ABSTRACT
Abstract The evolution in imaging evaluation of musculoskeletal sarcomas contributed to a significant improvement in the prognosis and survival of patients with these neoplasms. The precise characterization of these lesions, using the most appropriate imaging modalities to each clinical condition presented, is of paramount importance in the design of the therapeutic approach to be instituted, with a direct impact on clinical outcomes. The present article seeks to update the reader regarding imaging methodologies in the context of local and systemic evaluation of bone sarcomas and soft tissues.
Resumo A evolução na avaliação por imagens dos sarcomas musculoesqueléticos contribuiu para melhora significativa no prognóstico e na sobrevida dos portadores destas neoplasias. A caracterização precisa destas lesões, mediante utilização das modalidades de imagem mais adequadas a cada condição clínica apresentada, é de suma importância no delineamento da abordagem terapêutica a ser instituída, com impacto direto sobre os desfechos clínicos. O presente artigo busca atualizar o leitor a propósito das metodologias de imagem no contexto da avaliação local e sistêmica dos sarcomas ósseos e das partes moles.
Subject(s)
Humans , Sarcoma/radiotherapy , Diagnostic Imaging , Neoplasms, Bone Tissue/diagnosis , Multimodal ImagingABSTRACT
El tumor mesenquimático fosfatúrico es una entidad clinicopatológica sumamente infrecuente. Además de provocar dolor óseo insidioso y polimialgias, se acompaña de alteraciones del metabolismo fosfocálcico de difícil manejo clínico. El abordaje multidisciplinario resulta la clave del éxito en esta enfermedad. Presentamos una paciente de 52 años de edad con antecedente de tumor mesenquimático fosfatúrico en la hemipelvis derecha con extensión a la cadera homolateral de 10 años de evolución. Clínicamente presentaba osteomalacia oncogénica (hipofosfatemia e hiperfosfaturia) que no se corregía, pese a un agente de última generación, el burosumab, un inhibidor del factor de crecimiento fibroblástico 23, que aumenta la reabsorción tubular renal de fosfatos. En un comité multidisciplinario, se decidió la resección con márgenes oncológicos y se logró una mejoría clínica franca. Comunicamos este caso, debido a que es un cuadro infrecuente. Nivel de Evidencia: IV
Phosphaturic mesenchymal tumor (PMT) is an infrequent clinicopathological entity. It presents insidious bone pain and polymyalgia, accompanied by alterations in calcium and phosphorus metabolism that are difficult to resolve clinically. A multidisciplinary approach is a key to success in this pathology. We present the case of a 52-year-old female patient with a 10-year history of PMT in the right hemipelvis with ipsilateral hip extension. From the clinical point of view, she presented oncogenic osteomalacia (hypophosphatemia and hyperphosphaturia) that did not correct despite being administered the latest generation medication, burosumab, an FGF-23 inhibitor that increases renal tubular phosphate reabsorption. Resection with oncological margins was decided by a multidisciplinary committee resolving her clinical condition. Due to the rarity of this pathology, we decided to report the case. Level of Evidence: IV
Subject(s)
Middle Aged , Osteomalacia , Pelvis/surgery , Pelvis/pathology , Neoplasms, Bone Tissue , Mesenchymoma/surgery , Neoplasms, Connective TissueABSTRACT
Abstract Bone and soft tissue sarcomas consist of a heterogeneous group of malignant tumors of mesenchymal origin that can affect patients from any age. The precise staging of these lesions determines the best therapeutic strategies and prognosis estimates. Two staging systems are the most frequently used: the system proposed by the University of Florida group, led by Dr. William F. Enneking (1980) and adopted by the Musculoskeletal Tumor Society (MSTS), and the system developed by the American Joint Committee on Cancer (AJCC) (1977), currently in its 8th edition (2017). This paper updates the reader on the staging of bone and soft tissue sarcomas affecting the musculoskeletal system.
Resumo Os sarcomas ósseos e das partes moles consistem em grupo heterogêneo de neoplasias malignas de origem mesenquimal que podem ocorrer em qualquer faixa etária. O estadiamento preciso destas lesões determina as melhores estratégias terapêuticas e estimativas de prognóstico. Dois sistemas de estadiamento são os mais frequentemente empregados no manejo destas neoplasias: o sistema proposto pelo grupo da Universidade da Flórida, liderado pelo Dr. William F. Enneking (1980), adotado pela Musculoskeletal Tumor Society (MSTS) e o sistema desenvolvido pela American Joint Committee on Cancer (AJCC) (1977) que se encontra em sua 8a edição (2017). O presente artigo busca atualizar o leitor a respeito do estadiamento dos sarcomas ósseos e das partes moles que acometem o sistema musculoesquelético.
Subject(s)
Humans , Pathology, Surgical , Sarcoma , Diagnostic Imaging , Neoplasms, Bone Tissue , Neoplasm Staging , Neoplasms, Connective TissueABSTRACT
Abstract Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissues arcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bones arcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.
Resumo Os sarcomas musculoesqueléticos são doenças raras que exigem atenção. Frequentemente, apresentam alto grau de malignidade ao diagnóstico e se subestimados podem evoluir de forma agressiva local e sistemicamente. Apresentam-se como sarcoma de partes moles e sarcomas ósseos, sendo os de partes moles quatro a cinco vezes mais comuns. A maioria dos sarcomas de partes moles ocorre nos membros. Os subtipos mais comuns nas crianças e adolescentes são o rabdomiossarcoma e o sarcoma sinovial, nos adultos o sarcoma pleomórfico indiferenciado, lipossarcoma, leiomiossarcoma, mixofibrossarcoma e sarcoma sinovial; todos de alto grau de malignidade histológica. Muitos sarcomas de partes moles são confundidos com tumores benignos de partes moles, 100 vezes mais comuns, por isso são ressecados sem o planejamento necessário, acarretando em amputação de um membro que poderia ter sido preservado. Como em todos os cânceres, o fator prognóstico mais importante é a doença metastática. Na sua vigência, a taxa de sobrevida global cai em torno de 20 a 30%. As taxas de sobrevida no geral são parecidas entre os sarcomas ósseos e de partes moles, portanto o sarcoma de partes moles, além de mais prevalente, mostra-se tão agressivo quanto os sarcomas ósseos, por isso merece muita atenção dos ortopedistas que frequentemente são a primeira linha de atendimento dos portadores destes tumores.
Subject(s)
Humans , Sarcoma/diagnosis , Sarcoma/therapy , Sarcoma/epidemiology , Neoplasms, Bone Tissue , Early Detection of Cancer , Neoplasm MetastasisABSTRACT
Introducción: El sarcoma de tejidos blandos (STB) constituye un grupo variado de más de 60 neoplasias que solo suponen 1 por ciento del total de los tumores. Objetivo: Describir el caso de un paciente con sarcoma de partes blandas con metástasis ósea y manifestaciones paraneoplásicas. Caso clínico: Se presenta el caso de un paciente masculino de 55 años de edad con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que refiere que hace 50 días inició con decaimiento marcado, luego de permanecer 1 mes con este síntoma inició con dolor en la columna en la parte más baja de la espalda, conjuntamente iniciando con un cuadro febril. Además, tuvo pérdida de la fuerza principalmente en las piernas, y hablaba cosas incoherentes sin sentido, gran inapetencia, con pérdida de peso de 50 lb en 20 días. Ante cuadro de pérdida de la conciencia y de la fuerza principalmente en las piernas se decidió su remisión hacia el Hospital Clínico Quirúrgico Lucía Íñiguez Landín de Holguín donde se concluyó como sarcoma de partes blandas con manifestaciones paraneoplásicas y metástasis ósea. Conclusiones: El caso presentado de sarcoma de partes blandas pretende aportar información sobre esta enfermedad tan infrecuente en nuestro medio(AU)
Introduction: Soft tissue sarcoma (STB) constitutes a varied group of more than 60 malignancies that only accounts for 1% of all tumors. Objective: To report a case of a patient with soft tissue sarcoma with bone metastases and paraneoplastic manifestations. Clinical case report: We report the case of a 55-year-old male patient with a history of type 2 diabetes mellitus and hypertension, who stated that 50 days ago he began to feel markedly listless. And after a month he began to suffer pain in the spine, in the lower back, and have a fever. In addition he had loss of strength mainly in the legs, and spoke inconsistently, nonsense, great loss of appetite, with weight loss of 50 lbs in 20 days. Given the condition of loss of consciousness and strength, mainly in the legs, it was decided to refer him to Lucía Iñiguez Landín Surgical Clinical Hospital in Holguín, where a soft tissue sarcoma with paraneoplastic manifestations and bone metastases was diagnosed. Conclusions: The report of this case of soft tissue sarcoma aims to provide information about this disease, which is so rare in our setting(AU)
Subject(s)
Humans , Male , Paraneoplastic Syndromes , Biopsy/methods , Sarcoma, Alveolar Soft Part/complications , Neoplasms, Bone Tissue/secondary , Leiomyosarcoma/diagnostic imagingABSTRACT
INTRODUCCIÓN: Los tumores óseos primarios son raros. La localización, extensión y el carácter lítico dificultan el tratamiento aunque el comportamiento biológico sea benigno. OBJETIVO: Valorar las características y tratamientos de los tumores óseos benignos primarios líticos activos o agresivos tratados en el Hospital El Cruce. Comparar la resección realizada con la sugerida por los criterios de Enneking. MATERIAL Y MÉTODOS: se evaluaron historias clínicas y ateneos postquirúrgicos de pacientes con diagnóstico de tumores músculoesqueléticos atendidos en el Servicio de Ortopedia y Traumatología del Hospital El Cruce desde agosto de 2008 hasta junio de 2017. Se utilizaron los criterios de Ennekingde comportamiento biológico y resección. RESULTADOS: Se evaluaron 26 pacientes con diagnóstico de tumor benigno lítico activo o agresivo sobre un total de 124 tumores musculoesqueléticos primarios operados. Un paciente se reclasificó como maligno en la resección completa, por lo que se consideraron 25. Se trató de 16 mujeres, 9 varones, edad media 28,4.La localización fue: 13 miembro inferior (1coxal, 7 fémur, 3 tibia y 2 calcáneo), 9 miembro superior (1 húmero, 2 radio, 2 cúbito, 3 metacarpianos y 2 falanges) y 3 columna (1 cervical, 1 torácica y 1 sacra). Un paciente tuvo localización múltiple en la mano. Según Enneking 10 eran activos y 13 agresivos. Histología: 12 Tumores de células gigantes (TCG), 5 condromas, 4 quiste óseo simple, 3 quiste óseos aneurismáticos y 1 hemangioma. La resección fue intralesional en 16, en 3 marginal y en 6 amplia (1 amputación). La media de días de estada fue 7 días (0 a 33), 5 internación en UTI. La reconstrucción se hizo con: Injerto de Banco 7, Injerto de cresta 7, Injerto M.sup 3, Prótesis 2, Peroné vascularizado 2, Sustituto óseo y cemento en 1. Sin reconstrucción en 2. Dos tuvieron adyuvancia con denosumab. Once tuvieron complicaciones. Hubo un óbito. En 18/25 la resección realizada coincidió con los criterios de resección (Enneking). CONCLUSIONES: A pesar de la benignidad histológica, los tumores líticos primarios requirieron procedimientos complejos, con un 40 % de complicaciones. En el 72% la resección realizada concordó con los criterios de resección de Enneking.
INTRODUCTION: Primary bone tumors are rare. The localization, extension and lytic character make the treatment difficult even though the biological behavior is benign. OBJECTIVES: Assess the characteristics and treatments of active or aggressive benign primary lytic bone tumors treated in the El Cruce hospital. Compare the resection made with the one suggested by Enneking criteria. MATERIALS AND METHODS: Clinical records and postsurgical grand rounds were evaluated on patients diagnosed with musculoskeletical tumors treated in the Orthopedics and Traumatology Service of Hospital El Crucefrom August 2008 to June 2017. Enneking's criteria based on biologic behavior and resection were used. RESULTS:Twenty-six patients diagnosed with active or aggressive lytic bone tumor were evaluated out of a total of 124 operated primary musculoskeletal tumors. A patient was reclassified as malignant on the complete resection, therefore 25 were considered. There were 16 women, 9 men; average age was 28.4.The tumor localization was: 13 in lower extremity (1 coxal, 7 femoral, 3 tibial and 2 in calcaneus), 9 in upper extremity (1 humeral, 2 radial, 2 ulnar, 3 metacarpal y 2 in phalanges) and 3 in spine (1 cervical, 1 thoracic y 1 sacral). A patient had multiple localizations in his hand. Based on Enneking, 10 were active and 13 were aggressive. Histology: 12 giant-cell tumors (GCT), 5 chondromas, 4 simple bone cysts, 3 aneurysmal bone cysts and 1 hemangioma. The resection was intralesional in 16, marginal in 3 and ample in 6 (1 amputation). Mean hospitalization stay was 7 days (0 to 33), 5 days in ICU. The reconstruction was made with: draft from banks in 7, drafts from crests in 7, draft from upper extremity in 3, prosthesis in 2, vascularized fibula in 2, bone substitute and cement in 1. No reconstruction in 2.Two had adjuvant therapy with denosumab. Eleven had complications. There was one death. In 18 out of 25, the resection matched the resection criteria (Enneking). CONCLUSIONS: Despite the histological benignity, primary lytic tumors required complex procedures and there were 40% complications. In72%, the resection matched the resection criteria (Enneking).
Subject(s)
Humans , Chondroma , Giant Cell Tumors , Hemangioma , Neoplasms, Bone Tissue , Neoplasms, Muscle Tissue , Treatment OutcomeABSTRACT
Las enfermedades malignas de cabeza y cuello en la población pediátrica son poco frecuentes. Considerando todos los tumores de cabeza y cuello, tanto benignos como malignos, éstos están representados por una frecuencia entre un 2% a 5% de todos los tumores pediátricos. A nivel de los maxilares, los tumores malignos en niños está representado por un rango que varía entre un 7% a 51%. En Chile, se estiman menos de 500 casos nuevos por año y en el rango entre los 5 y 15 años constituyen la segunda causa de muerte, precedida por los accidentes, traumatismos y violencias. Es importante considerar, al estudiar este grupo de enfermedades, que existen diferencias con los tumores de adultos, tanto en su incidencia, biología, comportamiento, histología y manejo. Es por esta razón que nos parece necesario realizar una revisión de la literatura científica para obtener un perfil del cáncer bucomaxilofacial infantil, que aporte en el entendimiento y aplicación de programas adecuados en el contexto de la salud bucomaxilofacial. Adicionalmente, buscamos actualizar el conocimiento de estas patologías, caracterizándolas en relación a su epidemiología, etiopatogenia y tratamiento, de manera que contribuya a los cirujanos dentistas para realizar diagnósticos y la pronta derivación a un centro especializado de patología oral. Para un estudio preciso, esta revisión constará de tres partes: la primera es sobre "tumores malignos de tejido óseo", mencionando, entre ellos, al osteosarcoma, sarcoma de Ewing, linfomas tanto Hodgkin como No Hodgkin, fibrosarcoma e histiocitosis de células de Langerhans.
Malignant head and neck disease in the pediatric population are rare. Considering all head and neck tumors, both benign and malignant, they represent between 2% to 5% of all pediatric tumors. At the level of the jaws, malignant tumors in children present in a range that varies from 7% to 51%. In Chile, fewer than 500 new cases are reported each year and in the 5 to 15 year-range are estimated to constitute the second cause of death, preceded by accidents, injuries and violence. When studying this group of diseases it is important to consider the differences with adult tumors, both in incidence, biology, behavior, histology and management. Therefore we believe it necessary to conduct a review of scientific literature to obtain a profile of child oral maxillofacial cancer, contributing to the understanding and implementation of appropriate programs in the context of maxillofacial health. In addition, we seek to update knowledge of these pathologies, characterizing their epidemiology, pathogenesis and treatment, so as to contribute to dental surgeons for diagnosis and early referrals to specialized centers for oral pathology. For a detailed study, this review will consist of three parts: the first is on "malignant bone tumors", included among these are: osteosarcoma, Ewing's sarcoma, Hodgkin's lymphoma, non-Hodgkin's lymphoma, Langerhans cell histiocytosis and fibrosarcoma.
Subject(s)
Humans , Child , Facial Neoplasms , Mouth Neoplasms , Maxillary Neoplasms , Neoplasms, Bone TissueABSTRACT
Fundamento: el sarcoma de Ewing es una neoplasia de alta malignidad que afecta a niños y adolescentes en mayor grado. R epresenta el 9 % de las neoplasias de hueso. La quimioterapia, radioterapia y cirugía combinadas o solitarias son los tratamientos disponibles. Objetivo: familiarizar a los residentes con una técnica quirúrgica poco utilizada de amputación interescapulotoráxica empleada en un paciente con diagnóstico de sarcoma de Ewing. Presentación del caso: e scolar femenina de 9 años de edad, con aumento de volumen del hombro izquierdo y tercio proximal del brazo ipsilateral de 12 semanas de evolución. La paciente presentó diagnóstico histopatológico de sarcoma de Ewing con infiltración al músculo deltoides. El tratamiento inicial consistió en quimioterapia por 10 semanas y después se le realizó tratamiento quirúrgico definitivo consistente en amputación interescapulotoráxica por vía posterior mediante la técnica de Littlewood. Conclusiones: la amputación interescapulotoráxica de Littlewood es un procedimiento que permite incrementar la supervivencia de los pacientes oncológicos, por lo que estar familiarizado con esta técnica es necesario en la formación de los especialistas en ortopedia y traumatología en la aplicación de la buena práctica médica.
Background: Ewing's sarcoma is a highly malignant neoplasm which mainly affects children and teenagers. It represents a 9 % of bone tumors. Chemotherapy, radiotherapy and surgery combined or alone are the available treatments. Objective: to familiarize residents with a less practiced surgical technique of forequarter amputation used in an Ewing's sarcoma case. Case presentation: a nine year old scholar female, complaining of volume increasing of the left shoulder and the proximal third of the ipsilateral arm of twelve weeks duration. The patient presented a histopathology diagnostic of Ewing's sarcoma with deltoid muscle infiltration. The early treatment included chemotherapy for ten weeks and afterwards a forequarter amputation was practiced by a posterior approach through Littlewood technique. Conclusions: Littlewood forequarter amputation is a procedure which allows increasing the survival, mainly in oncologic patients, that is why to be familiarized with the technique is necessary in the formation of orthopedics and traumatology specialists for good medical practice.
Subject(s)
Sarcoma, Ewing , Neoplasms, Bone Tissue , Amputation, Surgical/methodsABSTRACT
Los odontomas, tumores benignos odontogénicos, por lo general son hallazgos revelados durante la realización de técnicas de imagenología,para tratamientos variados.No obstante, la ausencia de una pieza dentaria, un tratamiento de ortodoncia, un aumento de volumen en el hueso maxilar, alertan desu existencia.En pocos casos, 23 con esta presentación, el odontoma erupciona hacia la cavidad bucal.La poca frecuencia de estas erupciones y, por lo general, de patologías concomitantes, demuestra la benignidad de estos tumores.No obstante, aún no se ha podido establecer el mecanismo de la erupción.El aporte de nuevos casos es importante para su dilucidación
Odontomas, benign odontogenic tumors, are usually revealed as radiografic findings during rutin examinations for various treatments.Nevertheless, the abscence of a tooth, an orthodontic treatment, an augmentation in the volume of maxillary/mandibulary bone, mayalert of its existance.In few cases, 23 with the ones presented here, the odontoma erupts into the oral cavity.The infrecuency of these eruptions and the fact that they are frecuently presented without any other sinthoms, shows the benignity ofthese lesions.However, the eruption mecanism has not yet been established.The contribution of new cases is important for its elucidation.
Subject(s)
Humans , Male , Female , Adolescent , Middle Aged , Odontoma/pathology , Odontoma , Odontogenic Tumors/pathology , Mouth/pathology , Neoplasms, Bone TissueABSTRACT
Introducción: los sarcomas son tumores malignos de partes blandas que representan 0,7 porciento de los tumores malignos en general, con una tasa de 3 por cada 100 000. El histiocitoma fibroso maligno resulta un tumor de alto grado incluido en el grupo de los sarcomas de partes blandas. Objetivo: exponer las características citohistológicas, el tratamiento y la evolución de este tumor en un caso estudiado y tratado en el Hospital General Provincial Docente Roberto Rodríguez Fernández de Ciego de Ávila. Descripción: se presentó el caso de un varón de 60 años con una gran masa de partes blandas en el muslo izquierdo, de crecimiento progresivo. La masa se trató quirúrgicamente con exéresis en bloque, previos estudios analíticos, arteriografía femoral y ecografía. Conclusiones: el diagnóstico anatomopatológico fue de histiocitoma fibroso maligno variedad pleomórfica; fue remitido al servicio de oncología donde lo trataron con radioterapia. A los 6 meses se encontraba libre de la enfermedad. Se realizó una revisión de la literatura y se analizó su presentación clínica, también los hallazgos histológicos, las pruebas diagnósticas de imagen y el procedimiento terapéutico
Introduction: the sarcomas are soft tissue malignant tumors accounting for the 0.7 percent of malignant tumors in general with a rate of 3 by 100 000. The malignant fibrous histiocytoma is a high grade tumor included in the group of soft tissue tumors. Objective: to expose the cytohistologic features, treatment and course of this tumor in study case and treated in Roberto Rodríguez Fernández Teaching Provincial Hospital of Ciego de Avila municipality. Description: this is the case of a male patient aged 60 presenting with an increasing large soft tissue mass in the left thigh. The mass was operated on with block exeresis, analytical previous studies, femoral arteriography and echography. Conclusions: the anatomic and pathologic diagnosis was a malignant fibrous histiocytoma pleomorphous type; the patient was referred to Oncology service where was treated with radiotherapy. At 6 months he was free of disease. A review of literature was performed analyzing its clinical presentation also, its histological findings, the imaging diagnostic test and the therapeutical procedure
Subject(s)
Humans , Male , Middle Aged , Histiocytoma, Malignant Fibrous/surgery , Histiocytoma, Malignant Fibrous/radiotherapy , Neoplasms, Bone Tissue/pathology , Case ReportsABSTRACT
Las metástasis acrales en huesos o partes blandas de manos o pies son localizaciones poco comunes de la diseminación a distancia de una enfermedad oncológica. El propósito de este trabajo es presentar el caso de un paciente con cáncer de esófago con metástasis acral en mano izquierda y una revisión de trabajos acerca de esta rara entidad.
Acral metastases in bone or soft parts of the hands or feet are rare entities of distant dissemination of an oncological disease. The purpose of this paper work is to present a patient case with esophageal cancer with metastases acral in his left hand and to revie the literature about the pathology.
Subject(s)
Humans , Male , Middle Aged , Carcinoma/complications , Finger Phalanges/pathology , Esophageal Neoplasms/complications , Neoplasms, Bone Tissue/secondaryABSTRACT
Objective. Classic high-grade osteosarcoma is uncommon in preadolescents (less than or equal to 10 years of age). The possibilities of clinicopathologic differences from the typical adolescent osteosarcoma patient have been raised. We sought to compare the presentation, treatment and survival of this subgroup of patients with published rates in order to determine if there is a need to use a treatment regimen different from that for regular adolescent osteosarcoma patients. Methods. Records of the University of the Philippines-Musculoskeletal Tumor Unit (UP-MuST) over a 15-year period (1993-2008) were reviewed and data collected on patients 10 years and younger with biopsy-proven classic high-grade intramedullary osteosarcoma who underwent complete treatment by the Unit. Demographics and survival rates were then compared with published rates for preadolescent and regular adolescent osteosarcoma cases. Results. There were fourteen patients; (6M:8F; age: 4-10 years). The most common presentation was a painful mass in the distal femur (8); the tumors most commonly had osteoblastic histology (12). Treatment consisted of neoadjuvant chemotherapy, wide surgical excision through ablation (9) or limb-saving surgery (5), and postoperative chemotherapy. There was a good histologic response (over 90% tumor necrosis) in four patients. Seven patients are ANED (alive no evidence of disease) 25 to 186 months after diagnosis. Five-year survival estimate is 52%, compared to a dismal 5 to 10% 15 years ago. Conclusion. Clinicopathologic presentation, clinical course, and overall survival in this subgroup of patients are comparable with published results for both preadolescent and adolescent osteosarcoma patients. There is no need to alter the present treatment regimen for this group of young patients.
Subject(s)
Humans , Male , Female , Child , Osteosarcoma , Therapeutics , Therapeutics , Neoplasms , Neoplasms by Histologic Type , Neoplasms, Connective and Soft Tissue , Neoplasms, Connective Tissue , Neoplasms, Bone TissueABSTRACT
Este artículo describe el caso de una mujer de 24 años de edad con un condroblastoma de localización pélvica, con una masa a la altura de la cresta iliaca derecha, de seis meses de evolución y crecimiento progresivo. Los rayos X mostraron una lesión osteolítica con densidad heterogénea y extensión hacia los tejidos blandos; el estudio histopatológico evidenció un condroblastoma. Los condroblastomas son tumores óseos benignos productores de cartílago que aparecen en las epífisis de los huesos largos de personas jóvenes. Alrededor del 75% de estos tumores afectan los huesos largos, principalmente el fémur, la tibia y el húmero; excepcionalmente se localizan en los huesos planos craneofaciales y en los huesos pélvicos. Los condroblastomas tienen características radiológicas e histopatológicas distintivas, y pese a su comportamiento biológico benigno, pueden causar una alta morbilidad para los pacientes, debido a su localización y a su tratamiento exclusivamente quirúrgico.
This article describes the case of a 24-year old woman with a pelvic chondroblastoma localized at the top of the right iliac crest, with six months of evolution and progressive growth. X-rays revealed an osteolytic lesion with heterogeneous density, extending toward soft tissue; the hisopathologic study provided evidence of chondroblastoma. Chondroblastomas are benign bone tumors producers of cartilage which appears in the long bone epiphysis of young people. Nearly 75% of such tumors affect the long bones, principally the femur, the tibia, and the humerus; exceptions include those in the flat craniofacial bones and the pelvis bones. Chondroblastomas have distinct radiological and histopathologic characteristics, and despite their benign biological behavior, can cause elevated morbidity among patients due to their localization and being treated exclusively with surgery.
Subject(s)
Humans , Female , Young Adult , Chondroblastoma/pathology , Epiphyses , Neoplasms, Bone Tissue , Pelvic Neoplasms , Surgical Procedures, Operative/methods , Surgical Procedures, Operative , ColombiaABSTRACT
Fueron evaluados 25 pacientes que acudieron a la Unidad de Tumores Óseos y Partes Blandas (UTO-PB) del Hospital "Manuel Núñez Tovar" entre enero de 2002 a enero de 2008, con fracturas de la región proximal del fémur, provocadas por lesiones metastásicas de carcinomas, predomino en el estudio el sexo masculino, las edades de los pacientes estuvieron por encima de los 40 años, con mayor numero en la década de los 50, el cáncer mas importante en el hombre fue el de próstata y en la mujer el de cuello uterino. En todos los procedimientos se seleccionó pacientes con expectativa de vida superior a 4 semanas y se persiguió como objetivo fundamental mejorar calidad de vida, aliviar dolor y reincorporar al paciente a su estilo de vida previo a sufrir la fractura. Se realizó artroplastia para lesiones cervicales y osteosíntesis con clavos intramedulares y sistemas de placas con tornillos deslizantes para la región subtrocantérica e intertrocantérica, obteniéndose mejores resultados funcionales con las artroplastias y menor índice de complicaciones con respecto al uso de clavos intramedulares y a los DCS, DHS.
We evaluated 25 patients who came to the Unit of Bone and soft tissue tumors (UTOPB) Hospital "Manuel Núñez Tovar" between january 2002 and january 2008, with fractures of the proximal femur, lessions caused by metastic carcinoma, predominance in the study males, ages of the patients were above age 40, with the highest number in the 50s, the most important cancer in men was prostate cancer and women in the cervical. All procedures were selected patients with life expectancy greater than 4 weeks and was pursued as a key objective to improve quality of life, alleviate pain and return patients to their lifestyle prior to suffering the fracture. Arthroplasty was performed for cervical lesions and osteosynthesis with intramedullary nails and screws systems with sliding plates for the region subtrocanterica and intertrocanterica, obtained better functional results with arthroplasties and lower complication rate with respect to the use of intramedullary nails and DCS, DHS.
Subject(s)
Humans , Male , Adult , Female , Arthroplasty/methods , Carcinoma/therapy , Femoral Fractures/diagnosis , Femoral Fractures/therapy , Neoplasm Metastasis/therapy , Neoplasms, Bone Tissue , Neoplasms, Bone Tissue/therapy , Soft Tissue Neoplasms/therapy , Medical Oncology , TraumatologyABSTRACT
OBJETIVO: Verificar se havia risco de contaminação por células neoplásicas no trajeto da biópsia e estudar as alterações histológicas locais. MÉTODOS: Realizou-se um estudo prospectivo com trajetos de biópsias de pacientes operados por tumores musculoesqueléticos malignos no Instituto de Ortopedia e Traumatologia da Faculdade de Medicina da Universidade de São Paulo no período de abril de 2006 a abril de 2007. Foram estudados por histopatologia 25 casos. RESULTADOS: Houve implante de células neoplásicas em 32 por cento dos trajetos. As alterações histológicas mais comuns nos casos positivos foram classificadas como: fibrose acentuada, componente inflamatório leve e neovascularização acentuada. CONCLUSÃO: Sugerimos a ressecção tradicional oncológica do trajeto junto com a peça.
OBJECTIVE: To investigate whether there is any risk of seeding by neoplasic cells along biopsy tracks and to study local histological abnormalities. METHODS: This was a prospective study on biopsy tracks in patients who underwent operations due to malignant musculoskeletal tumors between April 2006 and April 2007. RESULTS: Neoplasic cell implantation occurred in 32 percent of the tracks. The most common histological abnormalities in positive cases were classified as severe fibrosis, mild inflammatory component and severe neovascularization. CONCLUSION: We suggest that traditional oncological resection of the track should be carried out together with excision of the specimen.
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Infections , Neoplasms, Bone Tissue , Neoplasms, Bone Tissue/pathology , Soft Tissue Neoplasms , Sarcoma/diagnosis , Biopsy , Brazil , Histological Techniques , Prospective StudiesABSTRACT
La exostosis múltiple hereditaria es un trastorno autosómico dominante caracterizado por la aparición de osteocondromas que afectan generalmente al antebrazo, rodilla y tobillo. Estas masas pueden producir deformidades angulares a nivel de las extremidades y alteración mecánica de las articulaciones generando dolor e incapacidad funcional. Describimos el caso de un niño de 6 años de edad que debutó con genu valgo izquierdo patológico secundario a osteocondromatosis múltiple hereditaria y la corrección quirúrgica del mismo.
Subject(s)
Exostoses, Multiple Hereditary , Neoplasms, Bone Tissue , OsteochondromaSubject(s)
Humans , Male , Adult , Spine/physiopathology , Neoplasms, Bone Tissue , Osteoma, Osteoid/diagnosis , Diagnostic Imaging , Scoliosis , Tomography, X-Ray ComputedABSTRACT
Se presenta la experiencia del Servicio de Partes Blandas y Tumores Oseos del Hospital Oncológico Padre Machado, de Caracas, en el manejo de los tumores del esqueleto, por un período de 20 años (1984-2003). la evaluación de la experiencia fue retrospectiva, con un análisis objetivo estadístico lineal, presentándose el espectro de la patología atendida, y la diversidad de alternativas terapéuticas quirúrgicas utilizadas. Se reporta una incidencia preferencial de esta patología en los miembros inferiores (58,58 por ciento), particularmente alrededor de la rodilla (fémur distal y tibia proximal). El grupo de tumores más frecuentemente observado fue el de formadores de tejido óseo (20,95 por ciento, seguido de los formadores de tejido cartilaginoso (14,79 por ciento), tumor de células gigantes (9,93 por ciento), y las lesiones pseudotumorales (5,85 por ciento). Entre los tumores formadores de tejido óseo llama la atención la alta incidencia de las variedades malignas (90,52 por ciento); miemtras que en los formadores de tejido cartilaginoso, las variedades benignas fueron las más frecuentes (69,80 por ciento). Entre los tipos de cirugía realizadas, una proporción importante tuvo finalidad diagnóstica (58,41 por ciento), aunque en muchas de ellas la intención fue simultánea o adicionalmente terapéutica (escisión, drenaje, relleno, etc.). Entre las cirugías con finalidad terapéutica, hay una amplia diversidad de opciones, destacándose, la posibilidad de la práctica de una cirugía preservadora, siempre que se respeten los principios oncológicos quirúrgicos. se resalta la importancia del manejo de esta patología en centros espeializados.
The experience of the Service of Solft Tissue and Bone Tumors, of the Oncology Hospital Padre Machado, Caracas, in the management of the tumors affecting the skeleton, for a period of 20 years (1984-2003), is presented. The evaluation of this experience was a retrospective revision, with an objective statistics lineal analysis, showing the spectrum of the pathology, and the therapeutic surgical alternatives utilized. The report shows a higher incidence of this pathology affecting the lower limbs (58,58 per cent), particular..around the knee joint (distal femur and proximal tibia). The most frequent type of tumor was the bone forming tumors group (20.95 per cent), followed by the cartilaginous tissue forming tumors group (14.79 per cent, giant cell tumors (9.93 per cent), and pseudotumoral lesions (5.85 per cent). Among the bone forming tumor group, called our attention the prevalence of the malignant variety (90.52 per cent); whereas in the cartilaginous tissue forming type, the bening options were seen more frequently (69.80 per cent). Looking at the performed surgeries, an important proportion had a diagnostic goal (58.41 per cent), although many of them allowed a therapeutic solution at the same time (resection, drainage, folling defects, etc). Among the surgical options with a therapeutic goal, we performed a very large variety of them, proposing limb presenvation when the surgical oncology principles could be respected. The importance of treating this pathology in specialized centers is underlined.
Subject(s)
Humans , Male , Female , Medicine , Neoplasms, Bone Tissue/surgery , Neoplasms, Bone Tissue/pathology , Osteochondroma/surgery , Osteochondroma/pathology , Biopsy/methods , Giant Cells/physiology , Orthopedic Equipment , Skeleton , Medical OncologyABSTRACT
OBJETIVO: No grupo pediátrico, o diagnóstico radiológico dos tumores dos ossos ilíacos, ísquios e púbis apresenta dificuldades e peculiaridades próprias, mas a literatura revisada não trata especificamente desse tema. Este trabalho pretende investigar a existência de padrões radiológicos confiáveis para o diagnóstico diferencial desses tumores. MATERIAIS E MÉTODOS: Foram revistos os achados radiológicos de tumores dos ossos do quadril em dez pacientes com idades entre 8 e 19 anos. RESULTADOS: Reação óssea (esclerose ou lise), reação periosteal (lamelar em camada única, múltiplas camadas ou radial), extensão do tumor no osso e grau de invasão das partes moles revelaram baixa especificidade. As calcificações nas partes moles, consideradas em conjunto, foram inespecíficas. Contudo, separando as próximas do osso comprometido, que apresentam formas e tamanhos variados - padrão I -, daquelas afastadas do osso, finas e amorfas - padrão II -, observamos que o padrão I foi totalmente inespecífico e o padrão II foi identificado nos três casos de osteossarcoma (100 por cento) e em apenas um dos casos de Ewing (16,6 por cento). CONCLUSÃO: Neste material, as calcificações padrão II revelaram sensibilidade de 100 por cento e especificidade de 90 por cento para osteossarcoma. Contudo, sua importância pode não se limitar ao diagnóstico radiológico. As calcificações padrão II indicam, provavelmente, os sítios ideais para biópsia.
OBJECTIVE: In the pediatric group, the radiological diagnosis of bone tumors of the hip is difficult and presents some peculiarities, but reviewed literature does not approach this specific problem. The objective of the present study was to investigate the existence of reliable radiological patterns for the differential diagnosis of these tumors. MATERIALS AND METHODS: Radiological findings of bone tumors of the hip in ten patients in the age range between 8 and 19 years have been reviewed. RESULTS: Bone reaction (sclerosis or lysis), periosteal reaction (lamellar with single or multiple layers, or radial), tumor extent in the bone and level of soft tissues invasion have presented low specificity. Soft tissue calcifications, when considered as a whole, were non-specific. However, when those calcifications with varied shapes and sizes, nearby the affected bone (pattern I) were separated from those, thin and amorphous, away from the bone (pattern II), we have observed that the pattern I was totally non-specific, and the pattern II was found in the three cases of osteosarcoma (100 percent) and in only one case of Ewing's sarcoma (16.6 percent). CONCLUSION: In the present study, pattern II calcifications have shown a 100 percent sensitivity and 90 percent specificity for osteosarcoma. However, their importance may be not limited to the radiological diagnosis. Pattern II calcifications indicate probably ideal sites for biopsy.