scholarly journals Outcome in dedifferentiated chondrosarcoma for patients treated with multimodal therapy: Results from the EUROpean Bone Over 40 Sarcoma Study

2021 ◽  
Vol 151 ◽  
pp. 150-158
Author(s):  
Ivar Hompland ◽  
Stefano Ferrari ◽  
Stefan Bielack ◽  
Emanuela Palmerini ◽  
Kirsten S. Hall ◽  
...  
Keyword(s):  
Multimodal Therapy ◽  
Dedifferentiated Chondrosarcoma

Clival Chordomas: Long-Term Functional Outcome after Multimodal Therapy

Skull Base ◽  
2009 ◽  
Vol 19 (01) ◽  
Author(s):  
Hischam Bassiouni ◽  
S.A. Asgari ◽  
S.D. Dützmann ◽  
E.S. Sandalcioglu ◽  
G.M. Marquard ◽  
...  
Keyword(s):  
Functional Outcome ◽  
Multimodal Therapy

scholarly journals Enzyme replacement therapy and hematopoietic stem cell transplant: a new paradigm of treatment in Wolman disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jane E. Potter ◽  
Gemma Petts ◽  
Arunabha Ghosh ◽  
Fiona J. White ◽  
Jane L. Kinsella ◽  
...  
Keyword(s):  
Stem Cell ◽  
Multimodal Therapy ◽  
Hematopoietic Stem Cell Transplant ◽  
Stem Cell Transplant ◽  
Venous Access ◽  
Cell Transplant ◽  
New Paradigm ◽  
Hematopoietic Stem ◽  
Wolman Disease ◽  
Enzyme Replacement

Abstract Background Wolman disease is a rare, lysosomal storage disorder in which biallelic variants in the LIPA gene result in reduced or complete lack of lysosomal acid lipase. The accumulation of the substrates; cholesterol esters and triglycerides, significantly impacts cellular function. Untreated patients die within the first 12 months of life. Clinically, patients present severely malnourished, with diarrhoea and hepatosplenomegaly, many have an inflammatory phenotype, including with hemophagocytic lymphohistiocytosis (HLH). Hematopoietic stem cell transplant (HCT) had been historically the only treatment available but has a high procedure-related mortality because of disease progression and disease-associated morbidities. More recently, enzyme replacement therapy (ERT) with dietary substrate reduction (DSR) has significantly improved patient survival. However, ERT is life long, expensive and its utility is limited by anti-drug antibodies (ADA) and the need for central venous access. Results We describe five Wolman disease patients diagnosed in infancy that were treated at Royal Manchester Children's Hospital receiving ERT with DSR then HCT—multimodal therapy. In 3/5 an initial response to ERT was attenuated by ADA with associated clinical and laboratory features of deterioration. 1/5 developed anaphylaxis to ERT and the other patient died post HCT with ongoing HLH. All patients received allogeneic HCT. 4/5 patients are alive, and both disease phenotype and laboratory parameters are improved compared to when they were on ERT alone. The gastrointestinal symptoms are particularly improved after HCT, with reduced diarrhoea and vomiting. This allows gradual structured normalisation of diet with improved tolerance of dietary fat. Histologically there are reduced cholesterol clefts, fewer foamy macrophages and an improved villous structure. Disease biomarkers also show improvement with ERT, immunotherapy and HCT. Three patients have mixed chimerism after HCT, indicating a likely engraftment-defect in this condition. Conclusion We describe combined ERT, DSR and HCT, multimodal treatment for Wolman disease. ERT and DSR stabilises the sick infant and reduces the formerly described prohibitively high, transplant-associated mortality in this condition. HCT abrogates the problems of ERT, namely attenuating ADA, the need for continuing venous access, and continuing high cost drug treatment. HCT also brings improved efficacy, particularly evident in improved gastrointestinal function and histology. Multimodal therapy should be considered a new paradigm of treatment for Wolman disease patients where there is an attenuated response to ERT, and for all patients where there is a well-matched transplant donor, in order to improve long term gut function, tolerance of a normal diet and quality of life.

Metastases of Urothelium Carcinoma: Differential Diagnosis, Resection, and Survival

2021 ◽  
Author(s):  
Selma Oguzhan ◽  
Stefan Sponholz ◽  
Moritz Schirren ◽  
Mesut Mese ◽  
Joachim Schirren
Keyword(s):  
Differential Diagnosis ◽  
Multimodal Therapy ◽  
Pulmonary Metastases ◽  
Univariate Analysis ◽  
Pulmonary Carcinoma ◽  
Therapy Regimen ◽  
Urothelial Carcinomas ◽  
Common Diagnosis ◽  
Histological Confirmation ◽  
Systemic Therapies

Abstract Background Due to its very aggressive nature and low survival chances, the metastasized urothelium carcinoma poses a challenge in regard to therapy. The gold-standard chemotherapy is platinum based. The therapy options are considered controversial, including new systemic therapies. In this respect, surgical therapies, as already established for pulmonary metastases of other tumor entities play an increasingly important role. The consumption of nicotine is a risk factor not only for urothelium carcinoma but also for a pulmonary carcinoma. Thus, we examined the frequency of a second carcinoma in this cohort. Methods We retrospectively examined patients who had a differential diagnosis of pulmonary metastases, as well as those patients who underwent a surgery due to pulmonary metastases of a urothelium carcinoma between 1999 and 2015. Results A total of 139 patients came to our clinic with the differential diagnosis of pulmonary metastases of a urothelium carcinoma. The most common diagnosis was pulmonary carcinoma (53%). Thirty-one patients underwent surgeries due to pulmonary metastases of a urothelium carcinoma. The median survival was 53 months and the 5-year survival was 51%. With the univariate analysis, only the relapse-free interval of more than 10 months was statistically significant (p < 0.001). Conclusion There is a high coincidence of urothelial carcinoma and lung carcinoma. A histological confirmation should be endeavored. Selected patients undergoing a pulmonary metastasis resection have a survival advantage during the multimodal treatment of pulmonary metastasized urothelial carcinomas. For a definitive recommendation, randomized trials including a uniform multimodal therapy regimen and higher numbers of patients are necessary.

Melanin Nanoparticles Combined with CaO2 Nanoparticles for Image-Guided Tumor Microenvironment-Responsive Multimodal Therapy

2021 ◽  
Vol 4 (2) ◽  
pp. 1351-1363
Author(s):  
Jia-Hui Yan ◽  
Wei Meng ◽  
Hu Shan ◽  
Xiao-Ping Zhang ◽  
Li-Min Zou ◽  
...  
Keyword(s):  
Tumor Microenvironment ◽  
Multimodal Therapy ◽  
Image Guided ◽  
Melanin Nanoparticles

scholarly journals Primary Malignant Orbital Tumors

2021 ◽  
Vol 82 (01) ◽  
pp. 081-090
Author(s):  
Jacquelyn Laplant ◽  
Kimberly Cockerham
Keyword(s):  
Multimodal Therapy ◽  
Surgical Techniques ◽  
Surgical Approaches ◽  
Imaging Features ◽  
Orbital Surgery ◽  
Diagnosis And Management ◽  
Microsurgical Techniques ◽  
Invasive Techniques ◽  
Traditional Approaches ◽  
Orbital Imaging

Abstract Objective Primary orbital malignancy is rare. Awareness of the characteristic clinical and imaging features is imperative for timely identification and management. Surgery remains an important diagnostic and treatment modality for primary orbital malignancy, but determining the optimal surgical approach can be challenging. The purpose of this article is to explore recent advances in the diagnosis, management, and surgical approaches for primary orbital malignancies. Design In this review, the clinical presentation, imaging features, and medical and surgical management of primary orbital malignancies with representative cases will be discussed. Setting Outpatient and inpatient hospital settings. Participants Patients with diagnosed primary orbital malignancies. Main Outcome Measures Descriptive outcomes. Results Advancements in orbital imaging, microsurgical techniques, and multimodal therapy have improved the diagnosis and management of primary orbital malignancies. Special considerations for biopsy or resection are made based on the tumor's location, characteristics, nearby orbital structures, and goals of surgery. Minimally invasive techniques are supplanting traditional approaches to orbital surgery with less morbidity. Conclusions Advances in imaging technologies and surgical techniques have facilitated the diagnosis and management of primary orbital malignancies. Evolution toward less invasive orbital surgery with focus on preservation and restoration of function is underway.

Dedifferentiated chondrosarcoma of the lung: Report of a case

Surgery Today ◽  
2011 ◽  
Vol 41 (2) ◽  
pp. 251-254 ◽  
Author(s):  
Daigo Kawano ◽  
Ichiro Yoshino ◽  
Fumihiro Shoji ◽  
Kensaku Ito ◽  
Tokujiro Yano ◽  
...  
Keyword(s):  
Dedifferentiated Chondrosarcoma

Chromosomal changes in a dedifferentiated chondrosarcoma

2001 ◽  
Vol 124 (2) ◽  
pp. 105-111 ◽  
Author(s):  
Dennis P O'Malley ◽  
Kent E Opheim ◽  
Todd S Barry ◽  
Douglas B Chapman ◽  
Mary J Emond ◽  
...  
Keyword(s):  
Dedifferentiated Chondrosarcoma ◽  
Chromosomal Changes
Sign in / Sign up

Export Citation Format

Share Document