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Deutsches Arzteblatt International Jun 2023Osteosarcoma and Ewing's sarcoma in children and adolescents require age-specific interdisciplinary diagnosis and treatment to achieve optimal therapeutic outcomes.
BACKGROUND
Osteosarcoma and Ewing's sarcoma in children and adolescents require age-specific interdisciplinary diagnosis and treatment to achieve optimal therapeutic outcomes.
METHODS
The diagnosis and treatment of malignant bone tumors in childhood and adolescence are presented in the light of publications retrieved by a selective search, pertinent guidelines, and the authors' extensive experience in an interdisciplinary cancer center.
RESULTS
Bone sarcomas make up approximately 5% of all malignancies in children and adolescents; the most common types are Ewing's sarcoma and osteosarcoma. Patients are often not referred to a specialized center until long after the onset of symptoms, as they and their physicians rarely consider the possibility of a bone tumor, and the symptoms are often trivialized. Bone pain of unknown origin, swelling, and functional limitations should be investigated with conventional x-rays. Lesions of unclear origin should be biopsied after a meticulous clinical and radiologic evaluation. Multimodal treatment consists of neo - adjuvant chemotherapy, limb-preserving resection if possible, and radiotherapy where indicated. In multicenter studies, patients with osteosarcoma achieve event-free survival in 64% of cases if their disease is localized, and 28% if it is metastatic; the corresponding figures for patients with Ewing's sarcoma are 80% and 27%, respectively.
CONCLUSION
With implementation of the current treatment recommendations, most children and adolescents with malignant bone tumors can be treated successfully with curative intent. These patients should be referred to a sarcoma center for diagnosis and treatment.
Topics: Humans; Child; Adolescent; Sarcoma, Ewing; Osteosarcoma; Bone Neoplasms; Combined Modality Therapy
PubMed: 37097079
DOI: 10.3238/arztebl.m2023.0079 -
The International Journal of... 2019Although rare among the general population, bone malignancies have a high rate of incidence among children and adolescents and are associated with high mortality rates.... (Review)
Review
Although rare among the general population, bone malignancies have a high rate of incidence among children and adolescents and are associated with high mortality rates. Osteosarcoma (also known as osteogenic sarcoma) is the most frequent primary cancer of bone and shows a high tendency to metastasize to the lung. Despite the frequent use of osteosarcoma-derived cell lines in basic biomechanical research and for the evaluation of cell responses to new biomaterials, the mechanical phenotype and the differences between osteosarcoma cells and related cell types, such as mesenchymal cells, osteoblasts and osteocytes, remain largely unknown. In the present review we summarize current knowledge of the biophysical and mechanical properties of the niche of primary osteosarcomas and of the malignant cells, and discuss the impact of these features on the progression of malignancy.
Topics: Animals; Bone Neoplasms; Disease Progression; Humans; Osteosarcoma; Phenotype; Tumor Microenvironment
PubMed: 30919911
DOI: 10.1387/ijdb.190019us -
Frontiers in Bioscience (Landmark... Aug 2021: Osteosarcoma is a common bone tumor with extremely high malignancy, occurring mostly in children and adolescents. At present, the survival rate of osteosarcomas has... (Review)
Review
: Osteosarcoma is a common bone tumor with extremely high malignancy, occurring mostly in children and adolescents. At present, the survival rate of osteosarcomas has made progress in some aspects; however, this can only be regarded as a partial success because substantial progress has not been made in the last few decades. : The kinesin superfamily is a group of proteins that play regulatory roles in various metabolic processes and are closely related to tumor metastasis. Increasing evidence shows that kinesins play key roles in the occurrence and development of human cancer. : This review summarizes the roles of the kinesin superfamily proteins in osteosarcoma and related functions.
Topics: Bone Neoplasms; Humans; Kinesins; Osteosarcoma
PubMed: 34455766
DOI: 10.52586/4950 -
Experimental Biology and Medicine... Jun 2016Osteosarcoma is the most frequent type of primary bone tumors among children and adolescents. During the past years, little progress has been made regarding prognosis of... (Review)
Review
Osteosarcoma is the most frequent type of primary bone tumors among children and adolescents. During the past years, little progress has been made regarding prognosis of osteosarcoma patients, especially for those with metastatic disease. Genomic instability and gene alterations are common, but current data do not reveal a consistent and repeatable pattern of osteosarcoma development, thus paralleling the tumor's high heterogeneity. Critical signal transduction pathways have been implicated in osteosarcoma pathobiology and are being evaluated as therapeutic targets, including receptor activator for nuclear factor-κB (RANK), Wnt, Notch, phosphatidylinositol 3-kinase/Akt/mammalian target of rapamycin, and mechanotransduction pathways. Herein, we recapitulate and discuss recent advances in the context of molecular mechanisms and signaling networks that contribute to osteosarcoma progression and metastasis, towards patient-tailored and novel-targeted treatments.
Topics: Animals; Gene Regulatory Networks; Humans; Osteosarcoma; Signal Transduction
PubMed: 27190271
DOI: 10.1177/1535370216648806 -
Cells Dec 2021Tumorigenesis is a long-term and multistage process that often leads to the formation of metastases. During this pathological course, two major events appear to be... (Review)
Review
Tumorigenesis is a long-term and multistage process that often leads to the formation of metastases. During this pathological course, two major events appear to be crucial: primary tumour growth and metastatic expansion. In this context, despite research and clinical advances during the past decades, bone cancers remain a leading cause of death worldwide among paediatric cancer patients. Osteosarcomas are the most common malignant bone tumours in children and adolescents. Notwithstanding advances in therapeutic treatments, many patients succumb to these diseases. In particular, less than 30% of patients who demonstrate metastases at diagnosis or are poor responders to chemotherapy survive 5 years after initial diagnosis. LIM kinases (LIMKs), comprising LIMK1 and LIMK2, are common downstream effectors of several signalization pathways, and function as a signalling node that controls cytoskeleton dynamics through the phosphorylation of the cofilin family proteins. In recent decades, several reports have indicated that the functions of LIMKs are mainly implicated in the regulation of actin microfilament and the control of microtubule dynamics. Previous studies have thus identified LIMKs as cancer-promoting regulators in multiple organ cancers, such as breast cancer or prostate cancer. This review updates the current understanding of LIMK involvement in osteosarcoma progression.
Topics: Animals; Bone Neoplasms; Bone Remodeling; Humans; Lim Kinases; Models, Biological; Osteogenesis; Osteosarcoma
PubMed: 34944050
DOI: 10.3390/cells10123542 -
Cell Proliferation Jan 2021Osteosarcoma is the most common primary bone malignancy and is a neoplasm thought to be derived from the bone-forming mesenchymal stem cells. Aberrant activation of... (Review)
Review
Osteosarcoma is the most common primary bone malignancy and is a neoplasm thought to be derived from the bone-forming mesenchymal stem cells. Aberrant activation of oncogenes and inactivation of tumour suppressor genes by somatic mutations and epigenetic mechanisms play a pivotal pathogenic role in osteosarcoma. Aside from alterations in these protein-coding genes, it has now been realized that dysregulation of non-coding RNAs (ncRNAs), including microRNAs (miRNAs), long non-coding RNAs (lncRNAs) and the recently discovered circular RNAs (circRNAs), is crucial to the initiation and progression of osteosarcoma. CircRNAs are single-stranded RNAs that form covalently closed loops and function as an important regulatory element of the genome through multiple machineries. Recently, an increasing number of studies suggested that circRNAs also played critical roles in osteosarcoma. This review summarizes recent development and progression in circRNA transcriptome analysis and their functions in the modulation of osteosarcoma progression.
Topics: Humans; Osteosarcoma; RNA, Circular
PubMed: 33103338
DOI: 10.1111/cpr.12936 -
BMC Cancer Sep 2022The timing of events in the management of osteosarcoma may be critical for patient survivorship; however, the prognostic value of factors such as onset of symptoms or... (Review)
Review
BACKGROUND
The timing of events in the management of osteosarcoma may be critical for patient survivorship; however, the prognostic value of factors such as onset of symptoms or initiation of therapy in these patients has not been studied. This study sought to review the literature reporting treatment of osteosarcoma to determine the utility of event timing as a prognostic indicator. Due to significant heterogeneity in the literature, this study was conducted as a scoping review to assess the current state of the literature, identify strengths and weaknesses in current reporting practices, and to propose avenues for future improvement.
MAIN BODY
This review screened 312 peer-reviewed studies of osteosarcoma in any anatomic location published in an English journal for reporting of an event timing metric of any kind in a population of 6 or more. Thirty-seven studies met inclusion/exclusion criteria and were assessed for level of evidence, quality, and event timing metric. Reviewers also collated: publication year, population size, population age, tumor site, tumor type, surgical treatment, and adjuvant medical treatment. Extracted event timing data were further characterized using nine standardized categories to enable systematic analysis. The reporting of event timing in the treatment of osteosarcoma was incomplete and heterogenous. Only 37 of 312 (11.9%) screened studies reported event timing in any capacity. The period between patient-reported symptom initiation and definitive diagnosis was the most reported (17/37, 45.9%). Symptom duration was the second most reported period (10/37, 27.0%). Event timing was typically reported incidentally and was never rigorously incorporated into data analysis or discussion. No studies considered the impact of event timing on a primary outcome. The six largest studies were assessed in detail to identify pearls for future researchers. Notable shortcomings included the inadequate reporting of the definition of an event timing period and the pooling of patients into poorly defined timing groups.
CONCLUSIONS
Inconsistent reporting of event timing in osteosarcoma treatment prevents the development of clinically useful conclusions despite evidence to suggest event timing is a useful prognostic indicator. Consensus guidelines are necessary to improve uniformity and utility in the reporting of event timing.
Topics: Bone Neoplasms; Humans; Osteosarcoma; Prognosis
PubMed: 36088295
DOI: 10.1186/s12885-022-10061-0 -
Annals of the Royal College of Surgeons... Jan 1969
Topics: Adolescent; Adult; Age Factors; Aged; Amputation, Surgical; Animals; Bone Neoplasms; Child; Child, Preschool; Dogs; Femoral Neoplasms; Humans; Infant; Lung Neoplasms; Middle Aged; Neoplasm Metastasis; Osteosarcoma; Radiography; Tibia
PubMed: 5251424
DOI: No ID Found -
Head and Neck Pathology Mar 2020Gnathic fibro-osseous lesions are a diverse group of disease processes which share overlapping microscopic features characterized by fibroblastic stroma with variable... (Review)
Review
Gnathic fibro-osseous lesions are a diverse group of disease processes which share overlapping microscopic features characterized by fibroblastic stroma with variable cellularity and a range of bone forming pathological processes leading to woven, sclerotic and cementum-like structures. Some of the lesions are unique to craniofacial location and a combination of clinical, radiological and pathological correlation is often necessary for diagnostic accuracy. Gnathic osteosarcomas are rare tumors with differences in age distribution and behavior as compared to osteosarcoma of long bones. This review will discuss the clinicopathological and radiological features of gnathic fibro-osseous lesions and osteosarcoma with updates on current genetics and molecular pathogenesis.
Topics: Cementoma; Fibroma, Ossifying; Fibrous Dysplasia of Bone; Head and Neck Neoplasms; Humans; Osteomyelitis; Osteosarcoma
PubMed: 31950477
DOI: 10.1007/s12105-019-01094-2 -
Oncology Reports Aug 2017Osteosarcomas (OS), especially those with metastatic or unresectable disease, have limited treatment options. The greatest advancement in treatments occurred in the... (Review)
Review
Osteosarcomas (OS), especially those with metastatic or unresectable disease, have limited treatment options. The greatest advancement in treatments occurred in the 1980s when multi-agent chemotherapy, including doxorubicin, cisplatin, high-dose methotrexate, and, in some regimens, ifosfamide, was demonstrated to improve overall survival compared with surgery alone. However, standard chemotherapeutic options have been limited by poor response rates in patients with relapsed or advanced cases. It has been reported that VEGFR expression correlates with the outcome of patients with osteosarcoma and circulating VEGF level has been associated with the development of lung metastasis. At present, it seems to us that progress has not been made since Grignani reported a phase II cohort trial of sorafenib and sorafenib combined with everolimus for advanced osteosarcoma, which, in a sense, have become a milestone as a second-line therapy for osteosarcoma. Although the recognization of muramyltripepetide phosphatidyl-ethanolamine has made some progress based on its combination with standard chemotherapy, its effect on refractory cases is controversial. Personalized comprehensive molecular profiling of high-risk osteosarcoma up to now has not changed the therapeutic prospect of advanced osteosarcoma significantly. Thus, how far have we moved forward and what therapeutic strategy should we prefer for anti-angiogenesis therapy? This review provides an overview of the most updated anti-angiogenesis therapy in OS and discusses some clinical options in order to maintain or even improve progression-free survival.
Topics: Disease-Free Survival; Gene Expression Regulation, Neoplastic; Humans; Lung Neoplasms; Neoplasm Metastasis; Neoplasm Staging; Neovascularization, Pathologic; Osteosarcoma; Vascular Endothelial Growth Factor A; Vascular Endothelial Growth Factor Receptor-1
PubMed: 28656259
DOI: 10.3892/or.2017.5735