Bone Cancers
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Primary bone tumours are tumours that start in the bone. In contrast, secondary bone cancer is where the cancer started in another part of the body but has then spread to the bones. The most common types of primary bone tumour are osteosarcoma and Ewing's sarcoma, both of which are most frequently diagnosed in children and young adults. Other less common types of bone cancer include: Chondrosarcoma (a cancer arising in cartilage cells, usually found in adults between ages 50-75, though the less common mesenchymal-chondrosarcoma is more frequent in younger patients), Malignant Fibrous Histiocytoma of bone (MFH), Chondoma (a rare low grade malignancy occuring mostly between ages 30 -70), and other rare tumours.

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Information for Patients and the Public
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Ewing's Sarcoma
Primary Lymphoma of Bone
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Information Patients and the Public (9 links)

Information for Health Professionals / Researchers (11 links)

Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Dierselhuis EF, van den Eerden PJ, Hoekstra HJ, et al.
Radiofrequency ablation in the treatment of cartilaginous lesions in the long bones: results of a pilot study.
Bone Joint J. 2014; 96-B(11):1540-5 [PubMed] Related Publications
Atypical cartilaginous tumours are usually treated by curettage. The purpose of this study was to show that radiofrequency ablation was an effective alternative treatment. We enrolled 20 patients (two male, 18 female, mean age 56 years (36 to 72) in a proof-of-principle study. After inclusion, biopsy and radiofrequency ablation were performed, followed three months later by curettage and adjuvant phenolisation. The primary endpoint was the proportional necrosis in the retrieved material. Secondary endpoints were correlation with the findings on gadolinium enhanced MRI, functional outcome and complications. Our results show that 95% to 100% necrosis was obtained in 14 of the 20 patients. MRI had a 91% sensitivity and 67% specificity for detecting residual tumour after curettage. The mean functional outcome (MSTS) score six weeks after radiofrequency ablation was 27.1 (23 to 30) compared with 18.1 (12 to 25) after curettage (p < 0.001). No complications occurred after ablation, while two patients developed a pathological fracture after curettage. We have shown that radiofrequency ablation is capable of completely eradicating cartilaginous tumour cells in selective cases. MRI has a 91% sensitivity for detecting any residual tumour. Radiofrequency ablation can be performed on an outpatient basis allowing a rapid return to normal activities. If it can be made more effective, it has the potential to provide better local control, while improving functional outcome.

Wang X, Goldstein D, Crowe PJ, Yang JL
Impact of STAT3 inhibition on survival of osteosarcoma cell lines.
Anticancer Res. 2014; 34(11):6537-45 [PubMed] Related Publications
BACKGROUND/AIM: Osteosarcoma is often a fatal malignancy. Constitutive STAT3 activation is associated with various human cancers and commonly suggests poor prognosis. We aimed to investigate the effect and potential molecular mechanisms of STAT3 inhibition on osteosarcoma.
MATERIALS AND METHODS: STAT3 inhibitor S3I-201 was investigated in six osteosarcoma cell lines. Crystal violet colorimetric, clonogenic, cleaved caspase-3 assays and western blot were performed to measure the effect and mechanisms of STAT3 inhibition.
RESULTS: All osteosarcoma cell lines expressed phosphorylated STAT3. Anti-proliferative effects of S3I-201 were dose- and time-dependent. S3I-201 also inhibited colony-formation and induced apoptosis through the caspase cleavage pathway. Finally, molecular mechanism studies suggested that down-regulation of STAT3 phosphorylation and downstream STAT3-target genes such as cyclin D1 and survivin may contribute to S3I-201-mediated anti-proliferation and apoptosis.
CONCLUSION: Inhibition of STAT3 signalling suppressed osteosarcoma cell growth and induced apoptosis, and indicated that STAT3 targeted-therapy may have therapeutic potential in osteosarcoma.

Related: Apoptosis Osteosarcoma STAT3

Tonak M, Becker M, Graf C, et al.
HDAC inhibitor-loaded bone cement for advanced local treatment of osteosarcoma and chondrosarcoma.
Anticancer Res. 2014; 34(11):6459-66 [PubMed] Related Publications
UNLABELLED: The treatment of osteosarcoma, especially wide resection, is challenging. An additional local drug therapy after resection using anti-neoplastic bone cement (Polymethylmethacrylate (PMMA)) could help improve the outcome of therapy. In this study, we evaluated the effects of PMMA loaded with valproic acid (VPA) and suberoylanilide hydroxamic acid (SAHA) on the cell activity of a SaOs-2 cell culture, as well as the elution rate of the drugs out of the bone cement.
MATERIALS AND METHODS: In our experiments, we used the SaOs-2 osteosarcoma and the SW1353 chondrosarcoma cell line. Bone cement clots (5 g) were prepared and loaded with different drug concentrations of VPA (25 mg and 50 mg) and SAHA (1 mg, 2.5 mg and 5 mg). Two control groups were established, one with a native cement clot, the other with human mesenchymal stem cells, in order to evaluate toxicity on non tumor-cells. Cell activity was measured using an Alamar Blue assay on days 1, 2, 3, 4 and 7. The cement clots were additionally examined in a material testing unit for biomechanical and structural changes.
RESULTS: Tumor cells showed a significant and complete reduction of activity under therapy with VPA and SAHA. Drug release of VPA was extensive between days 0 and 3 and decreased progressively to day 7. Cumulative drug concentration in the medium continuously increased. Biomechanical testing of the cement clots showed no differences in stability and architecture compared to the control group. SaOs-2 and SW1353 cells with medium from native cement clots without drug therapy presented a cell activity of 100% in all groups and during all measurements. Human mesenchymal stem cells were not significantly affected during therapy with VPA and low concentrations of SAHA. In contrast, cell activity of human mesenchymal stem cells was significantly reduced under therapy with higher concentrations of SAHA, with an approximately linear decrease between days 0-3 and a rapidly decreasing activity between days 4-7.
CONCLUSION: A local cytotoxic therapy in the treatment of osteosarcoma and chondrosarcoma might improve the rate of metastasis and survival of patients. Our results present an encouraging approach to loading PMMA with anti-neoplastic drugs.

Related: Apoptosis Chondrosarcoma Osteosarcoma

Max D, Kühnöl CD, Burdach S, et al.
Indoleamine-2,3-dioxygenase in an immunotherapy model for Ewing sarcoma.
Anticancer Res. 2014; 34(11):6431-41 [PubMed] Related Publications
BACKGROUND/AIM: Interleukin-2 (IL2) transgenic Ewing sarcoma cells reduce tumor growth in vivo and in vitro. In the present study we analyzed the expression of immune suppressive indoleamine-2,3-dioxygenase (IDO) in this model.
MATERIALS AND METHODS: Expression of IDO was analyzed by polymerase chain reaction. The impact of the cluster of differentiation 137 (CD137)/CD137 ligand (CD137L) co-stimulatory system on expression of IDO and different cytokines was analyzed both in vivo and in vitro.
RESULTS: Tumors that developed in vivo in the presence of IL2 transgenic tumor cells expressed IDO. The presence of CD137L transgenic tumor cells led to down-regulation of IDO. Further in-vitro analysis of this phenomenon indicated that IDO was expressed in tumor cells as a consequence of interferon-gamma produced by lymphocytes in response to IL2. Depending on the concentration of IL2, stimulation of CD137 increased or reduced cytokine production in lymphocytes.
CONCLUSION: Our data indicate that the CD137/CD137L pathway can modulate the immune response against Ewing sarcoma cells.

Related: Cytokines Interleukin 2 (Aldesleukin) Ewing's Sarcoma

Morii T, Ohtsuka K, Ohnishi H, et al.
BH3 mimetics inhibit growth of chondrosarcoma--a novel targeted-therapy for candidate models.
Anticancer Res. 2014; 34(11):6423-30 [PubMed] Related Publications
BACKGROUND: Chondrosarcoma is refractory to conventional chemotherapy. BH-3 mimetics ABT-737 and ABT-263 are synthetic small-molecule inhibitors of anti-apoptotic proteins B-cell lymphoma-2 (Bcl2) and Bcl-xL, which play a critical role in survival of chondrosarcoma cells.
MATERIALS AND METHODS: Chondrosarcoma cell lines SW-1353 and CS-1 were used as the disease model. We used immunoblotting to assess the expression of target molecules Bcl2 and Bcl-xL, and the apoptotic inducers Bcl2-associated X (Bax) and Bcl2-antagonist/killer (Bak). In vitro growth inhibition by BH-3 mimetics was confirmed by photomicroscopic cell counting and 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium, inner salt (MTS) assay. Apoptotic induction was confirmed by Enzyme-Linked ImmunoSorbent Assay (ELISA). In vivo growth inhibition was assessed in a non-obese diabetic/severe combined immunodeficient (NOD/SCID) mouse model.
RESULTS: Expression of the target and effector molecules was confirmed in chondrosarcoma cell lines. BH3 mimetics significantly inhibited cell growth and induced apoptosis in vitro. Administration of ABT-263 inhibited chondrosarcoma growth and improved survival in a mouse model.
CONCLUSION: BH3 mimetics represent a novel treatment modality for chondrosarcoma.

Related: Apoptosis Chondrosarcoma BAK1

Wilartratsami S, Muangsomboon S, Benjarassameroj S, et al.
Prevalence of primary spinal tumors: 15-year data from Siriraj Hospital.
J Med Assoc Thai. 2014; 97 Suppl 9:S83-7 [PubMed] Related Publications
OBJECTIVE: To determine prevalence, demographic data and clinical presentation of primary vertebral tumors.
MATERIAL AND METHOD: A retrospective study of the primary spine tumor specimens from Siriraj bone tumor registry from 1996 to 2010.
RESULTS: From the study, primary spinal tumors constituted 85 of 1,679 bone tumor cases (5.06%). The common benign spinal tumors were giant cell tumor and hemangioma. The common malignant spinal tumors were chordoma, chondrosarcoma, and osteosarcoma. The mean age ofpresentation was 44.68 years. Fifty-three percent of tumors occurred in females. Pain was the most common presenting symptom, occurring in 73.53% of malignant and 52.94% of benign tumors. Neurological involvement occurred in 25% of malignant tumor Malignant lesions predominated in the sacral region while the most common location ofbenign specimens was thoracic region.
CONCLUSION: The present study was the first demographic study ofprimary spinal tumor in Thai showed variety of prevalence when compared with similar studies based on Western patients. Whether these results reflect differences in the population, race and data collection method.

Related: Chondrosarcoma Osteosarcoma Thailand

Vijayakumar V, Lowery R, Zhang X, et al.
Pediatric osteosarcoma: a single institution's experience.
South Med J. 2014; 107(11):671-5 [PubMed] Related Publications
OBJECTIVES: The aim of the study was to evaluate outcomes with an examination of individual predictors influencing survival at a single institution.
METHODS: This was a retrospective review of the 28 pediatric osteosarcoma patients diagnosed and studied from 2000 through 2012. Twenty-eight patient charts and imaging studies were reviewed for age, race, sex, location, extent of disease at presentation, imaging results, histology, treatment options, and overall survival.
RESULTS: Of the 28 patients who were identified, the median age at diagnosis was 14 years. The majority of the patients were male African Americans with the tumor located in the lower long bones and most had conventional osteosarcoma histology. Four patients had metastasis at diagnosis. Of the 28 patients, 16 patients underwent limb salvage surgery, 6 underwent amputation, 4 had biopsy only, 1 had hip disarticulation, and 1 moved out of state and had no information available. All 28 patients received chemotherapy. Four patients received additional radiation therapy. On follow-up, 15 patients were still alive at last clinical contact and 13 died. Of the deceased, the median survival time was 2.3 years. The patient who lived the longest survived 8.3 years. Metastasis at diagnosis was associated with poorer outcome (P = 0.002). The 5-year overall survival rate was 40% (95% confidence interval 18-62) for our entire population of patients.
CONCLUSIONS: Survival in our patient cohort tended to be at the lower end of the spectrum reported by other contemporary treatment centers of excellence or Surveillance, Epidemiology, and End Results databases probably because of the large number of African American patients with associated poor socioeconomic status. Future studies should be conducted to explore biological and nonbiological factors that may affect the prognosis in this disease.

Related: Osteosarcoma

De Meis E, Brandão BC, Capella FC, et al.
Catastrophic antiphospholipid syndrome in cancer patients: an Interaction of clotting, autoimmunity and tumor growth?
Isr Med Assoc J. 2014; 16(9):544-7 [PubMed] Related Publications
Thrombosis is a common phenomenon in patients with malignancies. It is believed that thrombosis is multifactorial and that in addition to mechanisms directly associated with cancer and its treatment, it may also be related to the interaction between the immune system and clotting. The present work describes four cancer patients (three adults and one child) whose clinical course was characteristic of catastrophic antiphospholipid syndrome (CAPS) in intensive care units of the National Cancer Institute of Rio de Janeiro. The presence of findings similar to those in CAPS can be attributed to an unbalanced interaction between the immune system and coagulation.

Related: Extra-Hepatic Bile duct cancer (cholangiocarcinoma) Lung Cancer Ewing's Sarcoma

Oksüz DC, Tural D, Dincbas FÖ, et al.
Non-metastatic Ewing's sarcoma family of tumors of bone in adolescents and adults: prognostic factors and clinical outcome-single institution results.
Tumori. 2014 Jul-Aug; 100(4):452-8 [PubMed] Related Publications
AIMS AND BACKGROUND: There is limited data regarding outcomes of Ewing's sarcoma family of tumors in adolescents and adults compared with the same tumors in childhood. The aim of the study was to analyze prognostic factors and treatment results in a cohort of adolescents and adults with non-metastatic skeletal Ewing's sarcoma family of tumors.
METHODS AND STUDY DESIGN: From 1992-2008, 90 adolescents and adults with Ewing's sarcoma family of tumors of the bone were referred to our institution. Sixty-five (72%) non-metastatic patients with analyzable data and treated in our institution were retrospectively evaluated. All patients were treated with alternated chemotherapy regimens administered every 3 weeks. The local treatment modality was selected according to tumor and patient characteristics.
RESULTS: The median age was 21 years (range, 13-50). Most patients (74%) were >17 years of age. Forty-six percent of the tumors were located in the extremities. Local therapy was surgery in 45 patients and radiotherapy alone in 19 patients. Twenty-one patients received preoperative and 13 patients postoperative radiotherapy. Median follow-up was 43 months (range, 7-167). The 5-year event-free and overall survival rates for all patients were 44% and 49%, respectively. On univariate survival analysis, event-free and overall survival were worse for patients >17 years of age, tumor size >8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (<90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis.
CONCLUSIONS: We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.

Related: Ewing's Sarcoma

Salunke AA, Chen Y, Tan JH, et al.
Does a pathological fracture affect the prognosis in patients with osteosarcoma of the extremities? : a systematic review and meta-analysis.
Bone Joint J. 2014; 96-B(10):1396-403 [PubMed] Related Publications
Opinion remains divided as to whether the development of pathological fracture affects the prognosis of patients with an osteosarcoma of the extremities. We conducted a comprehensive systematic review and meta-analysis of papers which reported the outcomes of osteosarcoma patients with and without a pathological fracture. There were eight eligible papers for final analysis which reported on 1713 patients, of whom 303 (17.7%) had a pathological fracture. The mean age for 1464 patients in six studies was 23.2 years old (2 to 82). The mean follow-up for 1481 patients in seven studies was 90.1 months (6 to 240). The pooled estimates of local recurrence rates in osteosarcoma patients with and without pathological fractures were 14.4% (8.7 to 20.0) versus 11.4% (8.0 to 14.8). The pooled estimate of relative risk was 1.39 (0.89 to 2.20). The pooled estimates of five-year event-free survival rates in osteosarcoma patients with and without a pathological fracture were 49.3% (95% CI 43.6 to 54.9) versus 66.8% (95% CI 60.7 to 72.8). The pooled estimate of relative risk was 1.33 (1.12 to 1.59). There was no significant difference in the rate of local recurrence between patients who were treated by amputation or limb salvage. The development of a pathological fracture is a negative prognostic indicator in osteosarcoma and is associated with a reduced five-year event-free survival and a possibly higher rate of local recurrence. Our findings suggest that there is no absolute indication for amputation, as similar rates of local recurrence can be achieved in patients who are carefully selected for limb salvage.

Related: Osteosarcoma

Dings JP, Mizbah K, Bergé SJ, et al.
Secondary closure of small- to medium-size palatal defects after ablative surgery: reappraisal of reconstructive techniques.
J Oral Maxillofac Surg. 2014; 72(10):2066-76 [PubMed] Related Publications
PURPOSE: Postoncologic reconstruction of the palate represents a major surgical challenge with respect to the thin intraoral and intranasal lining. Current reconstructive methods have ranged from obturative closure of the defect to microsurgical free tissue transfer. The final choice of treatment will be influenced by the size and location of the defect and surgeon experience. The goals of palate repair include optimizing palatal function for speech and eating, and avoiding dehiscence or postoperative fistulas. This study assessed the reliability of locoregional flaps for reconstructing maxillary defects.
PATIENTS AND METHODS: The present study described the surgical outcome of locoregional reconstruction of the hard and soft palate of 5 patients who had previously undergone tumor ablative surgery. They ranged in age from 19 to 64 years. None had received postoperative radiotherapy. The resultant surgical defects ranged in size from 2.5 to 12 cm(2). One patient experienced velopharyngeal insufficiency.
RESULTS: In all cases, the palate was closed at the first attempt without complications. All flaps survived, and complete closure was obtained in these 4 patients. The patient with the velopharyngeal insufficiency experienced a significant improvement in articulation and swallowing function.
CONCLUSIONS: The results of these 5 cases indicate that secondary locoregional flaps are a suitable alternative for palatal defect management. They have a high success rate and functional outcome. These secondary techniques can be reliably used to reconstruct small- to moderate-size palatal defects and represent a reliable reconstructive option with minimal morbidity.

Related: Osteosarcoma

Mei J, Zhu XZ, Wang ZY, Cai XS
Functional outcomes and quality of life in patients with osteosarcoma treated with amputation versus limb-salvage surgery: a systematic review and meta-analysis.
Arch Orthop Trauma Surg. 2014; 134(11):1507-16 [PubMed] Related Publications
INTRODUCTION: To perform a meta-analysis for comparing the functional outcomes and quality of life (QOL) of osteosarcoma patients receiving amputation or limb-salvage surgeries.
MATERIALS AND METHODS: A search was conducted of the Medline, Cochrane, EMBASE, and Google Scholar on September 30, 2013. Studies were included in the analysis if there were patients who underwent amputation and limb-salvage surgery for osteosarcoma or Ewing's sarcoma, and for whom postoperative functional outcomes and QOL were evaluated. Outcomes were compared between participants who underwent limb-salvage operation and those who underwent amputation. The methodological quality of non-randomized comparative studies was assessed using the Newcastle-Ottawa Scale.
RESULTS: A total of 121 studies were identified and 6 were included in the meta-analysis. Quality assessment indicated that all six studies were of high quality. The mean age of the participants ranged from 17 to 37 years, and among them 118 underwent amputations and 138 underwent limb-salvage procedures. The mean length of follow-up ranged from 28 to 145 months. The meta-analysis indicated that functional outcomes and QOL were similar between patients who underwent amputation and those who underwent a limb-salvage procedure.
CONCLUSIONS: This meta-analysis including six high-quality studies indicates that amputation and limb-salvage surgery provide similar functional outcomes and quality of life for patients with osteosarcomas.

Related: Osteosarcoma Ewing's Sarcoma

Tao J, Jiang MM, Jiang L, et al.
Notch activation as a driver of osteogenic sarcoma.
Cancer Cell. 2014; 26(3):390-401 [PubMed] Article available free on PMC after 08/09/2015 Related Publications
Osteogenic sarcoma (OS) is a deadly skeletal malignancy whose cause is unknown. We report here a mouse model of OS based on conditional expression of the intracellular domain of Notch1 (NICD). Expression of the NICD in immature osteoblasts was sufficient to drive the formation of bone tumors, including OS, with complete penetrance. These tumors display features of human OS; namely, histopathology, cytogenetic complexity, and metastatic potential. We show that Notch activation combined with loss of p53 synergistically accelerates OS development in mice, although p53-driven OS is not Rbpj dependent, which demonstrates a dual dominance of the Notch oncogene and p53 mutation in the development of OS. Using this model, we also reveal the osteoblasts as the potential sources of OS.

Related: Osteosarcoma

Tang J, Shen L, Yang Q, Zhang C
Overexpression of metadherin mediates metastasis of osteosarcoma by regulating epithelial-mesenchymal transition.
Cell Prolif. 2014; 47(5):427-34 [PubMed] Related Publications
OBJECTIVES: Osteosarcoma (OS) is one of the most common primary malignant bone tumours of childhood and adolescence, and is characterized by high propensity for metastasis (specially to the lung), which is the main cause of death. However, molecular mechanisms underlying metastasis of OS are still poorly understood.
MATERIALS AND METHODS: Metadherin (MTDH) was identified to be significantly upregulated in OS tissues that had metastasized compared to OS without metastasis, using a two-dimensional approach of electrophoresis, coupled with mass spectrometry. To understand the function of MTDH in OS, OS cell lines U2OS and SOSP-M were transfected with retroviral shRNA vector against MTDH.
RESULTS: It was found that metastatic propensity as well as cell proliferation were significantly reduced in both U2OS and SOSP-M. Migration and invasion of U2OS and SOSP-M cells were significantly lower after knock-down of MTDH. In addition, epithelial-mesenchymal transition (EMT) was reduced after knock-down of MTDH. Clinicopathologically, overexpression of MTDH was significantly associated with metastasis and poor survival of patients with OS.
CONCLUSION: Taken together, our results demonstrate that MTDH mediated metastasis of OS through regulating EMT. This could be an ideal therapeutic target against metastasis of OS.

Related: Osteosarcoma

Tang YJ, Wang JL, Nong LG, et al.
Associations of IL-27 polymorphisms and serum IL-27p28 levels with osteosarcoma risk.
Medicine (Baltimore). 2014; 93(10):e56 [PubMed] Related Publications
Interleukin (IL)-27 is a novel cytokine secreted by stimulation of antigen-presenting cells. No previous studies currently reported the role of IL-27 in the carcinogenesis of osteosarcoma. We aimed to investigate the association of IL-27 polymorphisms and serum IL-27p28 with osteosarcoma risk in a Chinese population.One hundred and sixty osteosarcoma patients and 250 health controls were selected. IL-27 gene -964 A/G, 2905 T/G, and 4730 T/C polymorphisms were determined by using polymerase chain reaction-restriction fragment length polymorphism. Enzyme-linked immunosorbent assay were used to detect serum IL-27p28 levels.The serum IL-27p28 levels were significantly lower in osteosarcoma patients compared with those in controls (P < 0.01). Serum IL-27p28 levels in stages III-IV were lower than those in stages I-II of osteosarcoma (P < 0.05); similar results were also found in patients with metastasis, that is, patients with metastasis have higher IL-27p28 levels than those without metastasis (P < 0.05). There were no associations between genotype and allele frequencies of IL-27 -964 A/G, 2905 T/G, 4730 T/C, and the risk of osteosarcoma (P > 0.05). Stratification analysis also failed to show the associations between -964 A/G, 2905 T/G, and 4730 T/C polymorphisms and the clinical stage and metastasis of osteosarcoma (P > 0.05). Three possible haplotypes (ATT, GTT, and GGC) were identified, but no associations were found between them and the osteosarcoma risk (P > 0.05).This study indicates that the lower serum IL-27p28 levels may be associated with development and progression of osteosarcoma, but IL-27 gene -964 A/G, 2905 T/G, and 4730 T/C polymorphisms and their haplotypes are not associated with osteosarcoma risk.

Related: Osteosarcoma

Ren K, Yao N, Wang G, et al.
Vasculogenic mimicry: a new prognostic sign of human osteosarcoma.
Hum Pathol. 2014; 45(10):2120-9 [PubMed] Related Publications
Vasculogenic mimicry (VM), a formation of nonendothelial microvascular channels, has been generally recognized as a new pattern of neovascularization in aggressive malignancies. However, whether VM is present and clinically significant in osteosarcoma remains unknown. We identified VM by CD34/periodic acid-Schiff double staining of osteosarcoma specimens before chemotherapy and investigated its prognostic implications. Tumors were also immunohistochemically stained for focal adhesion kinase (FAK) and migration inducing gene 7 (Mig-7) to determine whether these markers are associated with the occurrence of VM. VM was found in 15 of 66 osteoblastic-type osteosarcoma samples (22.7%), and the incidence of VM did not differ with respect to patient sex, age, tumor size, tumor site, surgical type, or histologic response to preoperative chemotherapy. However, Kaplan-Meier survival analysis determined that the presence of VM and the tumor necrosis rate after preoperative chemotherapy are associated with both the overall survival (P = .011 and P = .040, respectively) and metastasis-free survival (P = .002 and P = .045, respectively). Furthermore, Cox proportional hazards analysis showed that the presence of VM and the histologic response to preoperative chemotherapy were independent indicators for both poor overall survival (P = .007 and P = .024, respectively) and poor metastasis-free survival (P = .002 and P = .027, respectively). The expression level of FAK and Mig-7 were higher in the VM group than the non-VM group (P = .017 and P = .021, respectively). These results demonstrate the presence of VM in osteoblastic osteosarcoma and suggest that VM is an unfavorable prognostic factor with FAK and Mig-7 expressions as a potential mechanism of VM formation in osteosarcoma.

Related: Angiogenesis and Cancer Osteosarcoma

Tian J, He H, Lei G
Wnt/β-catenin pathway in bone cancers.
Tumour Biol. 2014; 35(10):9439-45 [PubMed] Related Publications
The Wnt signaling pathway regulates some of the crucial aspects of cellular processes. The beta-catenin dependent Wnt signaling (Wnt/β-catenin) pathway controls the expression of key developmental genes, and acts as an intracellular signal transducer. The association of Wnt/β-catenin pathway is often reported with different cancers. In this study, we have reviewed the association of Wnt/β-catenin pathway with bone cancers, focusing on carcinogenesis and therapeutic aspects. Wnt/β-catenin pathway is a highly complex and unique signaling pathway, which has ability to regulate gene expression, cell invasion, migration, proliferation, and differentiation for the initiation and progression of bone cancers, especially osteosarcoma. Association of Wnt/β-catenin pathway with chondrosarcoma, Ewing's sarcoma and chondroma is also documented. Recently, targeting Wnt/β-catenin pathway has gained significant interests as a potential therapeutic application for the treatment of bone cancers. Small RNA technology to knockdown aberrant Wnt/β-catenin or inhibition of β-catenin expression by natural component has shown promising effects against bone cancers. Advances in understanding the mechanisms of Wnt signaling and new technologies have facilitated the discovery of agents that can target and regulate Wnt/β-catenin signaling pathway, and these may provide a basement for the innovative therapeutic approaches in the treatment of bone cancers.

Related: Signal Transduction

Xu S, Yang S, Sun G, et al.
Transforming growth factor-beta polymorphisms and serum level in the development of osteosarcoma.
DNA Cell Biol. 2014; 33(11):802-6 [PubMed] Related Publications
The transforming growth factor-beta (TGF-β) signaling pathway plays critical roles in the development of various diseases. The current study investigated whether TGF-β was involved in the pathogenesis of osteosarcoma from the genetic polymorphism perspective and serum level perspective. We first examined two TGF-β1 polymorphisms, rs1800469C/T and rs1800470T/C, in 202 osteosarcoma patients and 216 healthy controls. Data revealed that the prevalence of rs1800470TT genotype and T allele was significantly elevated in patients than in controls (odds ratio [OR]=2.28, 95% confidence interval [CI]: 1.30-3.98, p<0.001, and OR=1.49, 95% CI: 1.14-1.96, p<0.001). Function analyses showed that healthy controls carrying rs1800470TT genotype had a significantly higher serum level of TGF-β than those carrying the rs1800470CC genotype (191.1±15.7 pg/mL vs. 129.4±10.9 pg/mL, p=0.003). We then compared the serum level of TGF-β between osteosarcoma patients and healthy controls. Results demonstrated a significantly increased serum level of TGF-β in patients than in controls. Further analyses identified that patients with metastasis had augmented levels of serum TGF-β than those without metastasis. These data indicate that TGF-β may be closely involved in the pathogenesis of osteosarcoma.

Related: Osteosarcoma TGFB1

Brown MT, Gikas PD, Bhamra JS, et al.
How safe is curettage of low-grade cartilaginous neoplasms diagnosed by imaging with or without pre-operative needle biopsy?
Bone Joint J. 2014; 96-B(8):1098-105 [PubMed] Related Publications
The pre-operative differentiation between enchondroma, low-grade chondrosarcoma and high-grade chondrosarcoma remains a diagnostic challenge. We reviewed the accuracy and safety of the radiological grading of cartilaginous tumours through the assessment of, first, pre-operative radiological and post-operative histological agreement, and second the rate of recurrence in lesions confirmed as high-grade on histology. We performed a retrospective review of major long bone cartilaginous tumours managed by curettage as low grade between 2001 and 2012. A total of 53 patients with a mean age of 47.6 years (8 to 71) were included. There were 23 men and 30 women. The tumours involved the femur (n = 20), humerus (n = 18), tibia (n = 9), fibula (n = 3), radius (n = 2) and ulna (n = 1). Pre-operative diagnoses resulted from multidisciplinary consensus following radiological review alone for 35 tumours, or with the addition of pre-operative image guided needle biopsy for 18. The histologically confirmed diagnosis was enchondroma for two (3.7%), low-grade chondrosarcoma for 49 (92.6%) and high-grade chondrosarcoma for two (3.7%). Three patients with a low-grade tumour developed a local recurrence at a mean of 15 months (12 to 17) post-operatively. A single high-grade recurrence (grade II) was treated with tibial diaphyseal replacement. The overall recurrence rate was 7.5% at a mean follow-up of 4.7 years (1.2 to 12.3). Cartilaginous tumours identified as low-grade on pre-operative imaging with or without additional image-guided needle biopsy can safely be managed as low-grade without pre-operative histological diagnosis. A few tumours may demonstrate high-grade features histologically, but the rates of recurrence are not affected.

Related: Chondrosarcoma

Gillis CC, Street JT, Boyd MC, Fisher CG
Pelvic reconstruction after subtotal sacrectomy for sacral chondrosarcoma using cadaveric and vascularized fibula autograft: Technical note.
J Neurosurg Spine. 2014; 21(4):623-7 [PubMed] Related Publications
A novel method of spinopelvic ring reconstruction after partial sacrectomy for a chondrosarcoma is described. Chondrosarcoma is one of the most common primary malignant bone tumors, and en bloc resection is the mainstay of treatment. Involvement of the pelvis as well as the sacrum and lumbar spine can result in a technically difficult challenge for en bloc resection and for achievement of appropriate load-bearing reconstruction. After en bloc resection in their patient, the authors achieved reconstruction with a rod and screw construct including vascularized fibula graft as the main strut from the lumbar spine to the pelvis. Additionally, a cadaveric allograft strut was used as an adjunct for the pelvic ring. This is similar to a modified Galveston technique with vascularized fibula in place of the Galveston rods. The vascularized fibula provided appropriate biomechanical support, allowing the patient to return to independent ambulation. There was no tumor recurrence; neurological status remained stable; and the allograft construct integrated well and even increased in size on CT scans and radiographs in the course of a follow-up longer than 7 years.

Related: Chondrosarcoma

Lo Vasco VR, Leopizzi M, Stoppoloni D, Della Rocca C
Silencing of phosphoinositide-specific phospholipase C ε remodulates the expression of the phosphoinositide signal transduction pathway in human osteosarcoma cell lines.
Anticancer Res. 2014; 34(8):4069-75 [PubMed] Related Publications
BACKGROUND: Ezrin, a member of the ezrin-radixin-moesin family, is involved in the metastatic spread of osteosarcoma. Ezrin binds phosphatydil inositol-4,5-bisphosphate (PIP2), a crucial molecule of the phosphoinositide signal transduction pathway. PIP2 levels are regulated by phosphoinositide-specific phospholipase C (PI-PLC) enzymes. PI-PLCε isoform, a well-characterized direct effector of rat sarcoma (RAS), is at a unique convergence point for the broad range of signaling pathways that promote RAS GTPase-mediated signalling.
MATERIALS AND METHODS: By using molecular biology methods and microscopic analyses, we analyzed the expression of ezrin and PLC genes after silencing of PLCE (OMIM *608414) in 143B and Hs888 cell lines.
RESULTS: The growth rate of the cells was slowed, and the expression of ezrin, PLCB1, PLCG2 and PLCD4 was significantly modified. Ezrin displacement from the plasma membrane was observed.
CONCLUSION: The present results corroborate the hypothesis that ezrin and the PI signal transduction system are involved in a common network.

Related: Osteosarcoma Signal Transduction

Jentzsch T, Robl B, Husmann M, et al.
Worse prognosis of osteosarcoma patients expressing IGF-1 on a tissue microarray.
Anticancer Res. 2014; 34(8):3881-9 [PubMed] Related Publications
BACKGROUND: It is hardly possible to define osteosarcoma (OS) patients at greatest risk for non-response to chemotherapy, metastasis and short survival times. Our goal was the investigation of local expression of insulin-like growth factor (IGF-1) with regard to survival time of OS patients using a tissue microarray (TMA).
MATERIALS AND METHODS: Tumor tissue specimens from surgical primary tumor resections were collected from patients with OS. A TMA was composed, sections were stained with rabbit anti-IGF-1 and grading was performed. Statistics involved Kaplan-Meier curves and the log-rank test.
RESULTS: We analyzed immunohistochemical expression of local IGF-1 on a TMA based on surgical primary tumor resections of 67 OS patients. The mean clinical follow-up time was 98 months. Twenty-two (33%) OS patients stained negatively and 44 (66%) OS patients stained positively for IGF-1. Significantly shorter survival was detected with expression of IGF-1 (p=0.007). The 5-year survival rate for patients expressing IGF-1 was 63% compared to 92% in patients without expression of IGF-1. Non-responders to chemotherapy and patients with metastasis, who also stained positively for IGF-1 manifested a significantly (p=0.002 and p<0.0001, respectively) shorter survival.
CONCLUSION: Expression of local IGF-1 in primary tumor tissue appears to significantly affect the aggressiveness of OS, may predict survival time and, above all, may discriminate patients with non-response to chemotherapy and metastasis. This represents the basis for successful patient selection with regard to the decision process for or against chemotherapy and the choice of the most effective therapeutic drug. It may be a more important marker of tumor progression and indicator of prognosis than serum IGF-1. Novel tumor markers and therapeutic agents targeting the local IGF-1 pathway may increase the likelihood of therapeutic success.

Related: IGF1 Osteosarcoma

Leddy LR, Holmes RE
Chondrosarcoma of bone.
Cancer Treat Res. 2014; 162:117-30 [PubMed] Related Publications
Chondrosarcoma is a cartilage forming neoplasm, which is the second most common primary malignancy of bone. Clinicians who treat chondrosarcoma patients must determine the grade of the tumor, and must ascertain the likelihood of metastasis. Acral lesions are unlikely to metastasize, regardless of grade, whereas axial, or more proximal lesions are much more likely to metastasize than tumors found in the distal extremities with equivalent histology. Chondrosarcoma is resistant to both chemotherapy and radiation, making wide local excision the only treatment. Local recurrence is frequently seen after intralesional excision, thus wide local excision is sometimes employed despite significant morbidity, even in low-grade lesions. Chondrosarcoma is difficult to treat. The surgeon must balance the risk of significant morbidity with the ability to minimize the chance of local recurrence and maximize the likelihood of long-term survival.

Related: Chondrosarcoma

Moore DD, Haydon RC
Ewing's sarcoma of bone.
Cancer Treat Res. 2014; 162:93-115 [PubMed] Related Publications
Ewing's sarcoma of bone is a primary bone sarcoma found predominantly in patients during their second decade of life. It is a high-grade aggressive small round blue cell tumor that is part of the Ewing's family of tumors. Its exact eitiology is unknown but it commonly demonstrates reproducible staining of CD99 and translocations of the EWS gene. Historically, this diagnosis was associated with near certain metastasis and subsequent mortality. However, current management consists of extensive chemotherapy in addition to local control with surgical resection and/or radiation. As a result, survival has improved to the 55-75% range in those patients who present without known metastases. Current research aims to continue this improvement by looking further into the associated gene abnormalities and possibly targeted therapies.

Related: Ewing's Sarcoma

Moore DD, Luu HH
Cancer Treat Res. 2014; 162:65-92 [PubMed] Related Publications
Osteosarcoma is a malignant tumor that primarily affects the long bones but can also involve other bones in the body.  It has a bimodal distribution with peaks in the second decade of life and late adulthood.  This chapter will highlight the clinical presentation, diagnosis, and treatment of osteosarcoma.

Related: Osteosarcoma

Xia T, Guan Y, Chen Y, Li J
Askin tumor: CT and FDG-PET/CT imaging findings and follow-up.
Medicine (Baltimore). 2014; 93(6):e42 [PubMed] Related Publications
The aim of the study was to describe the imaging findings of Askin tumors on computed tomography (CT) and fluorine 18 fluorodeoxyglucose-positron emission tomography (FDG-PET/CT).Seventeen cases of Askin tumors confirmed by histopathology were retrospectively analyzed in terms of CT (17 cases) and FDG-PET/CT data (6 cases).Fifteen of the tumors were located in the chest wall and the other 2 were in the anterior middle mediastinum. Of the 15 chest wall cases, 13 demonstrated irregular, heterogeneous soft tissue masses with cystic degeneration and necrosis, and 2 demonstrated homogeneous soft tissue masses on unenhanced CT scans. Two mediastinal tumors demonstrated the irregular, heterogeneous soft tissue masses. Calcifications were found in 2 tumors. The tumors demonstrated heterogeneously enhancement in 16 cases and homogeneous enhancement in 1 case on contrast-enhanced scans. FDG-PET/CT images revealed increased metabolic activity in all 6 cases undergone FDG-PET/CT scan, and the lesion SUVmax ranged from 4.0 to 18.6. At initial diagnosis, CT and FDG-PET/CT scans revealed rib destruction in 9 cases, pleural effusion in 9 cases, and lung metastasis in 1 case. At follow-up, 12 cases showed recurrence and/or metastases, 4 cases showed improvement or remained stable, and 1 was lost to follow-up.In summary, CT and FDG-PET/CT images of Askin tumors showed heterogeneous soft tissue masses in the chest wall and the mediastinum, accompanied by rib destruction, pleural effusion, and increased FDG uptake. CT and FDG-PET/CT imaging play important roles in the diagnosis and follow-up of patients with Askin tumors.

Related: Ewing's Sarcoma

Zhang FY, Tang W, Zhang ZZ, et al.
Systematic review of high-dose and standard-dose chemotherapies in the treatment of primary well-differentiated osteosarcoma.
Tumour Biol. 2014; 35(10):10419-27 [PubMed] Related Publications
The objective of this study is to evaluate whether high-dose chemotherapy is more efficacious than standard-dose chemotherapy in the treatment of primary well-differentiated osteosarcoma. The Cochrane systematic evaluation method was adopted. A database search was conducted in MEDLINE, Embase, OVID, the Cochrane Central Register of Controlled Trials database and the Chinese Biomedical Literature CD-ROM Database. The quality of the included studies was jointly evaluated by two reviewers, and homogeneous studies were included for meta-analysis. A total of five studies were included in this meta-analysis, with 1,415 subjects with primary, nonmetastatic, well-differentiated osteosarcoma in the limbs. No statistically significant differences were found between the high-dose chemotherapy group and the low-dose group in 5-year event-free survival [RR 1.04, 95 %CI (0.95, 1.13)], 5-year overall survival [RR 1.02, 95 %CI (0.95, 1.10)], local recurrence rate [RR 0.90, 95 %CI (0.59, 1.39)], proportion of subjects with good histological response [RR 0.93, 95 %CI (0.81, 1.07)], or limb salvage rate [RR 0.97, 95 %CI (0.92, 1.02)]. A statistically significant difference was observed in the 5-year event-free survival between the subjects with good histological response to preoperative chemotherapy and the subjects with poor histological response [RR 1.55, 95 %CI (1.19, 2.00), P < 0.001]. High-dose chemotherapy did not show superior efficacy to low-dose chemotherapy in the treatment of primary well-differentiated osteosarcoma. Further high-quality randomized controlled trials are needed to provide additional reliable evidence for our observation.

Related: Osteosarcoma

van Driel M, van Leeuwen JP
Cancer and bone: a complex complex.
Arch Biochem Biophys. 2014; 561:159-66 [PubMed] Related Publications
Primary and secondary bone cancers are rare events. However, once settled, a complex process is started involving an extensive amount of factors and interactions. The bone micro-environment is a preferential site for (metastatic) tumor cells to enter, stay, colonize and expand. The fact that the tumor cells affect the complete bone environment involving many cell types and regulatory pathways to stimulate their own growth and escape from therapy is devastating for the patient. Many efforts have been made to get more insight into the mechanisms underlying the communication between bone cells and cancer cells and progress is made in therapeutic interventions. This review will discuss the biological mechanisms of primary bone malignancies (osteosarcoma, Ewing's sarcoma, chondrosarcoma, multiple myeloma) and secondary bone malignancies (bone metastases) and therapeutic interventions.

Iwata S, Nakamura T, Gaston CL, et al.
Diaphyseal osteosarcomas have distinct clinical features from metaphyseal osteosarcomas.
Eur J Surg Oncol. 2014; 40(9):1095-100 [PubMed] Related Publications
AIMS: The aim of this study was to clarify the clinical features and outcomes of diaphyseal osteosarcoma.
METHODS: Patients with newly-diagnosed high-grade osteosarcoma occurring in the long bone were eligible for this retrospective study. Clinicopathological information was collected from our database and compared with 36 diaphyseal, 405 proximal and 519 distal metaphyseal, and 14 whole bone osteosarcoma patients. Additionally, case-control study matching by age, gender, site, and metastatic status at diagnosis with 1:3 ratio of 36 diaphyseal to 108 metaphyseal osteosarcomas patients was also conducted.
RESULTS: Five-year overall survival and metastasis-free survival of the three groups including diaphyseal, metaphyseal, and whole bone osteosarcoma patients showed significant difference (P = .029 and P = .013, respectively), although there is no difference for the survivals between proximal and distal metaphyseal osteosarcoma patients. Case-control study showed that patients with diaphyseal osteosarcomas had a significantly larger tumour (mean 13.5 cm vs 10 cm, P = .026), and demonstrated higher pathologic fracture rate (28% vs 12%, P = .033), superior 5-year metastasis-free survival (74% vs 40%, P = .0068), and slightly better 5-year overall survival (68% vs 46%, P = .074). Prognostic factor analysis showed that a pathologic fracture significantly decreased the survival of the patients with diaphyseal osteosarcoma.
CONCLUSIONS: The current study showed that diaphyseal osteosarcoma has distinct clinical features from metaphyseal osteosarcoma having an increased risk of pathologic fractures but with favorable survival outcome.

Related: Osteosarcoma

Casey DL, Alektiar KM, Gerber NK, Wolden SL
Whole lung irradiation for adults with pulmonary metastases from Ewing sarcoma.
Int J Radiat Oncol Biol Phys. 2014; 89(5):1069-75 [PubMed] Related Publications
PURPOSE: To evaluate feasibility and patterns of failure in adult patients with Ewing sarcoma (ES) treated with whole lung irradiation (WLI) for pulmonary metastases.
METHODS AND MATERIALS: Retrospective review of all ES patients treated at age 18 or older with 12-15 Gy WLI for pulmonary metastases at a single institution between 1990 and 2014. Twenty-six patients met the study criteria.
RESULTS: The median age at WLI was 23 years (range, 18-40). The median follow-up time of the surviving patients was 3.8 years (range, 1.0-9.6). The 3-year cumulative incidence of pulmonary relapse (PR) was 55%, with a 3-year cumulative incidence of PR as the site of first relapse of 42%. The 3-year event-free survival (EFS) and overall survival (OS) were 38 and 45%, respectively. Patients with exclusively pulmonary metastases had better outcomes than did those with extrapulmonary metastases: the 3-year PR was 45% in those with exclusively lung metastases versus 76% in those with extrapulmonary metastases (P=.01); the 3-year EFS was 49% versus 14% (P=.003); and the 3-year OS was 61% versus 13% (P=.009). Smoking status was a significant prognostic factor for EFS: the 3-year EFS was 61% in nonsmokers versus 11% in smokers (P=.04). Two patients experienced herpes zoster in the radiation field 6 and 12 weeks after radiation. No patients experienced pneumonitis or cardiac toxicity, and no significant acute or late sequelae were observed among the survivors.
CONCLUSION: WLI in adult patients with ES and lung metastases is well tolerated and is associated with freedom from PR of 45% at 3 years. Given its acceptable toxicity and potential therapeutic effect, WLI for pulmonary metastases in ES should be considered for adults, as it is in pediatric patients. All patients should be advised to quit smoking before receiving WLI.

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