Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0279530 (bone cancer)
 1,036 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Temporal bone cancer, a relatively rare disease, often presents in a subtle manner, which may delay diagnosis. It should be suspected in any case of persistent otitis media or otitis externa that fails to improve with adequate treatment. This article discusses physical and diagnostic imaging indications and epidemiology and histologic distribution of these tumors. Staging systems, patient selection criteria are presented as well as operative techniques and follow-up treatment. Despite advances in operative technique and postoperative care, long-term survival remains poor.
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PMID:Temporal bone cancer. 829 70

Ear infection is the most common cause of brain abscess. A suppurative process in the ear has access to the central nervous system by direct extension or through preformed pathways, either developmental, traumatic or pathologic, or by retrograde spread along the thromobophlebitic vein. Squamous cell carcinoma of middle ear or mastoid cavity is a rare disease and often associated with chronic otitis or chronic draining ear. Ear malignancy easily produces the destruction of temporal bone which may serve as a route for coexistent infection spreading into the brain. This paper reports a case of brain abscess occurring in a patient with temporal bone cancer. To the best of our knowledge, no more than three similar cases have been reported in the literature. The authors suggest that if an otogenic brain abscess is concomitant with infection and granulomatous mass in the ear, biopsy should be performed to rule out malignancy.
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PMID:[Brain abscess as a complication of ear malignancy: a case report]. 885 95

Childhood osteogenic sarcoma (OS) is a rare bone cancer occurring primarily in adolescents. The North American pediatric cooperative groups have performed a series of clinical treatment trials in this disease over the past several decades, and biology studies of tumor tissue have been an important study component. A meeting was held in Bethesda, Maryland on November 29-30, 2001, sponsored by the NIH Office of Rare Diseases, the Children's Oncology Group, and the National Cancer Institute-Cancer Therapy Evaluation Program with the general objectives: (a) to review the current state of knowledge regarding OS biology; (b) to identify, prioritize, and support the development of biology studies of potential clinical relevance in OS; and (c) to discuss the available tissue resources and the appropriate methods for analysis of OS samples for the conduct of biology studies. This report summarizes the information presented and discussed by the meeting participants.
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PMID:Biology of childhood osteogenic sarcoma and potential targets for therapeutic development: meeting summary. 1465 23

Temporal bone cancer, a relatively rare disease, accounting for less than 0.2% of all tumors of the head and neck and is associated with a poor outcome; often presents in a subtle manner, which may delay diagnosis. It should be suspected in any case of persistent otitis media or otitis externa that fails to improve with adequate treatment. Despite advances in operative technique and postoperative care, long-term survival remains poor). It includes cancers arising from pinna that spreads to the temporal bone, primary tumors of the external auditory canal (EAC), middle ear, mastoid, petrous apex, and metastatic lesions to the temporal bone. Here is a report on a case of temporal bone carcinoma presenting with right otalgia, otorrhea and facial paralysis. The patient was initially diagnosed as mastoiditis and later the clinical impression was revised to temporal bone carcinoma (undifferentiated type), based on the pathologic findings.
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PMID:Rare case of temporal bone carcinoma with intracranial extension. 2429 89

Osteosarcoma is the most common bone cancer in children and young adults. Surgery and multi-agent chemotherapy are the standard treatment regimens for this disease. New therapies are being investigated to improve overall survival in patients. Molecular targets that actively modulate cell processes, such as cell-cycle control, cell proliferation, metabolism, and apoptosis, have been studied, but it remains a challenge to develop novel, effective-targeted therapies to treat this heterogeneous and complex disease. MicroRNAs (miRNAs) are small non-coding RNAs that play critical roles in regulating cell processes including growth, development, and disease. miRNAs function as oncogenes or tumor suppressors to regulate gene and protein expression. Several studies have demonstrated the involvement of miRNAs in the pathogenesis of osteosarcoma with the potential for development in disease diagnostics and therapeutics. In this review, we discuss the current knowledge on the role of miRNAs and their target genes and evaluate their potential use as therapeutic agents in osteosarcoma. We also summarize the efficacy of inhibition of oncogenic miRNAs or expression of tumor suppressor miRNAs in preclinical models of osteosarcoma. Recent progress on systemic delivery as well as current applications for miRNAs as therapeutic agents has seen the advancement of miR-34a in clinical trials for adult patients with non-resectable primary liver cancer or metastatic cancer with liver involvement. We suggest a global approach to the understanding of the pathogenesis of osteosarcoma may identify candidate miRNAs as promising biomarkers for this rare disease.
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PMID:MicroRNAs and Potential Targets in Osteosarcoma: Review. 2638 Feb 45

Osteosarcoma is the predominant form of bone cancer, affecting mostly adolescents. Recent progress made in molecular genetic studies of osteosarcoma has changed our view on the cause of the disease and ongoing therapeutic approaches for patients. As we draw closer to gaining more complete catalogs of candidate cancer driver genes in common forms of cancer, the landscape of somatic mutations in osteosarcoma is emerging from its first phase. In this review, we summarize recent whole genome and/or whole exome genomic studies, and then put these findings in the context of genetic hallmarks of somatic mutations and mutational processes in human osteosarcoma. One of the lessons learned here is that the extent of somatic mutations and complexity of the osteosarcoma genome are similar to that of common forms of adult cancer. Thus, a much higher number of samples than those currently obtained are needed to complete the catalog of driver mutations in human osteosarcoma. In parallel, genetic studies in other species have revealed candidate driver genes and their roles in the genesis of osteosarcoma. This review also summarizes newly identified drivers in genetically engineered mouse models (GEMMs) and discusses our understanding of the impact of nature and number of drivers on tumor latency, subtypes, and metastatic potentials of osteosarcoma. It is becoming apparent that a synergistic team composed of three drivers (one 'first driver' and two 'synergistic drivers') may be required to generate an animal model that recapitulates aggressive osteosarcoma with a short latency. Finally, new cancer therapies are urgently needed to improve survival rate and quality of life for osteosarcoma patients. Several vulnerabilities in osteosarcoma are illustrated in this review to exemplify the opportunities for next generation molecularly targeted therapies. However, much work remains in order to complete our understanding of the somatic mutation basis of osteosarcoma, to develop reliable animal models of human disease, and to apply this information to guide new therapeutic approaches for reducing morbidity and mortality of this rare disease.
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PMID:Molecular genetics of osteosarcoma. 2776 Mar 7

Osteosarcoma (OS) is a heterogeneous and rare disease with a disproportionate impact because it mainly affects children and adolescents. Lamentably, more than half of patients with OS succumb to metastatic disease. Clarification of the etiology of the disease, development of better strategies to manage progression, and methods to guide personalized treatments are among the unmet health needs for OS patients. Progress in managing the disease has been hindered by the extreme heterogeneity of OS; thus, better models that accurately recapitulate the natural heterogeneity of the disease are needed. For this study, we used cell lines derived from two spontaneous canine OS tumors with distinctly different biological behavior (OS-1 and OS-2) for heterotypic in vivo modeling that recapitulates the heterogeneous biology and behavior of this disease. Both cell lines demonstrated stability of the transcriptome when grown as orthotopic xenografts in athymic nude mice. Consistent with the behavior of the original tumors, OS-2 xenografts grew more rapidly at the primary site and had greater propensity to disseminate to lung and establish microscopic metastasis. Moreover, OS-2 promoted formation of a different tumor-associated stromal environment than OS-1 xenografts. OS-2-derived tumors comprised a larger percentage of the xenograft tumors than OS-1-derived tumors. In addition, a robust pro-inflammatory population dominated the stromal cell infiltrates in OS-2 xenografts, whereas a mesenchymal population with a gene signature reflecting myogenic signaling dominated those in the OS-1 xenografts. Our studies show that canine OS cell lines maintain intrinsic features of the tumors from which they were derived and recapitulate the heterogeneous biology and behavior of bone cancer in mouse models. This system provides a resource to understand essential interactions between tumor cells and the stromal environment that drive the progression and metastatic propensity of OS.
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PMID:Heterotypic mouse models of canine osteosarcoma recapitulate tumor heterogeneity and biological behavior. 2787 35