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Danish Medical Journal Sep 2014Hereditary multiple cartilaginous exostoses is a syndrome characterised by the development of multiple osteochondromas. The diagnosis is typically made around the age of... (Review)
Review
INTRODUCTION
Hereditary multiple cartilaginous exostoses is a syndrome characterised by the development of multiple osteochondromas. The diagnosis is typically made around the age of 12 years, and the prevalence is estimated at 1:50,000. During skeletal growth, the osteochondromas are benign, but in adult life malignant transformation into chondrosarcomas can occur.
METHODS
This study was a literature survey based on a systematic search of the PubMed database for articles with the term "hereditary multiple exostoses chondrosarcoma". The search returned 157 articles, of which 13 had a sufficient level of evidence. These publications were examined thoroughly, focusing on the development of sarcomas, symptoms and the risk of malignant degeneration.
RESULTS
There is no consensus regarding the frequency of malignant transformation of multiple cartilaginous exostoses into sarcomas, which varies from less than 1% to 25%. The most reliable estimation seems to be 1-2%. The survey of the literature shows that no risk groups can be identified. However, exostoses in the axial skeleton are more prone to develop into chondrosarcomas than peripheral exostoses.
CONCLUSION
It is indisputable that malignant transformation occurs, and we therefore propose that a follow-up programme be launched with clinical examination by magnetic resonance imaging or bone scintigraphy every second year. The purpose of such programme would be to discover the sarcomatous development as early as possible to improve the survival prognosis of the patients. This screening programme should be centralised at tumour departments.
Topics: Bone Neoplasms; Cell Transformation, Neoplastic; Chondrosarcoma; Early Detection of Cancer; Exostoses, Multiple Hereditary; Humans; Precancerous Conditions
PubMed: 25186537
DOI: No ID Found -
Cancer Treatment Reviews Nov 2016Patients with advanced lung cancer commonly have bone metastases. Compared with other malignancies, the use of bone-targeted agents (e.g. bisphosphonates and denosumab)... (Review)
Review
BACKGROUND
Patients with advanced lung cancer commonly have bone metastases. Compared with other malignancies, the use of bone-targeted agents (e.g. bisphosphonates and denosumab) is less common in lung cancer patients. This may be due to the perception that bone-targeted agents are less effective in this population.
OBJECTIVE
To perform a systematic review to evaluate data from randomized trials of bone-targeted agents in lung cancer patients with bone metastases.
METHODS
A systematic search of Medline, Embase and the Cochrane Register of Controlled Trials through May 2015 was performed. Randomized trials of bone-targeted therapies in lung cancer patients with bone metastases were sought. Outcomes studied included skeletal related events (SREs), pain, quality of life, progression-free survival and overall survival. Random effects meta-analyses were planned if studies were judged homogeneous.
RESULTS
Of 632 abstracts, 17 publications describing 13 studies were included. Sample sizes ranged between 50 and 1776. Of 3379 patients, 1903 had lung cancer, with subgroup data available for 8 of 13 studies. Patient demographics were comparable, but enrollment criteria and endpoints were heterogeneous across studies, precluding meta-analysis. Study-specific results suggested that bone-modifying agents reduce the incidence of SREs and bone pain in lung cancer patients. Three studies suggested a survival benefit.
CONCLUSION
Data from included trials suggests benefit of bone-targeted agents in lung cancer for the prevention of SREs and bone pain. There is a trend toward improvement in overall survival and progression-free survival, although further research is needed. Impact on quality of life and key subgroups for benefit both require future research.
Topics: Bone Density Conservation Agents; Bone Neoplasms; Carcinoma, Non-Small-Cell Lung; Denosumab; Diphosphonates; Disease-Free Survival; Fractures, Spontaneous; Humans; Lung Neoplasms; Randomized Controlled Trials as Topic; Spinal Cord Compression; Spinal Neoplasms
PubMed: 27716496
DOI: 10.1016/j.ctrv.2016.09.013 -
European Archives of... Aug 2022Hemangiopericytoma is a rare tumor of the sino-nasal tract. Its clinical behavior is controversial. Whereas some describe an indolent course, others consider it to be an...
BACKGROUND
Hemangiopericytoma is a rare tumor of the sino-nasal tract. Its clinical behavior is controversial. Whereas some describe an indolent course, others consider it to be an aggressive lesion with a tendency toward rapid local recurrence. Here, we describe our experience in the management of sino-nasal hemangiopericytoma (SN-HPC), comparing our experience with the current literature, and evaluating signs and tools to improve diagnosis and treatment.
METHODS
All cases of SN-HPC between 2010 and 2020 were extracted and reviewed from our institutional electronic medical records. SN-HPC cases from PubMed and EMBASE between 2010 and 2020 were analyzed in a systematic literature review using the preferred reporting items for systematic review and meta-analysis (PRISMA) guidelines. Data regarding demographics, presentation, diagnosis, treatment, and outcome were collected.
RESULTS
We identified four cases of SN-HPC in the nasal cavity in our institution and an additional 53 cases in previous reports. The mean age at the time of diagnosis was 59 years, with a 1.2:1 male to female ratio. SN-HPC mostly appears unilaterally, arising in the ethmoid sinus (42.1%). The most common presenting symptoms were epistaxis (47.3) and nasal obstruction (47.3%). Both computed tomography (CT) and magnetic resonance imaging (MRI) were required for diagnosis and for tailoring the treatment plan. Endoscopic surgical excision was used in 85.9% of the patients, and in 15.7%, an additional preoperative embolization was performed, which was associated with septal necrosis in one patient (2.6%). The recurrence rate was 7%.
CONCLUSION
Although previous reports attribute an aggressive tumoral behavior to SN-HPC, our experience and the literature review support a more indolent course with low recurrence rates following complete endoscopic resection. Preoperative embolization can be useful in certain cases, but due to potential complications, it should not be routinely indicated.
Topics: Ethmoid Sinus; Female; Hemangiopericytoma; Humans; Male; Nasal Cavity; Nose; Nose Neoplasms
PubMed: 35103868
DOI: 10.1007/s00405-021-07239-w -
Frontiers in Bioscience (Landmark... Apr 2022Primary bone cancers are rare malignant diseases with significant morbidity and mortality. The treatment regimen relies on a combination of surgery (often involving...
Primary bone cancers are rare malignant diseases with significant morbidity and mortality. The treatment regimen relies on a combination of surgery (often involving amputation), chemotherapy and radiotherapy with outcomes dependent on localization of the tumour, grade, size and response to chemotherapy. Both treatment options and survival statistics have remained constant over the past 40 years and alternative therapies need to be explored. Purinergic signalling involving the interaction of extracellular nucleotides with P2 receptors has been investigated in numerous cancers with activation or inhibition a topic of debate. To assess whether purinergic signalling could be a viable target in primary bone cancer a systematic review for relevant primary literature published in PubMed, MEDLINE and Web of Science was performed. Search terms were formulated around three separate distinct topics; expression of P2 receptors in primary bone cancer models, P2 receptor signalling pathways involved and the functional consequences of P2 receptor signalling. Searching identified 30 primary articles after screening and eligibility assessments. This review highlights the diverse expression, signalling pathways and functional roles associated with different P2 receptors in primary bone cancers and provides a systematic summary of which P2 receptors are exciting targets to treat primary bone cancer and its associated symptoms.
Topics: Bone Neoplasms; Humans; Nucleotides; Signal Transduction
PubMed: 35468681
DOI: 10.31083/j.fbl2704122 -
Radiotherapy and Oncology : Journal of... Jan 2024Advances in characterizing cancer biology and the growing availability of novel targeted agents and immune therapeutics have significantly changed the prognosis of many... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND PURPOSE
Advances in characterizing cancer biology and the growing availability of novel targeted agents and immune therapeutics have significantly changed the prognosis of many patients with metastatic disease. Palliative radiotherapy needs to adapt to these developments. In this study, we summarize the available evidence for stereotactic body radiotherapy (SBRT) in the treatment of spinal metastases.
MATERIALS AND METHODS
A systematic review and meta-analysis was performed using PRISMA methodology, including publications from January 2005 to September 2021, with the exception of the randomized phase III trial RTOG-0631 which was added in April 2023. Re-irradiation was excluded. For meta-analysis, a random-effects model was used to pool the data. Heterogeneity was assessed with the I-test, assuming substantial and considerable as I > 50 % and I > 75 %, respectively. A p-value < 0.05 was considered statistically significant.
RESULTS
A total of 69 studies assessing the outcomes of 7236 metastases in 5736 patients were analyzed. SBRT for spine metastases showed high efficacy, with a pooled overall pain response rate of 83 % (95 % confidence interval [CI] 68 %-94 %), pooled complete pain response of 36 % (95 % CI: 20 %-53 %), and 1-year local control rate of 94 % (95 % CI: 86 %-99 %), although with high levels of heterogeneity among studies (I = 93 %, I = 86 %, and 86 %, respectively). Furthermore, SBRT was safe, with a pooled vertebral fracture rate of 9 % (95 % CI: 4 %-16 %), pooled radiation induced myelopathy rate of 0 % (95 % CI 0-2 %), and pooled pain flare rate of 6 % (95 % CI: 3 %-17 %), although with mixed levels of heterogeneity among the studies (I = 92 %, I = 0 %, and 95 %, respectively). Only 1.7 % of vertebral fractures required surgical stabilization.
CONCLUSION
Spine SBRT is characterized by a favorable efficacy and safety profile, providing durable results for pain control and disease control, which is particularly relevant for oligometastatic patients.
Topics: Humans; Radiosurgery; Spinal Neoplasms; Prognosis; Spine; Spinal Fractures; Pain; Clinical Trials, Phase III as Topic; Randomized Controlled Trials as Topic
PubMed: 37922993
DOI: 10.1016/j.radonc.2023.109969 -
World Neurosurgery Jul 2016Hepatocellular carcinoma (HCC) frequently metastasizes to the spine. The impact of medical and/or surgical intervention on overall survival has been examined in a... (Review)
Review
BACKGROUND
Hepatocellular carcinoma (HCC) frequently metastasizes to the spine. The impact of medical and/or surgical intervention on overall survival has been examined in a limited number of clinical studies, and herein we systematically review these data.
METHODS
We performed a literature review using PubMed, Embase, CINAHL, and Web of Science to identify articles that reported survival, clinical outcomes, and/or prognostic factors associated with patients diagnosed with spinal metastases. The methodologic quality of each review was assessed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses tool.
RESULTS
There were 26 articles (152 patients) that met the inclusion criteria and were treated with either surgery, radiotherapy, chemotherapy, and/or observation. There were 3 retrospective cohort studies, 17 case reports, 5 case series, and 1 longitudinal observational study. Of the patients with known overall survival after diagnosis of spinal metastasis, survival at 3 months, 6 months, 1 year, 2 years, and 5 years was 95.2%, 83.0%, 28.6%, 2.0%, and 1.4%, respectively. The median survival after diagnosis of the metastasis was 0.7 months in the patients who received no treatment, 7 months in the patients treated with surgical intervention alone, 6 months for patients who received chemotherapy and/or radiation, and 13.5 months in the patients treated with a combination of surgery and medical management. All other clinical or prognostic parameters were of low or insufficient strength.
CONCLUSIONS
Patients diagnosed with HCC spinal metastasis have a 10.6-month overall survival. Further analysis of patients in prospective controlled trials will be essential to the development of treatment algorithms for these patients in the future.
Topics: Carcinoma, Hepatocellular; Female; Humans; Liver Neoplasms; Male; Middle Aged; Spinal Neoplasms; Survival Analysis; Treatment Outcome
PubMed: 27090971
DOI: 10.1016/j.wneu.2016.04.026 -
Cancer Biotherapy & Radiopharmaceuticals Jun 2021Painful metastatic bone involvement is common in advanced stages of many cancers. Between available radionuclides for bone pain palliation, no consensus has been... (Meta-Analysis)
Meta-Analysis
Painful metastatic bone involvement is common in advanced stages of many cancers. Between available radionuclides for bone pain palliation, no consensus has been reached on lutetium ethylenediaminetetramethylene phosphonate (Lu-EDTMP) administration in this milieu. The aim of this study is to evaluate the treatment efficacy, safety profile, and toxicities of Lu-EDTMP in patients with metastatic bone involvement, according to the published literature. A comprehensive literature search of PubMed/MEDLINE, Scopus, and Google Scholar databases was carried out to retrieve pertinent articles published until January 2019, concerning the clinical efficacy and safety of Lu-EDTMP for bone pain palliative purposes. Eight studies (172 patients) were included. This analysis revealed statistically significant effect of Lu-EDTMP therapy on the visual analog score (4.84% (95% CI: 3.88-5.81; < 0.001), bone palliative pain response (84%, 95% CI: 75%-90%; < 0.001), and Karnofsky performance status (21%, 95% CI: 18%-24%; < 0.001) overall (as well as in the high-dose and low-dose subgroups). Complete palliative pain response to treatment was observed in 32% (95% CI: 16%-53%) of patients receiving Lu-EDTMP. Anemia was found to be the most common hematologic toxicity imposed by this therapeutic approach (grade I/II anemia in 24% (95% CI: 14%-38%; < 0.001) and grade III/IV anemia in 19% (95% CI: 12%-28%; < 0.001)). Lu-EDTMP seems to have comparable efficacy and safety profile as that of the frequently administered radiopharmaceuticals for bone palliation. Therefore, this agent can be a good option for bone pain palliative purposes, in case of limited access to other bone palliative radiopharmaceuticals.
Topics: Anemia; Bone Neoplasms; Humans; Karnofsky Performance Status; Leukopenia; Organometallic Compounds; Organophosphorus Compounds; Pain; Pain Measurement; Palliative Care; Radiopharmaceuticals; Thrombocytopenia
PubMed: 33259726
DOI: 10.1089/cbr.2020.4323 -
The International Journal of Biological... Mar 2023Numerous studies have reported the clinical value of alkaline phosphatase (ALP) and its bone-specific isoforms (bone-specific alkaline phosphatase (BAP)) in breast... (Meta-Analysis)
Meta-Analysis Review
Numerous studies have reported the clinical value of alkaline phosphatase (ALP) and its bone-specific isoforms (bone-specific alkaline phosphatase (BAP)) in breast cancer. The purpose of this meta-analysis was to summarize the prognostic value of serum ALP and BAP in breast cancer, especially focused on bone metastasis and survival. PRISMA guidelines were followed to conduct this review. Observational studies were searched in PubMed, Cochcrane Library and EMBASE to January 1, 2022. Data were extracted to explore the prognostic value of ALP and BAP. The quality of the included studies was assessed and the outcome effects were evaluated. Subgroup and sensitivity analyses were performed to explore the potential sources of heterogeneity. Publication bias was assessed. There was a total of 53 studies with 22,436 patients included. For the primary outcome of survival, high levels of both ALP and BAP were associated with short survival time. The hazard ratio of high ALP level on overall survival was 1.72 (95% CI 1.37, 2.16, < 0.001). For the secondary outcomes, a high ALP level (not BAP) was detected in breast cancer compared with healthy controls, and high levels of both ALP and BAP were risk factors for bone metastasis, while ALP (not BAP) was a risk factor for non-bone metastasis. This study showed that high levels of both serum ALP and BAP were associated with metastasis (BAP was associated with bone metastasis) and survival in breast cancer. The biomarkers could provide useful information for the early diagnostic assessment and monitoring in the follow-up of breast cancer patients.
Topics: Humans; Female; Alkaline Phosphatase; Prognosis; Breast Neoplasms; Biomarkers; Bone Neoplasms
PubMed: 36775971
DOI: 10.1177/03936155231154662 -
Journal of Veterinary Internal Medicine 2015Osteosarcoma is a malignant mesenchymal neoplasm that accounts for the majority of primary bone tumors in dogs and shares biological and clinical similarities with... (Review)
Review
Osteosarcoma is a malignant mesenchymal neoplasm that accounts for the majority of primary bone tumors in dogs and shares biological and clinical similarities with osteosarcoma in humans. Despite dose intensification with conventional cytotoxic therapies, survival times for dogs and humans diagnosed with high-grade osteosarcoma have not changed in the past 20 years, with the principal cause of mortality being the development of pulmonary metastases. Given the therapeutic plateau reached for delaying metastatic progression with cytotoxic agents, exploration of alterative adjuvant therapies for improving management of osteosarcoma micrometastases is clinically justified. Evidence suggests that osteosarcoma is an immunogenic tumor, and development of immunotherapies for the treatment of microscopic lung metastases might improve long-term outcomes. In this review, the history and foundational knowledge of immune interactions to canine osteosarcoma are highlighted. In parallel, immunotherapeutic strategies that have been explored for the treatment of canine osteosarcoma are summarized. With a greater understanding and awareness for how the immune system might be redirected toward combating osteosarcoma metastases, the rational development of diverse immune strategies for managing osteosarcoma holds substantial promise for transforming the therapeutic landscape and improving disease management in both dogs and human beings.
Topics: Animals; Bone Neoplasms; Dog Diseases; Dogs; Immunity, Cellular; Immunity, Humoral; Immunotherapy; Osteosarcoma
PubMed: 25929293
DOI: 10.1111/jvim.12603 -
Skeletal Radiology Aug 2019To review the efficacy of percutaneous thermal ablation (TA) of bone metastases (radiofrequency ablation [RFA], microwave ablation [MWA], cryoablation [CA], and... (Review)
Review
OBJECTIVE
To review the efficacy of percutaneous thermal ablation (TA) of bone metastases (radiofrequency ablation [RFA], microwave ablation [MWA], cryoablation [CA], and MR-guided focused ultrasound [MRgFUS]) in reducing pain in patients with advanced stage cancer.
MATERIALS AND METHODS
We searched MEDLINE/PubMed, MEDLINE In-Process, BIDS ISI, Embase, CINAHL, and the Cochrane database using the keywords "ablation," "painful," "bone," and "metastases" combined in multiple algorithms. Inclusion criteria were: original clinical studies published between 2001 and 2018; performance of RFA, MWA, CA or MRgFUS; and quantitative pain assessment before/after TA of bone metastasis.
RESULTS
Eleven papers (3 on RFA, 1 on MWA, 2 on CA, and 5 on MRgFUS) involving 364 patients were reviewed. A technical success rate of 96-100% was reported, with follow-up for up to 6 months. At baseline, pain scores ranged from 5.4 to 8, at 1-4 weeks from 0.5 to 5, and at 12 weeks from 0.3 to 4.5. Mean pain reduction compared with baseline ranged from 26 to 91% at 4 weeks and from 16% to 95% at 12 weeks. MWA treatments caused no complications, whereas MRgFUS showed the highest complication rate. The number of minor complications observed ranged from 0 to 59 (complication ratio 0-1.17), whereas the number of significant adverse effects ranged from 0 to 4 (complication ratio 0-0.04).
CONCLUSION
All techniques achieved pain relief after 1 and 3 months, in up to 91% and 95% of patients respectively. MWA showed a negligible complication rate, whereas MRgFUS is associated with a noteworthy rate of adverse events. Future studies should adopt a standardized pain reporting scale to allow for meta-analysis.
Topics: Ablation Techniques; Bone Neoplasms; Cancer Pain; Humans; Musculoskeletal Pain
PubMed: 30627778
DOI: 10.1007/s00256-018-3140-0