scholarly journals Rhabdomyosarcoma in Adults: A Retrospective Analysis of Case Records Diagnosed between 1979 and 2018 in Western Denmark

Sarcoma ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Vivi-Nelli Mäkinen ◽  
Akmal Safwat ◽  
Ninna Aggerholm-Pedersen

Introduction. Adult rhabdomyosarcoma is a rare tumour that has an inferior survival compared to the paediatric patient population. The reason for this consistently worse outcome remains mostly unknown. It has been suggested that this disparity may be related to biological and/or treatment-related factors, which in the literature has been shown to be distributed differently among paediatric and adult patients. The aim of this study was to clarify treatment outcome and clinicopathological factors for adult patients with rhabdomyosarcoma that were treated in Aarhus, Denmark, since 1979. Methods. By searching the Aarhus Sarcoma Registers, data for all rhabdomyosarcoma patients, aged 18 years or more, between 1979 and 2018, were retrieved and analysed. Results. Data from 50 patients were collected. No patients were lost to follow-up. For the entire cohort, 5- and 10-year overall survival rates were 30% and 18%, respectively. The median age was 46.5 years, and the median overall survival was 2.3 years. Tumour histology was embryonal 18%, alveolar 22%, pleomorphic 44%, and not otherwise specified 16%. The tumour site was unfavourable in more than 80% of the patients. Significant factors associated with inferior overall survival were histology and disease stage, although histological subtype was not significant in the multivariate model. Five-year overall survival was 40% for localised disease versus 15% for metastatic disease. Conclusion. Rhabdomyosarcoma in adults has a poorer prognosis than paediatric rhabdomyosarcoma and other high-grade sarcomas in adults. Adult rhabdomyosarcoma should continue to be treated aggressively, but new and tailored treatment strategies are needed to improve the long-term outcome. Previous predictors of poor survival in paediatric patients were valid in adults except for age, site (favourable versus unfavourable), and tumour size.


2013 ◽  
Vol 72 (Suppl 3) ◽  
pp. A736.1-A736
Author(s):  
D. Dimopoulou ◽  
M. Trachana ◽  
P. Sidiropoulos ◽  
P. Tsitsi ◽  
A. Moihovitou ◽  
...  




2016 ◽  
Vol 10 (1) ◽  
pp. 22-26 ◽  
Author(s):  
Salvatore Perrone ◽  
Alberto Rossetti ◽  
Patrick Sportiello ◽  
Pierfrancesco Mirabelli ◽  
Pierangela Cimatti ◽  
...  

Purpose: To report on the outcome of conventional therapy in patients with Coats’ disease. Methods: Retrospective analysis of the charts of thirteen patients with Coats’ disease. Results: Mean age of 9 male (70%) and 4 female (30%) patients was 17.7 (range, 5-33) years; one female had bilateral disease. Eleven eyes with retinal telangiectasia and exudation were treated with argon laser photocoagulation alone or photocoagulation associated with cryotherapy; the mean follow up was 32.5 (range,17-41) years. In four eyes without foveal involvement (stage 2a) the mean presenting visual acuity (VA) remained at 0.8 or improved, whereas poor VA in seven stage 2b eyes deteriorated minimally over time. In one and two of the three eyes with total retinal detachment, phthisis or neovascular glaucoma ensued. Conclusion: About three decades after conventional treatment of Coats’ disease stage 2a, treated eyes maintained good VA, and stage 2b eyes did not progress to advanced stages.



1996 ◽  
Vol 40 (3) ◽  
pp. 527-527
Author(s):  
Robert Dunckelmann ◽  
Joachim Pietz ◽  
André Rupp ◽  
Hildgund Schmidt ◽  
Hans J Bremer


Cancers ◽  
2019 ◽  
Vol 11 (10) ◽  
pp. 1503 ◽  
Author(s):  
Loosen ◽  
Schulze-Hagen ◽  
Bruners ◽  
Tacke ◽  
Trautwein ◽  
...  

: Background and Aims: While transarterial chemoembolization (TACE) represents a standard of therapy for intermediate-stage hepatocellular carcinoma (HCC) and is also routinely performed in patients with liver metastases, it is still debated which patients represent the ideal candidates for TACE therapy in terms of overall survival. Sarcopenia, the degenerative loss of skeletal muscle mass and strength, has been associated with an adverse outcome for various malignancies, but its role in the context of TACE has largely remained unknown. Here, we evaluated the role of sarcopenia on the outcome of patients undergoing TACE for primary and secondary liver cancer. Methods: The patients’ psoas muscle size was measured on axial computed tomography (CT) scans and normalized for the patients’ height squared. This value was referred to as the psoas muscle index (PMI). The PMI was correlated with clinical and laboratory markers. Results: While pre-interventional sarcopenia had no impact on the direct tumor response to TACE, sarcopenic patients with a pre-interventional PMI below our ideal cut-off value of 13.39 mm/m2 had a significantly impaired long-term outcome with a median overall survival of 491 days compared to 1291 days for patients with a high PMI. This finding was confirmed by uni- and multivariate Cox-regression analyses. Moreover, a progressive rapid decline in muscle mass after TACE was a predictor for an unfavorable prognosis. Conclusion: Our data suggest that sarcopenia represents a previously unrecognized prognostic factor for patients undergoing TACE therapy which might yield important information on the patients’ post-interventional outcome and should therefore be implemented into clinical stratification algorithms.



2018 ◽  
Vol 31 (Supplement_1) ◽  
pp. 22-22
Author(s):  
Kazuki Odagiri ◽  
Makoto Yamasaki ◽  
Koji Tanaka ◽  
Yasuhiro Miyazaki ◽  
Tomoki Makino ◽  
...  

Abstract Background Salvage Lymphadenectomy is regarded as the only curative surgery to residual or recurrence lymph nodes of esophageal cancer after definitive chemoradiotherapy (dCRT). However, salvage lymphadenectomy is not described in the Japanese esophageal cancer treatment guideline because of little evidences for the safety and efficacy. Methods From January 2011 to December 2015, we performed 14 salvage lymphadenectomies to residual or recurrence LN of esophageal squamous cell carcinoma(ESCC) in Osaka University. We assessed postoperative complications and long-term outcome. Results Average age was 64 year-olds (SD: 5.2). Male: Female = 11: 3. cStage I: II-IV = 7: 7. Surgery to cervical LN were 11 patients and abdominal LN were 3 patients. Surgery to residual LN (res-LN) were 9 patients and recurrence LN (rec-LN) were 5 patients. rec-LN patient's median time to recurrence after dCRT was 14.3 months (10.2–29.3). 4 patients were performed lymphadenectomy resecting with adjacent organs, 3 patients were bronchus (trachea? ) and 1 patient was right subclavian artery. 4 patients had postoperative complication, two were pneumonia, one was pulmonary thrombosis and one was lymphorrhea, but there was no serious case (Clavien-Dindo Grade II or less). We didn’t have hospital death. Six of 14 patients had recurrence and died after salvage lymphadenectomy. Recurrence sites were 2 mediastinal lymph nodes and liver, lung, loco-regional and peritoneal. But no patients had recurrence of main tumor. 5-year overall survival rate was 51.1%. Median survival time in 9 patients, surgery to res-LN, was 18.9 months (10.4–132 months) and 5 patients, surgery to rec-LN, was 4.9 months (1.4–26.6 months). Surgery to res-LN patients were longer than rec-LN patients in overall survival after salvage lymphadenectomy (P = 0.395). There was no difference due to the difference in recurrence site of the cancer in overall survival after salvage lymphadenectomy. Conclusion Our data show salvage lymphadenectomy safety and effectiveness after dCRT. Salvage lymphadenectomy may extend the prognosis of patients with esophageal cancer after dCRT. Thus, salvage lymphadenectomy may be one of the treatment options for the patients with residual or recurrent, especially the former, lymph node after definitive CRT, although it is necessary to evaluate in many cases. Disclosure All authors have declared no conflicts of interest.



Neurosurgery ◽  
1996 ◽  
Vol 38 (1) ◽  
pp. 51-59 ◽  
Author(s):  
Christian Raftopoulos ◽  
Nicolas Massager ◽  
Danielle Baleriaux ◽  
Jeanine Deleval ◽  
Stephane Clarysse ◽  
...  


2010 ◽  
Vol 28 (22) ◽  
pp. 3644-3652 ◽  
Author(s):  
Renato Bassan ◽  
Giuseppe Rossi ◽  
Enrico M. Pogliani ◽  
Eros Di Bona ◽  
Emanuele Angelucci ◽  
...  

Purpose Short imatinib pulses were added to chemotherapy to improve the long-term survival of adult patients with Philadelphia chromosome (Ph) –positive acute lymphoblastic leukemia (ALL), to optimize complete remission (CR) and stem-cell transplantation (SCT) rates. Patients and Methods Of 94 total patients (age range, 19 to 66 years), 35 represented the control cohort (ie, imatinib-negative [IM-negative] group), and 59 received imatinib 600 mg/d orally for 7 consecutive days (ie, imatinib-positive [IM-positive] group), starting from day 15 of chemotherapy course 1 and from 3 days before chemotherapy during courses 2 to 8. Patients in CR were eligible for allogeneic SCT or, alternatively, for high-dose therapy with autologous SCT followed by long-term maintenance with intermittent imatinib. Results CR and SCT rates were greater in the IM-positive group (CR: 92% v 80.5%; P = .08; allogeneic SCT: 63% v 39%; P = .041). At a median observation time of 5 years (range, 0.6 to 9.2 years), 22 patients in the IM-positive group versus five patients in the IM-negative group were alive in first CR (P = .037). Patients in the IM-positive group had significantly greater overall and disease-free survival probabilities (overall: 0.38 v 0.23; P = .009; disease free: 0.39 v 0.25; P = .044) and a lower incidence of relapse (P = .005). SCT-related mortality was 28% (ie, 15 of 54 patients), and postgraft survival probability was 0.46 overall. Conclusion This imatinib-based protocol improved long-term outcome of adult patients with Ph-positive ALL. With SCT, post-transplantation mortality and relapse remain the major hindrance to additional therapeutic improvement. Additional intensification of imatinib therapy should warrant a better molecular response and clinical outcome, both in patients selected for SCT and in those unable to undergo this procedure.



2006 ◽  
Vol 20 (4) ◽  
pp. 214-221 ◽  
Author(s):  
M. Tisell ◽  
P. Hellström ◽  
G. Ahl-Börjesson ◽  
G. Barrows ◽  
E. Blomsterwall ◽  
...  


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 747-747
Author(s):  
Shaji Kumar ◽  
Morie A Gertz ◽  
Martha Q. Lacy ◽  
Suzanne R. Hayman ◽  
Francis Buadi ◽  
...  

Abstract Abstract 747 Background: Light chain amyloidosis is a monoclonal plasma cell proliferative disorder characterized by multiorgan deposition of monoclonal light chain derived amyloid fibrils. The diffuse organ infiltration by the amyloid fibrils leads to various AL related clinical features. Therapy of AL has been primarily aimed at elimination or control of the monoclonal plasma cells, thus decreasing the light chain available for amyloid formation. Assessment of response to therapy has been grouped into hematological response demonstrating elimination or reduction of the monoclonal population and organ response demonstrating actual improvement in its structure or function. Given the potentially long delay in observing organ improvement, hematological response has been the surrogate short term marker for long term outcome given the relatively rapid decline seen in the monoclonal protein with effective therapy, as well as studies showing improved long term outcome with a hematological response, especially complete response. However, conventional hematological response criteria give priority to the intact monoclonal protein rather than the light chain, which is the amyloidogenic protein. We hypothesized that a light chain response will correlate better with outcome than changes in the intact monoclonal protein levels. Methods: We identified 348 patients who had undergone stem cell transplant (SCT) for AL from among a large group of patients undergoing different treatments for AL, and in whom serial tests results were available. We identified the lowest value attained following SCT for various hematological response parameters (intact serum M-protein [MP], the difference between involved and uninvolved serum free light chain [FLC-diff], and urine M-protein [UP]) before an alternate therapy was instituted. We first estimated the best cutoff for the degree of change in each parameter that predicted 5-year survival from SCT. Cox proportional hazards model was used for multivariable analysis of factors influencing overall survival (OS). Results: We first examined the relative contribution of the MP and the FLC-diff on the overall survival following transplant. The best cutoff for reduction in FLC-diff to predict OS at 5 years from SCT was 88% if all patients were considered and 90% if only those with a baseline FLC diff > 7.5 mg/dL were considered. The best cutoff for MP reduction was 40% for 5 year survival for all patients and 67% for those with a baseline MP > 1.0 gm/dL. We then looked at the impact of FLC reduction and MP reduction in a multivariable analysis using OS as endpoint. For all patients, FLC reduction, but not MP reduction significantly impacted outcome, and results were similar when considering patients with MP>1.0 gm/dl and FLC-diff > 7.5 mg/dl at baseline. Among those with a baseline FLC-diff >7.5 mg/dL (n=125), the overall survival was 35 mos from SCT for those with less than 90% decrease in FLC-diff (45 pts) compared to not reached for those with at least 90% decrease (80 pts); P < 0.001 (Figure). Conclusions: The current study supports the notion that reductions in the free light chain parameters represent a more useful measure of hematological response that translates into better overall survival and possible better chance at organ improvement. A reduction in the FLC difference of 90% appears to correlate best with prolonged survival. Disclosures: Kumar: CELGENE: Research Funding; MILLENNIUM: Research Funding; BAYER: Research Funding; GENZYME: Research Funding; NOVARTIS: Research Funding. Gertz:celgene: Honoraria; millenium: Honoraria, Membership on an entity's Board of Directors or advisory committees. Lacy:celgene: Research Funding.



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