Presentation and management of pulmonary artery sarcoma
Conclusions PAS often presents with symptoms mimicking pulmonary hypertension, heart failure or thromboembolic disease. PEA provides good symptomatic relief and in some cases, offers a chance of long-term survival. Although outcome appears to be better when PEA is combined with post-operative chemo- and radio-therapy, further studies are warranted. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - January 21, 2015 Category: Cancer & Oncology Source Type: research

Regorafenib treatment for advanced, refractory gastrointestinal stromal tumor: a report of the UK managed access program
Conclusions These data demonstrate encouraging activity and tolerability of regorafenib in routine clinical practice. The documented adverse events are in line with previous trial data. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - December 4, 2014 Category: Cancer & Oncology Source Type: research

High-dose continuous-infusion ifosfamide in advanced well-differentiated/dedifferentiated liposarcoma
Conclusions These data suggest that ciHDIFX is active in WD/DDLPS, even in patients already treated with a combination of anthracyclines plus ifosfamide. In this series, ciHDIFX regimen was better tolerated than HDIFX in published studies. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - November 22, 2014 Category: Cancer & Oncology Source Type: research

Concomitant angiosarcoma and lymphoproliferative disorder in solid organ transplant recipients
This report describes two patients who developed high-grade angiosarcoma following a solid organ transplant. Notably, in both patients, the diagnosis of angiosarcoma was preceded by diagnosis of a lymphoproliferative disorder with monoclonal immunoglobulin heavy chain rearrangement. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - October 31, 2014 Category: Cancer & Oncology Source Type: research

Pneumothorax as adverse event in patients with lung metastases of soft tissue sarcoma treated with pazopanib: a single reference centre case series
Conclusion The risk of an, often difficult to treat, pneumothorax during pazopanib therapy should be discussed with the patient before initiation of treatment for a pulmonary metastasized sarcoma and physicians should be alert to the occurrence of such an event. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - October 1, 2014 Category: Cancer & Oncology Source Type: research

Successful use of pazopanib for treatment of refractory metastatic hemangiopericytoma
We describe three cases of metastatic hemangiopericytoma. In the first case, five lines of chemotherapeutic agents were used unsuccessfully in a patient with a 12-year history of metastatic hemangiopericytoma. After one cycle of pazopanib therapy, however, chest radiography showed a decrease in tumor volume of more than 30%. A marked decrease in FDG uptake on PET CT was also noted, and the patient is now on her 5th month of pazopanib therapy. The second case is a patient with a brain hemangiopericytoma with multiple liver, lung, and bone metastases. Pazopanib induced radiologic stabilization of metastatic disease over the ...
Source: Clinical Sarcoma Research - September 24, 2014 Category: Cancer & Oncology Source Type: research

Multiple or metastatic clear cell chondrosarcoma: a case report
We report multiple synchronous clear-cell chondrosarcomas in a 43-year-old patient. The patient had a lesion in the right proximal humerus and in the left femoral condyle. Bone scintigraphy revealed increased uptake in both foci. Pathological analysis confirmed the diagnosis in both locations. In the proximal humerus, wide resection of the tumour was performed with allograft reconstruction of the joint with osteosynthesis. The femoral condyle was treated with curettage, phenolization, and cementation. Over a follow-up of 10 years no recurrence or metastasis was observed. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - September 16, 2014 Category: Cancer & Oncology Source Type: research

Comprehensive analysis of published studies involving systemic treatment for chondrosarcoma of bone between 2000 and 2013
Conclusion Because chondrosarcoma of bone is an orphan disease it is difficult to conduct clinical trials. The meagre outcome data for locally advanced or metastatic patients indicate that new treatment options are needed. For the phase I trials it is difficult to draw conclusions because of the low numbers of chondrosarcoma patients enrolled, and at different dose levels. Some phase II trials show promising results which support further research. Retrospective studies are encouraged as they could add to the limited data available. Efforts to increase the number of studies for this orphan disease are ...
Source: Clinical Sarcoma Research - August 12, 2014 Category: Cancer & Oncology Source Type: research

Characterization and assessment of the sensitivity and resistance of a newly established human gastrointestinal stromal tumour xenograft model to treatment with tyrosine kinase inhibitors
Conclusions We established a novel human GIST xenograft, UZLX-GIST9, harbouring KIT exon 11 and 17 mutations and maintaining the pheno-and genotype of the original tumour. UZLX-GIST9 shows different levels of response to standard TKIs. This model will help to study TKI resistance and to explore novel treatment approaches for patients with TKI-resistant GIST. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 10, 2014 Category: Cancer & Oncology Source Type: research

Dystrophin deregulation is associated with tumor progression in KIT/PDGFRA mutant gastrointestinal stromal tumors
Conclusions Our study confirms the presence of DMD deletions only in KIT/PDGFRA mutant GIST and this event is almost associated with metastatic disease. These findings are, of course, quite preliminary but support development of potential therapeutic strategies that target and restore DMD function in the treatment of metastatic GIST. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 9, 2014 Category: Cancer & Oncology Source Type: research

Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature
We describe the first case of unresectable pancreatic metastases from DFSP treated with neoadjuvant Imatinib and subsequently R0 metastectomy. Additionally, a comprehensive systematic review of DFSP pancreatic metastases and the current published data on the use of Imatinib in DFSP is summarized. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 6, 2014 Category: Cancer & Oncology Source Type: research

Pazopanib in advanced desmoplastic small round cell tumours: a multi-institutional experience
Conclusion In this series, pazopanib showed interesting activity in DSRCT patients who progressed after prior chemotherapy without major toxicity. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 29, 2014 Category: Cancer & Oncology Source Type: research

Response of follicular dendritic cell sarcoma to gemcitabine and docetaxel: report of two cases and literature review
Abstract Follicular dendritic cell sarcoma is a rare malignancy arising from follicular dendritic cells, which form a meshwork within lymphoid follicles. Traditional treatment of metastatic disease with CHOP chemotherapy, the most commonly used regimen for lymphoid malignancies, has met with limited success. I report herein two cases of follicular dendritic cell sarcoma metastatic to the liver treated with the combination of gemcitabine and docetaxel showing unprecedented response in this disease. These observations coupled with recent evidence of a mesenchymal origin of follicular dendritic cells suppo...
Source: Clinical Sarcoma Research - June 28, 2014 Category: Cancer & Oncology Source Type: research

Treatment of hormone positive uterine leiomyosarcoma with aromatase inhibitors
Conclusions In this population of patients with hormone positive ULMS, AIs achieved a significant CBR (62.5%) in 1st line, which was retained in 2nd line (CBR: 50%). The relatively prolonged median PFS (14 months), along with the favourable toxicity profile could place AIs among the first choices of systemic treatment in hormone positive ULMS, preferably in strongly positive (>90%), and/ or low grade and low volume disease. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - June 26, 2014 Category: Cancer & Oncology Source Type: research

Workshop report on the 2 nd Joint ENCCA/EuroSARC European bone sarcoma network meeting: integration of clinical trials with tumour biology
Abstract This is the report of the 2nd Joint ENCCA/EuroSARC European Bone Sarcoma Network Meeting held in Leiden, The Netherlands, on 26-27 September 2013, bringing together preclinical and clinical investigators on bone sarcoma. The purpose of this workshop was to present the achievements of biological research and clinical trials in bone sarcomas and to stimulate crosstalk. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 1, 2014 Category: Cancer & Oncology Source Type: research