scholarly journals Primary Synovial Sarcomas of the Mediastinum: A Systematic Review and Pooled Analysis of the Published Literature

ISRN Oncology ◽  
2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Samer Salah ◽  
Ahmed Salem

Background. The aim of this systematic review is to attempt to provide a descriptive analysis for cases of synovial sarcoma (SS) arising in the mediastinum and to analyze prognostic factors. Methods. We performed PubMed database search in July 2013. Twenty-two studies, which included 40 patients, form the basis of this review. Demographic and disease-related factors were analyzed for possible influence on survival. Findings were compared with extremity SS studies reported in literature. Results. Sixteen cases (40%) presented with locally advanced unresectable disease, 2 (5%) with metastatic disease, and 22 (55%) with localized resectable disease. Median tumor size was 11 cm (range: 5–20 cm). Thirty patients were assessable for survival and had a 5-year OS of 36%. Completeness of resection was the only factor associated with significant improvement in OS (5-year survival of 63% and 0% in favor of complete resection, P=0.003). Conclusion. Mediastinal SS is associated with poor prognosis as more cases are diagnosed at an advanced stage and with larger tumor size compared to extremity SS. Complete surgical resection is the only identified factor associated with better prognosis and may result in survival outcomes that are comparable with those for localized SS of the extremity.

2020 ◽  
Vol 27 (3) ◽  
pp. 107327482093482 ◽  
Author(s):  
Josef Yayan

Background: Denosumab is a human monoclonal antibody that has been used successfully in the treatment of giant cell tumors of bone. These tumors are rare and, in principle, benign, but they are highly aggressive, locally advanced, osteolytic bone tumors that can metastasize to the lungs. Denosumab is an effective treatment when these tumors cannot be surgically removed or when surgical resection is likely to lead to severe morbidity (eg, loss of limbs or joints). The aim of this systematic review and meta-analysis was to investigate patients with giant cell tumors of bone who experienced tumor progression during treatment with denosumab and to compare them with patients who experienced reduction of their giant cell tumors of bone during treatment with denosumab. Methods: Embase, Cochrane Library, and MEDLINE/PubMed databases were searched for trials submitted by January 7, 2020, that reported the efficacy and safety of denosumab in patients with giant cell tumors of bone. Results: Sixty studies were reviewed, involving a total of 1074 patients who had giant cell tumors of bone and were treated with denosumab. Of the 60 studies, 58% of the patients were from case series studies, 39% from open-label phase II studies, and 3% from case reports. The response rate for denosumab as a treatment for giant cell tumors of bone was 97.5%, with statistical significance ( P < .0001). Pain in the limbs was statistically the most common adverse event for denosumab treatment in case series studies ( P < .0001). No treatment-related deaths occurred in the reviewed studies. Conclusion: Cumulative evidence supports the addition of surgery to optimal medical therapy with denosumab to reduce tumor size, clinical symptoms, and mortality among patients with giant cell tumors of bone.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 10603-10603
Author(s):  
M. Gaui ◽  
G. Amorim ◽  
R. Arcuri ◽  
G. Pereira ◽  
D. Moreira ◽  
...  

10603 Background: In Brazil, LABC remains a serious health problem, representing approx. 30% of all newly diagnosed breast cancers. Anthracycline-based neoadjuvant therapy is a standard treatment, but around 30% of pts do not respond. There is no standard approach in these refractory pts. In our institution, these pts receive RT, but almost half (46%) of pts are not rendered operable by RT alone. Because X is the reference treatment for anthracycline- and/or taxane-pretreated MBC and X and RT are supra-additive in in vivo models, there is a clear rationale for evaluating X-RT in this setting. Methods: Pts eligible for this prospective, open-label, single-center, non-randomized study had inoperable LABC refractory to FAC, ECOG PS ≥1 and adequate organ functions. Pts received RT 20c Gy/d ×5w (total dose 50Gy) plus X 850mg/m2 bid orally d1–14, q3w for 2 cycles. Pts underwent surgery, if possible, after completion of neoadjuvant therapy. Pts with hormone receptor-positive tumors received tamoxifen after surgery. Results: Baseline characteristics of the 30 pts enrolled between Jan 2003 and May 2004: median age 47 years (range, 26–70); median ECOG PS 1; median tumor size (after anthracyclines) 80 cm2 (range, 36–357cm2); tumor grade II/III/unknown 20/37/43%; inflammatory carcinoma 21%. Two pts were excluded from this analysis as they received the incorrect X dosing regimen. 23/28 evaluable pts (82%) were operable after X-RT; 4 (14%) pts did not undergo surgery because of disease progression. Median tumor size after X-RT was 49 cm2 (range, 6–196 cm2). The median relative reduction in tumor size was 33%. After surgery, median residual tumor size was 12 cm2 (range, 0−72) and the median number of positive nodes was 2 (range, 0−27). Pathological complete response was observed in 3 (11%) pts. The most common treatment-related adverse events (all grade 1/2) were mucositis, diarrhea, nausea and emesis. There were no grade 3/4 events. Conclusions: Our data indicate that neoadjuvant X-RT is feasible, well tolerated and appears more effective than RT alone in pts with LABC refractory to FAC, rendering a high proportion (82%) of pts eligible for surgery. No significant financial relationships to disclose.


2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
Author(s):  
Andre S. Giorelli ◽  
Pâmela Passos ◽  
Thiago Carnaval ◽  
Marleide da Mota Gomes

Background. Sleep complaints are common in patients with epilepsy (PWE). Excessive daytime sleepiness (EDS) is one of the most reported complaints and its impact is still a matter of debate. Objective. Evaluate the relationship between EDS and epilepsy, with emphasis on prevalence, assessment, and causes. Methods. A systematic review on PubMed database in the last 10 years (2002 to 2012). The search returned 53 articles and 34 were considered relevant. After citation analysis, 3 more articles were included. Results. Most studies were cross-sectional and questionnaire based. 14 papers addressed EDS as the primary endpoint. 14 adult and 3 children studies used subjective and objective analysis as methodology. The number of studies increased throughout the decade, with 21 in the last 5 years. Adult studies represent almost three times the number of children studies. EDS prevalence in PWE varies from 10 to 47.5%. Prevalence was higher in developing countries. Conclusion. EDS seems to be related more frequently to undiagnosed sleep disorders than to epilepsy-related factors, and although it affects the quality of life of PWE, it can be improved by treating comorbid primary sleep disorders.


2021 ◽  
Author(s):  
Moritz L Schmidbauer ◽  
Caroline Ferse ◽  
Farid Salih ◽  
Carsten Klingner ◽  
Rita Musleh ◽  
...  

Abstract Introduction: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) profoundly impacts on hemostasis and microvasculature. Correspondingly, antithrombotic therapy is frequently used for prophylaxis or treatment of thromboembolic complications as well as in the context of extracorporeal membrane oxygenation (ECMO). However, reports of intracranial hemorrhage (ICH) associated with Coronavirus disease 2019 (COVID-19) have also emerged. In the light of the dilemma between thromboembolic and hemorrhagic complications, we sought to systematically investigate incidence, mortality, radiological subtypes and clinical characteristics of ICH in COVID-19 patients.Methods: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, we performed a systematic review of literature by screening the PubMed database and included patients diagnosed with COVID-19 and concomitant ICH. Furthermore, we performed a pooled analysis including a prospectively collected cohort of critically ill COVID-19 patients with ICH as part of the PANDEMIC registry (Pooled Analysis of Neurologic Disorders Manifesting in Intensive care of COVID-19).Results: Our literature review revealed a total of 217 citations. After selection process, 79 studies and a total of 477 patients were included. Median age was 58.8 years (95% CI 54.8 years-62.9 years; I2 = 85,6%). 23.3% patients experienced critical stage of COVID-19 (95% CI 8.9% − 61.2%, I2 = 53.8%). 62.7% patients were on anticoagulation (95% CI 38.2% − 103.0%, I2 = 82,6%), and 27.5% patients received ECMO (95% CI 5.8% − 130.2%, I2 = 92.7%). Microbleeds (51.1%, 95% CI 31.1% − 84.2%, I2 = 85.1%), subarachnoid hemorrhage (SAH) (26.6%, 95% CI 16.8% − 42.0%, I2 = 61.2%) and intraparenchymal hemorrhage (IPH) (33.7%, 95% CI 23.3% − 48.8%, I2 = 63.7%) were most frequently documented as ICH subtypes. Incidence was at 0.85% (95% CI 0.36%-1.99%; I2 = 97.5%) and mortality at 52.18% (95% CI 40.40%-67.39%; I2 = 51.7%), respectively.Conclusion: ICH in COVID-19 patients is rare, but has a very poor prognosis. Different subtypes of ICH seen in COVID-19 support the assumption of heterogenous and multifactorial pathomechanisms contributing to ICH in COVID-19. Further data and pathophysiological insights are warranted to resolve the conflict between thromboembolic and hemorrhagic complications in the future.


2021 ◽  
Author(s):  
Sandeep Kumar ◽  
Anurag Ranjan Lila ◽  
Saba Samad Memon ◽  
Vijaya Sarathi ◽  
Virendra A Patil ◽  
...  

Objective: Risk of metastatic disease in the cluster 2-related pheochromocytoma/paraganglioma (PPGL) is low. In MEN2 patients, identification of origin of metastases from pheochromocytoma (PCC) or medullary thyroid carcinoma (MTC) is challenging as both are of neuroendocrine origin. We aim to describe our experience and perform a systematic review to assess prevalence, demographics, biochemistry, diagnostic evaluation, management and predictors of cluster 2-related metastatic PPGL. Design: Retrospective study with systematic review of literature. Methods: Data of 3 cases from our cohort and 43 cases from world literature were analyzed. For calculation of prevalence, all reported patients (n=3063) of cluster 2 were included. Results: The risk of metastasis in cluster 2-related PPGL was 2.6% (2% in RET, 5% in NF1, 4.8% in TMEM127 and 16.7% in MAX variation). In metastatic PCC in MEN2, median age was 39 years, bilateral tumors were present in 71% and median tumor size was 9.7 cm (range 4-19) with 43.5% mortality. All patients had primary tumor size ≥4cm. Origin of primary was diagnosed by histopathology of metastatic lesion in 11(57.9%), 131I-MIBG scan in 6(31.6%), and selective venous sampling and computed tomography in one (5.3%) each. In subgroup of NF1, median age was 46 years (range 14-59) with median tumor size 6cm and 57% mortality. Conclusion: The risk of metastatic disease in cluster 2-related PPGL is 2.6%, being especially high in tumors with size ≥4cm and is associated with high mortality. One-third patients of NF1 with metastatic PPGL had presented in 2nd decade of life. Long-term studies are needed to formulate management recommendations.


2020 ◽  
pp. 1-7
Author(s):  
Maria Peraire Lores ◽  
Maria Peraire Lores ◽  
Jose Luis Bauza Quetglas ◽  
Laura Aizpiri Antoñana ◽  
Luis Ladaria Sureda ◽  
...  

Background: RENAL and PADUA scoring systems have been designed and validated as a method to assess the complexity of renal masses and predict the risk of perioperative complications. We aimed to evaluate if there is an association between RENAL and PADUA nephrometry scores with the Trifecta and Pentafecta achievement. Materials and Methods: We retrospectively analysed the data from 102 patients with renal cell carcinoma who underwent partial nephrectomy from January 2011 to October 2018 at our institution. Radiological characteristics of the renal masses were scored according to the RENAL and PADUA classification. Trifecta and Pentafecta achievement were collected. We performed a descriptive analysis and used de χ2 test to evaluate the relationship between PADUA and RENAL scores and Trifecta and Pentafecta achievement. Result: Among 102 patients, the median tumor size was 2.7 cm (IQR 0.8-7.5), the median RENAL score was 7 (IQR 4-11) and PADUA score 8 (IQR 6-14). The overall rate of postoperative complications was 21.6% (n=22). The rates of Trifecta and Pentafecta achievement were 50% (n=51/102) and 46.1% (n=47/102), respectively. Trifecta and Pentafecta achievement were higher when the PADUA score was ≤10 (OR 3.62; IC95% (1.08-12.11); p=0.0317) and (OR 4.98; IC95% (1.32-18.7); p=0.0175), respectively. Likewise, Trifecta and Pentafecta achievement were higher in patients with RENAL score ≤ 8 (OR 4.09; IC95% (1.46-11.42); p=0.0072) and (OR 4.92; IC95% (1.66-14.51); p=0.0039), respectively. Conclusion: There is an association between the RENAL and PADUA nephrometry scores and the Trifecta and Pentafecta achievement.


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