Infantile myofibromatosis: Excellent prognosis but also rare fatal progressive disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry

2021 ◽  
Author(s):  
Monika Sparber‐Sauer ◽  
Christian Vokuhl ◽  
Guido Seitz ◽  
Benjamin Sorg ◽  
Möllers Tobias ◽  
...  
2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 5038-5038 ◽  
Author(s):  
M. P. Sablin ◽  
L. Bouaita ◽  
C. Balleyguier ◽  
J. Gautier ◽  
C. Celier ◽  
...  

5038 Background: Sorafenib (So) and sunitinib (Su) have been recently approved for the treatment of advanced renal cell carcinoma (RCC). Following this approval, sequential use of both drugs is often proposed, although very few data are available to support it. Material and Methods: We reviewed all patients (pts) who received sequentially So-Su or Su-So in the last 3 years from 4 sites in France. All pts had been enrolled in clinical studies or in the extended access programs. In all patients demography, prognostic factors (MSKCC score), number of metastatic sites, best response (BR) and PFS with each treatment were recorded. Some patients are still too early for response and PFS assessment under the second treatment. Results: 68 pts first received So while 22 pts received Su first (continuous dosing in 12, intermittent in 10). Results under the first treatments were as follows: median PFS 26.1 wks, mean duration of treatment 33.2 wks; and median PFS was 22 wks, mean duration of treatment 26.9 wks for So and Su respectively. BR in terms of partial response (PR), stable disease (SD), progressive disease (PD) is given in the table . 2 pts stopped So because of toxicity and response is not available (NA), and 22 pts (NE) are too early in the course of the second treatment. Overall, PR rate was 17.6% for So and 22.7% for Su. PR or SD was observed as best response in the second treatment in both sequences. Only 6 pts had PD with both drugs: all of them had 3 or more metastatic sites and were in intermediate or poor MSKCC risk groups. Conversely, 4 pts had PR with both drugs, and were in good (n=2) or intermediate (n=2) risk groups. 11 pts only have died so far. Conclusions: these results suggest the lack of cross resistance between So and Su, and support the sequential use of So and Su in RCC. Updated data will be presented at ASCO. [Table: see text] No significant financial relationships to disclose.


2018 ◽  
Vol 25 (5) ◽  
pp. 77-82 ◽  
Author(s):  
S. P. Rubnikovich ◽  
D. M. Borodin ◽  
Yu. L. Denisova ◽  
I. N. Baradina

Aim. The research was conducted to develop and implement the complex of diagnostic activities in patients with dysfunction of temporomandibular joints with signs of bruxism.Materials and methods. To achieve this aim we conducted a comprehensive diagnosis of 61 patients aged 20-29 years with bruxism and temporomandibular joints dysfunction (TMJD), the patients were divided into two groups. To diagnose and analyze the results of the disease treatment in patients we used a computer analysis with visualization of changes of the osteoarticular surface and parameters of the joint gap in the TMJ. Depending on the ratio of prognostic risk criteria and the development of bruxism, we distinguished the area of low, medium and high level of risk of disease progression.Results. Comparison of the treatment results of patients with bruxism from two groups in 12-24 months showed that patients from the second group had good treatment results. Patients of the second group underwent a complex of diagnostic activities and its data increased the treatment effectiveness. The results obtained from the treatment of patients from the first group indicate the lack of data from the standard examination of patients with bruxism.Conclusion. The developed complex diagnostic activities in patients with bruxism in combination with dysfunction of the TMJ and occlusive attrition of teeth allow to assess not only the local status but also to determine the functional state of dentoalveolar system and the cerebral cortex which affects the amount of therapeutic activities in the patient. 


Author(s):  
J.-P. Chippaux ◽  
A. N. Salas-Clavijo ◽  
J. R. Postigo ◽  
D. Schneider ◽  
J. A. Santalla ◽  
...  

1998 ◽  
Vol 84 (5) ◽  
pp. 558-561 ◽  
Author(s):  
Sergio Bretti ◽  
Alfredo Berruti ◽  
Luigi Dogliotti ◽  
Bruno Castagneto ◽  
Rossella Bertulli ◽  
...  

The Italian Group on Rare Tumors undertook a phase II study of a combination of epirubicin and interleukin-2 in 21 chemotherapy-naive patients with malignant mesothelioma. All patients had bidimensionally measurable disease at CT scan. Treatment included intravenous administration of epirubicin at a dose of 110 mg/m2 i.v. on day 1, and interleukin-2 at a dose of 9 MU subcutaneously from day 8 to day 12 and from day 15 to day 19. Cycles were repeated every three weeks, up to six times in the absence of progressive disease. Treatment response was evaluated after two cycles of therapy. Only one patient achieved a partial response, resulting in an overall response rate of 5% (1/21) with a median progression-free and overall survival of 5 and 10 months, respectively. Toxicity was relevant and caused treatment discontinuation in many patients. These results do not support the use of such a combination in the management of malignant mesothelioma.


2001 ◽  
Vol 6 (1) ◽  
pp. 1-3
Author(s):  
Robert H. Haralson

Abstract The AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), Fifth Edition, was published in November 2000 and contains major changes from its predecessor. In the Fourth Edition, all musculoskeletal evaluation and rating was described in a single chapter. In the Fifth Edition, this information has been divided into three separate chapters: Upper Extremity (13), Lower Extremity (14), and Spine (15). This article discusses changes in the spine chapter. The Models for rating spinal impairment now are called Methods. The AMA Guides, Fifth Edition, has reverted to standard terminology for spinal regions in the Diagnosis-related estimates (DRE) Method, and both it and the Range of Motion (ROM) Method now reference cervical, thoracic, and lumbar. Also, the language requiring the use of the DRE, rather than the ROM Method has been strengthened. The biggest change in the DRE Method is that evaluation should include the treatment results. Unfortunately, the Fourth Edition's philosophy regarding when and how to rate impairment using the DRE Model led to a number of problems, including the same rating of all patients with radiculopathy despite some true differences in outcomes. The term differentiator was abandoned and replaced with clinical findings. Significant changes were made in evaluation of patients with spinal cord injuries, and evaluators should become familiar with these and other changes in the Fifth Edition.


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