocompromized mice the characteristics of the parental tumor. A fresh sample of the patient tumor obtained from a representative biopsy or from surgical resection was implanted into nude mice. When tumor outgrowths reached ~1,500mm³, fresh PDX fragments were re-transplanted into new hosts. Engraftment in mice was obtained after a latency period of 19-225 days (median 92 days) in 40.54% of the implanted samples. We confirmed the histopathological fidelity between the patient tumor and their respective established PDXs, including the expression of biomarkers. PDX take rate was higher in surgical resection samples, in post-chemotherapy surgical samples and in samples from patients with metastatic disease at presentation. In conclusion, we have shown that the osteosarcoma PDX model reliably recapitulates the morphological aspects of the human disease after serial passage in mice. The observation that more aggressive forms of osteosarcoma, including those with metastatic disease at presentation, have a higher efficiency to generate PDXs provides a promising scenario to address several unanswered issues in clinical oncology.

RESUMO

PURPOSE: To compare the results for patients treated with intercalary endoprosthetic replacement (EPR) or intercalary allograft reconstruction for diaphyseal tumours of the femur in terms of: (1) reconstruction failure rates; (2) cause of failure; (3) risk of amputation of the limb; and (4) functional result. METHODS: Patients with bone sarcomas of the femoral diaphysis, treated with en bloc resection and reconstructed with an intercalary EPR or allograft, were reviewed. A total of 107 patients were included in the study (36 EPR and 71 intercalary allograft reconstruction). No differences were found between the two groups in terms of follow-up, age, gender and the use of adjuvant chemotherapy. RESULTS: The probability of failure for intercalary EPR was 36% at 5 years and 22% for allograft at 5 years (p = 0.26). Mechanical failures were the most prevalent in both types of reconstruction. Aseptic loosening and implant fracture are the main cause in the EPR group. For intercalary allograft reconstructions, fracture followed by nonunion was the most common complication. Ten-year risk of amputation after failure for both reconstructions was 3%. There were no differences between the groups in terms of the mean Musculoskeletal Tumor Society score (27.4, range 16-30 vs. 27.6, range 17-30). CONCLUSIONS: We have demonstrated similar failure rates for both reconstructions. In both techniques, mechanical failure was the most common complication with a low rate of limb amputation and good functional results. LEVEL OF EVIDENCE: Level III, therapeutic study.

RESUMO

INTRODUCTION: Primary bone tumors make up 1% of cancers in adults, the most common being osteosarcoma and chondrosarcoma. Giant cell tumor (GCT) is a locally aggressive benign neoplasm, accounting for 4-10% of all primary bone tumors, but in certain populations it accounts for up to 20%. The main objective of this work was to determine the proportion of GCTs, osteosarcomas and chondrosarcomas in a period of five years, and describe the characteristics of the patients from whom they come. Clinical implications: More information about the epidemiology of bone tumors is required to know their epidemiology in the Mexican population. MATERIAL AND METHODS: Descriptive observational study of a retrospective cross section including all patients who underwent surgery for tumoral resection, in public hospitals at national level, and whose histopathological pieces were processed and resulted in tissue compatible with a) GCT, b) osteosarcomas and c) chondrosarcomas. RESULTS: Between 2013 and 2017, 138 cases of the three primary bone tumors studied were reported, giant cell tumor (50%), osteosarcoma (25.36%) and chondrosarcoma (24.64%). The states with the highest number of cases were the state of Mexico (45.65%) and Mexico City (26.81%). Women had a higher prevalence (57.25%) than men (42.75%). The average age of presentation of the tumors was 36.80 years. CONCLUSIONS: GCT is not an uncommon tumor in the Mexican population, since it has its own characteristics.

INTRODUCCIÓN: Los tumores óseos primarios constituyen 1% de los cánceres en el adulto, siendo los más frecuentes el osteosarcoma y el condrosarcoma. La tumoración de células gigantes (TCG) es una neoplasia benigna localmente agresiva, que representa de 4-10% de todos los tumores óseos primarios, pero en ciertas poblaciones representa el hasta 20%. El objetivo principal de este trabajo fue determinar la proporción de los TCG, osteosarcomas y condrosarcomas en un período de cinco años y describir las características de las pacientes de los cuales proceden. Importancia clínica: se requieren más datos acerca de la epidemiología de los tumores de los huesos, para conocer su epidemiología en la población mexicana. MATERIAL Y MÉTODOS: Estudio observacional descriptivo de corte transversal retrospectivo incluyendo a la totalidad de pacientes que fueron intervenidos quirúrgicamente mediante resección tumoral, en los hospitales públicos a nivel nacional, y cuyas piezas histopatológicas fueron procesadas y dieron como resultado tejido compatible con: a) TCG, b) osteosarcomas y c) condrosarcomas. RESULTADOS: Entre el 2013 y 2017 se reportaron 138 casos de los tres tumores óseos primarios estudiados, el tumor de células gigantes (50%), el osteosarcoma (25.36%) y el condrosarcoma (24.64%). Los estados que tuvieron mayor número de incidencias fueron el Estado de México (45.65%) y la Ciudad de México (26.81%). Las mujeres presentaron una mayor prevalencia (57.25%) que los hombres (42.75%). La edad media de presentación de los tumores fue 36.80 años. CONCLUSIONES: El TCG no es una tumoración infrecuente en la población mexicana, ya que cuenta con características propias.

RESUMO

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.

RESUMO

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

RESUMO

miRNAs have been identified as key regulators of almost all cellular processes, therefore, their dysregulation is involved with several diseases, including cancer. miRNAs specifically related to the metastastic cascade are called metastamiRs and can be involved with different steps of this process, including loss of adhesion. Osteosarcoma (OS) is the most common primary malignant pediatric bone tumor that often presents metastatic disease at diagnosis; therefore, a deeper study of adhesion-associated miRNAs could shed light on its pathophysiology. Online databases were used to select four miRNAs (miR-139; miR-181b; miR-584; miR-708) predicted or validated to target proteins related to adherent junctions and focal adhesion pathways, and their expression levels and possible associations with clinical features evaluated in primary OS samples. Our results showed downregulation of miR-139-5p and miR-708-5p in OS samples compared to non-neoplastic controls. Moreover, lower expression of miR-708-5p was associated with poor overall survival and higher expression of miR-181b-5p related to worst chemotherapy response (low HUVOS level). Based on these results, we selected miR-139-5p and miR-708-5p for further functional testing. Inducing the expression of miR-139-5p diminished the clonogenic capacity of the HOS cell line, while upregulation of miR-708-5p was related to a lower cellular adhesion. In summary, this work identified new signatures of microRNA dysregulation that may serve as useful prognostic markers in this aggressive pediatric bone tumor.