Hallazgos tomográficos en paciente con osteosarcoma condroblástico mandibular recidivante de alto grado / Tomographic Findings in a Patient with Recurrent High - Grade Chondroblastic Osteosarcoma of the Jaw

Hallazgos de lesión expansiva que compromete la región hemifacial izquierda, con plano de clivaje sobre las estructuras adyacentes, que genera cambios líticos sobre la rama mandibular izquierda, en el cuerpo y la región del cóndilo mandibular. La lesión tiene un diámetro aproximado de 12,5 cm, con múltiples calcificaciones en su interior, que genera efecto de masa sobre las estructuras del piso de la boca y desvía la columna de la vía aérea en sentido dextroconvexo, sin evidenciar estenosis significativa(AU)

Mutation characteristics of osteosarcoma: a single center study of 64 cases using next-generation sequencing / 中华病理学杂志

Objective: To investigate the distribution and characteristics of gene mutations in osteosarcoma, and to analyze the frequency and types of detectable mutations, and to identify potential targets for individualized treatment of osteosarcoma. Methods: The fresh tissue or paraffin-embedded tissue samples of 64 cases of osteosarcoma that were surgically resected or biopsied and then subject to next generation sequencing, were collected from Beijing Jishuitan Hospital, China from November 2018 to December 2021. The tumor DNA was extracted to detect the somatic and germline mutations using targeted sequencing technology. Results: Among the 64 patients, 41 were males and 23 were females. The patient age ranged from 6 to 65 years with a median age of 17 years, including 36 children (under 18 years old) and 28 adults. There were 52 cases of conventional osteosarcoma, 3 cases of telangiectatic osteosarcoma, 7 cases of secondary osteosarcoma, and 2 cases of parosteosarcoma. The detection rate of gene mutations was overall 84.4% (54/64). There were 324 variations in 180 mutated genes, including 125 genes with copy number variations, 109 single nucleotide variants, 83 insertions or deletions, and 7 gene fusions. The most common mutated genes were TP53, VEGFA, CCND3, ATRX, MYC, RB1, PTEN, GLI1, CDK4 and PTPRD. Among them, TP53 had the highest mutation rate (21/64, 32.8%), single nucleotide variant was the main mutation type (14/23, 60.9%), and 2 cases carried the TP53 germline mutation. VEGFA and CCND3 showed copy number amplification simultaneously in 7 cases. Conclusions: The high-frequency mutation of TP53 suggests that it plays an important role in the pathogenesis and development of osteosarcoma. VEGFA, CCND3 and ATRX are mutated genes in osteosarcoma and worthy of further studies. Combination of pathologic diagnosis and next generation sequencing with clinical practice can guide individualized treatment for patients with refractory, recurrent and metastatic osteosarcoma.

Prognostic value and mechanism of long non-coding RNA DLEU1 in osteosarcoma / 中国骨伤

OBJECTIVE@#To investigate the prognostic value and mechanism of long non-coding RNA DLEU1(LncRNA DLEU1) in osteosarcoma.@*METHODS@#The tissue samples and clinical data of 86 patients with osteosarcoma treated by orthopaedic surgery in our hospital from January 2012 to December 2014 were retrospectively collected. The expression of LncRNA DLEU1 in pathological tissues was detected by qRT-PCR, then the patients were divided into high and low expression of LncRNA DLEU1 groups. Osteosarcoma cell line HOS was divided into two groups, down-regulated expression group (si-DLEU1 group) and negative control group (si-NC group). LncRNA DLEU1 siRNA and negative control sequence were transfected by Lipofectamine 3000. Chi-square test was used to analyze the relationship between the expression of LncRNA DLEU1 and the clinicopathological factors of osteosarcoma. Kaplan-Meier method was used to compare the difference of the overall survival rate of osteosarcoma patients between the high and low expression groups of LncRNA DLEU1. The risk factors affecting the overall survival rate of osteosarcoma were analyzed by single factor and multifactor analysis. The number of invasive cells in the two groups was determined and compared by Transwell assay.@*RESULTS@#The expression of LncRNA DLEU1 in osteosarcoma tissue was higher than that in adjacent tissues (P<0.001). The expression of LncRNA DLEU1 in human osteosarcoma cell lines (MG-63, U-2 OS, and HOS) was significantly higher than that in human osteoblast line hFOB 1.19 (P<0.001). The expression of LncRNA DLEU1 was significantly correlated with Enneking stage (P<0.001), distant metastasis (P=0.016), and histological grade (P=0.028). The 1-year overall survival rate of the LncRNA DLEU1 high expression group was significantly higher than that of the low expression group (90.7% vs 60.5%, P<0.001). The 5-year overall survival rate of the LncRNA DLEU1 high expression group was significantly higher than that of the low expression group (32.6% vs 11.6%, P<0.001). Univariate analysis showed that Enneking stage (P<0.001), tumor size (P=0.043), distant metastasis (P<0.001), histological grade (P<0.001), and expression of LncRNA DLEU1 (P<0.001) were risk factors for overall survival of osteosarcoma patients. Multivariate analysis showed that high expression of LncRNA DLEU1 [HR=1.948, 95% CI(1.141, 3.641), P=0.012] and distant metastasis[HR=4.108, 95% CI(2.169, 7.780), P<0.001] were independent risk factors for overall survival of osteosarcoma patients. The number of invasive cells in si-DLEU1 group was significantly lesser than that in si-NC group(139±13 vs 357±31, P<0.001).@*CONCLUSION@#High expression of LncRNA DLEU1 is a molecular marker affecting the prognosis of osteosarcoma patients. Downregulation of LncRNA DLEU1 can inhibit the invasion of osteosarcoma cells.

miR-125b-5p inhibits proliferation and migration of osteosarcoma cells by negatively regulating RAB3D expression / 南方医科大学学报

OBJECTIVE@#To investigate the inhibitory effect of miR-125b-5p on proliferation and migration of osteosarcoma and the role of RAB3D in mediating this effect.@*METHODS@#The expression level of miR-125b-5p was detected by qRT-PCR in a normal bone cell line (hFOB1.19) and in two osteosarcoma OS cell lines (MG63 and HOS). A miR-125b-5p mimic or inhibitor was transfected in the osteosarcoma cell lines via liposome and the changes in cell proliferation and migration were detected with EDU and Transwell experiments. Bioinformatic analysis was conducted for predicting the target gene of miR-125b-5p, and the expression level of RAB3D in hFOB1.19, MG63, and HOS cells was detected by Western blotting. In the two osteosarcoma cell lines transfected with miR-125b-5p mimic or inhibitor, the expression levels of RAB3D mRNA and protein in osteosarcoma cells were examined with qRT-PCR and Western blotting. The effects of RAB3D overexpression, RAB3D knockdown, or overexpression of both miR-125b-5p and RAB3D on the proliferation and migration of cells were assessed using EDU and Transwell experiments.@*RESULTS@#The two osteosarcoma cell lines had significantly lower expression levels of miR-125b-5p (P < 0.05). Bioinformatic analysis predicted that RAB3D was a possible target gene regulated by miR-125b-5p. In osteosarcoma cells, overexpression of miR-125b-5p significantly lowered the expression of RAB3D protein (P < 0.05); inhibiting miR-125b-5p expression significantly decreased RAB3D expression only at the protein level (P < 0.05) without obviously affecting its mRNA level. Modulation of miR-125b-5p and RAB3D levels produced opposite effects on proliferation and migration of osteosarcoma cells, and in cells with overexpression of both miR-125b-5p and RAB3D, the effect of RAB3D on cell proliferation and migration was blocked by miR-125b-5p overexpression (P < 0.05).@*CONCLUSION@#Overexpression of miR-125b-5p inhibits the proliferation and migration of osteosarcoma cells by regulating the expression of RAB3D at the post-transcriptional level.

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

Jaw osteosarcoma and pregnancy: a rare coexistence

Osteosarcoma of the jaw represents less than 1% of all head and neck malignancies. This malignancy in pregnant women occurs in one per 1000 deliveries. We report a case of a 29-year-old woman, in the 33rd week of gestation, who presented with an expansive tumor destroying the maxillary alveolar bone, histologically composed of pleomorphic, round, spindle, or epithelioid cells and osteoid/chondroid matrix. Upon final diagnosis of osteosarcoma, the lesion was excised. To the best of our knowledge, only 10 cases of jaw osteosarcoma in pregnant women have been reported to date in the English language literature. The use of ancillary examinations, malignancy diagnosis, and cancer treatment can be challenging during pregnancy. Knowledge about jaw osteosarcoma in pregnancy can increase healthcare providers' awareness, avoid delays and misdiagnosis and potentially improve maternal and neonatal outcomes.

Bioinformatics analysis of differently expressed genes in osteoblastic sarcoma and screening of key genes / 中华肿瘤杂志

Objective: To screen the different expressed genes between osteosarcoma and normal osteoblasts, and find the key genes for the occurrence and development of osteosarcoma. Methods: The gene expression dataset GSE33382 of normal osteoblasts and osteosarcoma was obtained from Gene Expression Omnibus (GEO) database. The different expressed genes between normal osteoblasts and osteosarcoma were screened by limma package of R language, and the different expressed genes were analyzed by Kyoto encyclopedia of genes and genomes (KEGG) pathway enrichment analysis. The protein interaction network was constructed by the String database, and the network modules in the interaction network were screened by the molecular complex detection (MCODE) plug-in of Cytoscape software. The different expressed genes contained in the first three main modules screened by MCODE were analyzed by gene ontology (GO) using the BiNGO module of Cytoscape software. The MCC algorithm was used to screen the top 10 key genes in the protein interaction network. The gene expression and survival dataset GSE39055 of osteosarcoma was obtained from GEO database, and the survival analysis was performed by Kaplan-Meier method. The data of 48 patients with osteosarcoma treated in the First Affiliated Hospital of Fujian Medical University from January 2005 to December 2015 were selected for verification. The expression of STC2 protein in osteosarcoma was detected by immunohistochemical method, and the survival analysis was carried out combined with the clinical data of the patients. Results: A total of 874 different expressed genes were identified from GSE33382 dataset, including 402 down-regulated genes and 472 up-regulated genes. KEGG enrichment analysis showed that different expressed genes were mainly related to p53 signal pathway, glutathione metabolism, extracellular matrix receptor interaction, cell adhesion molecules, folate tolerance, and cell senescence. The top 10 key genes in the interaction network were GAS6, IL6, RCN1, MXRA8, STC2, EVA1A, PNPLA2, CYR61, SPARCL1 and FSTL3. STC2 was related to the survival rate of patients with osteosarcoma (P<0.05). The results showed that the expression of STC2 protein was related to tumor size and Enneking stage in 48 cases of osteosarcoma. The median survival time of 25 cases with STC2 high expression was 21.4 months, and that of 23 cases with STC2 low expression was 65.4 months. The survival rate of patients with high expression of STC2 was lower than that of patients with low expression of STC2 (P<0.05). Conclusions: Bioinformatics analysis can effectively screen the different expressed genes between osteosarcoma and normal osteoblasts. STC2 is one of the important predictors for the prognosis of osteosarcoma.

A Bibliometric Analysis and Visualization of Current Research Trends in Chinese Medicine for Osteosarcoma / 中国结合医学杂志

BACKGROUND@#To illustrate the research framework, overall knowledge structure, and development trends of Chinese medicine (CM) treatment for osteosarcoma (OS) by using a bibliometric analysis and newly developed visualization tools.@*METHODS@#Research datasets were acquired from the Web of Science (WOS) database from January 1, 1980 to September 30, 2019. VOS viewer and Citespace software was used to analyze the data and generate visualization knowledge maps. Annual trends of publications, distribution of institutes, distribution of journals, citation and H-index status, co-authorship status, research hotspots and co-citation status were analyzed.@*RESULTS@#A total of 223 publications in the WOS database met the requirement. The number of published articles showed a rise but the citation frequency and the H-index of China were relatively low. The cooperation between the countries, institutes and authors were relatively weak. Most publications were basic researches. Most of the previous researches focused on basic mechanisms of CM in treating OS, and therapy and improvement of dosage form may become a frontier in this research field.@*CONCLUSIONS@#Compared with other fields, the field of CM treatment for osteosarcome is still in infancy. The distribution of researches is imbalanced and cooperation between countries, institutions and authors remains to be strengthened. Furthermore, basic research occupies an absolute dominant position, and the exploration of the molecular mechanism of CM in preventing and treating OS may become a key point in the future.

Bioinformatics-based identification of key genes CDC5L and related pathways in osteosarcoma and Ewing's sarcoma / 中国骨伤

OBJECTIVE@#Osteosarcoma(OS) and Ewing's sarcoma (EWS) are the two most common primary malignant bone tumors in children. The aim of the study was to identify key genes in OS and EWS and investigate their potential pathways.@*METHODS@#Expression profiling (GSE16088 and GSE45544) were obtained from GEO DataSets. Differentially expressed genes were identified using GEO2R and key genes involved in the occurrence of both OS and EWS were selected using venn diagram. Gene ontology and pathway enrichment analyses were performed for the ensembl. Protein-protein interaction (PPI) networks were established by STRING. Further, UCSC was used to predict the transcription factors of the cell division cycke 5-like(CDC5L) gene, and GEPIA was used to analyze the correlation between the transcription factors and the CDC5L gene.@*RESULTS@#The results showed that CDC5L gene was the key gene involved in the pathogenesis of OS and EWS. The gene is mainly involved in mitosis, and is related to RNA metabolism, processing of capped intron-containing pre-mRNA, mRNA and pre-mRNA splicing.@*CONCLUSION@#CDC5L, as a key gene, plays a role in development of OS and EWS, which may be reliable targets for diagnosis and treatment of these primary malignant tumors.

Bioinformatics Analysis on Key Genes and Immune Infiltration of Osteosarcoma / 中国医学科学院学报

Objective To screen the potential key genes of osteosarcoma by bioinformatics methods and analyze their immune infiltration patterns. Methods The gene expression profiles GSE16088 and GSE12865 associated with osteosarcoma were obtained from the Gene Expression Omnibus(GEO),and the differentially expressed genes(DEGs)related to osteosarcoma were screened by bioinformatics tools.Gene Ontology(GO)annotation,Kyoto Encyclopedia of Genes and Genomes(KEGG)pathway enrichment,and analysis of immune cell infiltration were then carried out for the DEGs.The potential Hub genes of osteosarcoma were identified by protein-protein interaction network,and the expression of Hub genes in osteosarcoma and normal tissue samples was verified via the Cancer Genome Atlas(TCGA). Results A total of 108 DEGs were screened out.GO annotation and KEGG pathway enrichment revealed that the DEGs were mainly involved in integrin binding,extracellular matrix (ECM) structural components,ECM receptor interactions,and phosphatidylinositol 3-kinase/protein kinase B(PI3K/Akt)signaling pathway.Macrophages were the predominant infiltrating immune cells in osteosarcoma.Secreted phosphoprotein 1(SPP1),matrix metallopeptidase 2(MMP2),lysyl oxidase(LOX),collagen type V alpha(II)chain(COL5A2),and melanoma cell adhesion molecule(MCAM)presented differential expression between osteosarcoma and normal tissue samples(all P<0.05). Conclusions SPP1,MMP2,LOX,COL5A2,and MCAM are all up-regulated in osteosarcoma,which may serve as potential biomarkers of osteosarcoma.Macrophages are the key infiltrating immune cells in osteosarcoma,which may provide new perspectives for the treatment of osteosarcoma.

GKK1032B from endophytic Penicillium citrinum induces the apoptosis of human osteosarcoma MG63 cells through caspase pathway activation / 中国天然药物

Chemical investigation of the culture extract of an endophytic Penicillium citrinum from Dendrobium officinale, afforded nine citrinin derivatives (1-9) and one peptide-polyketide hybrid GKK1032B (10). The structures of these compounds were determined by spectroscopic methods. The absolute configurations of 1 and 2 were determined for the first time by calculation of electronic circular dichroism (ECD) data. Among them, GKK1032B (10) showed significant cytotoxicity against human osteosarcoma cell line MG63 with an IC50 value of 3.49 μmol·L-1, and a primary mechanistic study revealed that it induced the apoptosis of MG63 cellsvia caspase pathway activation.

Reconstruction with a Custom made Prosthetic Wrist Arthrodesis after Bone Tumor Resections of the Distal Radius. Single Centre Experience / Reconstrução com implante metálico personalizado de artrodese após ressecções de tumor ósseo do rádio distal. Experiência em um único centro

Abstract Objective The present study aimed at analyzing the clinical, radiological and functional results of the reconstruction of the distal radius after tumor resection with a custom-made metal arthrodesis implant and compare them with other types of distal radius reconstruction, as presented in the literature. To our best knowledge, this is the first article describing this particular type of implant and patient functionality. Methods Functional outcomes of reconstruction of the distal radius were assessed in a series of 4 patients. Three of the patients having had resection of giant cell tumors (GCTs), one patient having had resection of osteosarcoma. Results There were no major implant-related complications like infection, nonunion or loosening. Two patients had to undergo further surgery for protruding metalwork. Overall function was good according to the Musculoskeletal Tumor Society MSTS and Disabilities of the Arm, Shoulder, and Hand (DASH) scores. Conclusion The present study shows that custom-made metal arthrodesis implant benefits from the fact that it can be used as a salvage option when other treatments have failed, or it can be used as a primary option in cases in which there is limited bone stock after distal radius tumor resection.

Resumo Objetivo O presente estudo teve como objetivo analisar os resultados clínicos, radiológicos e funcionais da reconstrução do rádio distal após a ressecção do tumor com implante metálico personalizado de artrodese e compará-los com outros tipos de reconstrução do rádio distal, conforme apresentado na literatura. Pelo que conhecemos, este é o primeiro artigo descrevendo esse tipo particular de implante e funcionalidade no paciente. Métodos Os desfechos funcionais de reconstrução do rádio distal foram avaliados em uma série de 4 pacientes. Três dos pacientes tiveram ressecção de tumores de células gigantes (TCGs), sendo um paciente com ressecção de osteossarcoma. Resultados Não houve complicações relacionadas ao implante, como infecção, não sindicalidade ou afrouxamento. Dois pacientes tiveram que passar por uma nova cirurgia para a protusão da prótese metálica. A função geral foi boa de acordo com as pontuações da Musculoskeletal Tumor Society (MSTS) e Disabilities of the Arm, Shoulder, and Hand (DASH). Conclusão O estudo mostra que o implante metálico personalizado de artrodese se beneficia do fato de que pode ser usado como opção de salvamento quando outros tratamentos falharam, ou pode ser usado como opção primária nos casos em que há estoque ósseo limitado após a ressecção do tumor do rádio distal.

Fibroma ossificante trabecular juvenil: um relato de caso / Juvenile ossifying fibroma: a case report

Objetivo: o fibroma ossificante trabecular juvenil (FOJT) é uma lesão fibro-óssea incomum, benigna, porém de comportamento agressivo. Ocorre, frequentemente, em crianças e adolescentes, com maior acometimento em maxila e mandíbula. O presente relato aborda os aspectos clínicos, imaginológicos, histopatológicos, imuno-histoquímicos e o tratamento de um caso de FOTJ. Além de discutir suas similaridades com o osteossarcoma de baixo grau e as alternativas para o seu diagnóstico. Relato de caso: paciente do sexo masculino, com 12 anos de idade, apresentando aumento de volume em mandíbula no lado esquerdo, perda de função mastigatória e de fala, com tempo de evolução de seis meses. Radiograficamente, a lesão apresentava aspecto unilocular, com limites definidos e de padrão misto. O tratamento consistiu em cirurgia de enucleação total da lesão, seguida de curetagem sob anestesia geral. A histopatologia e a ausência de marcação para MDM2 e CDK4 na imuno-histoquímica confirmaram o diagnóstico de FOTJ e excluíram o de osteossarcoma de baixo grau. No controle pós-operatório de 20 dias, o paciente relatou melhora da parestesia, da função mastigatória e da fala e ausência de dor. Não se observou recidiva no acompanhamento de 1 ano após a cirurgia. Considerações finais: a similaridade dos aspectos clínico, imaginológico e microscópico entre FOJT e osteossarcoma de baixo grau pressupõe o estudo imuno-histoquímico com os arcadores MDM2 e CDK4, minimizando equívocos no diagnóstico e orientando de forma mais eficiente o plano de tratamento adotado.(AU)

Objective: juvenile Trabecular Ossifying Fibromas (JTOF) are uncommon benign fibro-osseous lesions with a local aggressive behavior. They frequently occur in children and adolescents involving the jaw bones. This report addresses the clinical, imaging, histopathological, and immunohistochemical aspects and the treatment of a case of JTOF. In addition, it discusses similarities between JTOF and low-grade osteosarcomas and alternatives for their diagnosis. Case report: male patient, 12 years old, with a tumor in his left mandible, loss of masticatory function and speech, with a 6-month time course. Radiographically, the lesion had an unilocular aspect, with defined borders and a mixed image pattern. Treatment consisted surgical enucleation, followed by bone curettage, under general anesthesia. Histopathology presented fibrous stroma interspaced with bone trabeculae without signs of cellular malignancy. MDM2 and CDK4 markers in immunohistochemistry, confirmed the diagnosis of JTOF and excluded low-grade osteosarcoma. At the 20-day postoperative follow-up, the patient reported improvement in paresthesia, masticatory function and speech, and the absence of pain. Evaluation 1 year after surgery showed no signs of local recurrence. Final considerations: the similarity of clinical, imaging, and microscopic aspects between JTOF and low-grade osteosarcoma prompts an immunohistochemical study including MDM2 and CDK4 markers in order to improve final diagnosis and guide the treatment plan more efficiently.(AU)

Osteosarcoma of the hard palate in a dog - case report / [Osteossarcoma em palato duro de cão - relato de caso]

The osteosarcoma (OSA) is the most diagnosed primary bone cancer in canine patients. This work reports a case of a canine, six years old, mongrel, female, intact, with an OSA in the hard palate. Physical examination detected a firm mass in the palate. Thoracic radiographs, hematological and biochemical exams, histopathological exams and computed tomography were requested. A chondroblastic OSA was diagnosed and the tumor was characterized by immunohistochemistry. There was never evidence of metastasis in this case. The treatment consisted of the combination of conventional chemotherapy, metronomic chemotherapy, and palliative care, aiming at greater survival and well-being of the patient since surgical excision was not possible due to the location and extension of the tumor. Osteogenic sarcomas of the hard palate are rarely seen and described in the literature. In this article we present a characterization of the osteosarcoma with uncommon localization in the hard palate.(AU)

O osteossarcoma (OSA) é a neoplasia óssea primária mais diagnosticada em pacientes caninos. Este trabalho relata o caso de um canino, com seis anos de idade, sem raça definida, fêmea, não castrado, apresentando OSA em região de palato duro. Ao exame clínico, constatou-se uma massa de consistência firme em região palatina. Além do exame clínico, foram solicitadas radiografias torácicas, exames hematológicos e bioquímicos, exames histopatológicos e tomografia computadorizada. Fora então dado o diagnóstico de OSA condroblástico, e o tumor foi caracterizado pela imuno-histoquímica. Não foram observadas evidências de metástases nesse caso. O tratamento instituído consistiu na combinação de quimioterapia convencional, quimioterapia metronômica e cuidados paliativos, almejando maior sobrevida e bem-estar do paciente, uma vez que a excisão cirúrgica não foi possível devido à localização e à extensão do tumor. Neste artigo, apresentou-se uma caracterização do osteossarcoma com localização incomum no palato duro.(AU)

Five Osteosarcoma of the lower extremity treated by Rotationplasty: University of the Philippines - Philippine General Hospital Experience

@#Functional and oncologic results of the initial series of children with osteosarcoma treated with rotationplasty at the University of the Philippines - Philippine General Hospital from 2014 to 2015 are reported. Five children (mean 13.2 years) with Enneking IIB osteosarcoma of the lower extremity underwent neoadjuvant chemotherapy, tumor resection with wide margins, and rotationplasty – four Winkelmann Type AI and one Type AII. The four Type-A cases were fixed using an intramedullary nail and the Type A2 case with a plate. Two cases had >90% tumor necrosis. Adjuvant chemotherapy was started 10 to 21 days following surgery. Functional results for the AI rotationplasty showed a good range of motion of the ankle (neo-knee) with preserved muscle strength. The patient with AII rotationplasty had a fair range of motion of the ankle with some muscle weakness. All had radiographic evidence of healing at an average of 12 weeks. The oncologic outcome showed two with local recurrences at five months from surgery; three died of disease at 7, 20, and 38 months; while two are alive without evidence of disease at 72 and 84 months. Three patients were able to fit with a prosthesis with good utility, ambulation status, and satisfaction.