Paolo G Casali

..all this highlights the current need for new methods to do clinical studies on rare cancers, amid highly specific though anecdotal proofs and less specific though statistically more powerful evidence...

..There are several instances in which part of the "adjuvant" treatment may be administered preoperatively, even on the face of a surgically resectable GIST, to make surgery more limited and/or safer...

..This was also due to increased consideration of their variegated histology. Trabectedin is a new chemotherapeutic agent, which significantly strengthens the armamentarium along this direction...

..Its eradicating capabilities, as well as the implications of secondary resistance, are to be understood. 7) Integrated, multimodality approaches, including surgery, may still be of value in the molecular-targeted therapy era...

..To review developments in chordoma treatment...

..To review current state-of-the-art knowledge about adjuvant medical therapy in adult soft tissue sarcomas...

..The current retrospective analysis examined a large group of patients of all ages who were treated at a single institution over a 30-year period...

..To correlate morphologic changes with molecular, biochemical, and cytogenetic profiles in gastrointestinal stromal tumor (GIST) patients before and after imatinib treatment...

..The objective of this study was to investigate prognostic factors and clinical outcome of myxoid/round cell and pleomorphic liposarcoma...

..SM has antitumor activity in SFT, possibly through a PDGFRB-mediated mechanism, but treatments with IGFIR/insulin receptor and possibly epidermal growth factor receptor inhibitors are worth testing...

..To investigate c-Kit/PDGFRA genomic alterations, KIT-PDGFRA coexpression in gastrointestinal stromal tumors (GISTs) and the role of Bcl-2...

..Evidence of microscopic residual disease at re-excision was a marker of clinical aggressiveness...

..To explore the prognostic impact of isolated limb perfusion (ILP) in locally advanced extremity soft tissue sarcomas (ESTS)...

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..The presence of NF-1 syndrome per se did not affect survival, but patients with NF-1 were more likely to have larger tumors. Therefore, such patients should be followed carefully to detect disease as early as possible...

..In conclusion, DFSP-derived FS maintains the fusion-gene, being sensitive to imatinib. However, responses are short-lasting. Secondary resistance to imatinib is not related to PDGFRB...

..We explored the prognostic meaning of local relapse and surgical margins in adult soft tissue sarcoma of the extremities...

..Typically, it occurs superficially as single/multiple nodules (nodular ES), or in deeper tissues as a mass. The correlation between initial presentation and clinical outcome was investigated...

..A series of patients with well differentiated (WD)/dedifferentiated (DD) retroperitoneal liposarcoma (RLS) was studied to evaluate the prognostic value of the presence, extension, and grade of the DD component...

..A nomogram for predicting long term tumor-specific death in patients with soft tissue sarcoma (STS) was developed at the Memorial Sloan-Kettering Cancer Center (MSKCC)...

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..quot; The aim of this retrospective case series analysis was to investigate prognostic factors and the clinical outcome of a cohort of patients with myxofibrosarcoma treated at a single institution...

..A comprehensive retrospective review was performed of clinical presentation, treatment, outcome, and patterns of failure in a consecutive series of patients with localized or metastatic ASPS between 1975 and 2008...

..The objective of this study was to evaluate whether the distinction between leiomyosarcomas (LMS) and sarcomas with myogenic differentiation (SMD), based on the expression of muscular markers, has any clinical implications...

..Our descriptive results support the idea that GISTs activate autophagy rather than apoptosis in response to imatinib treatment and that their molecular makeup includes fingerprints of autophagy...

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..2011 Wiley Periodicals, Inc...

..The aim of the study was to review a monoinstitutional series of IVC resections for RSTS (mainly IVC leiomyosarcoma), focusing on the type of vascular replacement, morbidity, and oncologic outcome...

..The tumor vascular pattern prompted us to use sunitinib malate (SM), a tyrosine kinase inhibitor with antiangiogenic properties...

..The disease remained stable in more than half of patients. This study was designed to evaluate this approach on the natural history of the disease in a larger series of patients...

..This article describes the results of comprehensive molecular/biochemical analyses of the three receptors targeted by the drug (PDGFRB, PDGFRA, and KIT) in a series of 31 chordoma patients...

..To the authors' knowledge, no effective medical therapy currently is available for advanced chordoma. Imatinib mesylate is a tyrosine kinase inhibitor targeting platelet-derived growth factor receptor-beta (PDGFRB), BCR-ABL, and KIT...

..abstract:..

..Interestingly, this substitution is a homologue to the T315I mutation already reported in CML, where it is responsible for acquired resistance to imatinib...

..Primary end points were progression-free survival (PFS) and disease-specific survival (DSS)...

..Published series have reported definitively worse results for adults with RMS compared with children with RMS. This finding casts doubt on whether RMS is the same disease in adults as it is in children...

..Several series of patients with retroperitoneal sarcoma were reported, providing widely variable data regarding local and distant failure...

..Imatinib (IM) is active in advanced chordoma. The evidence of upstream and/or downstream mammalian target of rapamycin (mTOR) pathway activation prompted us to combine an mTOR inhibitor, sirolimus, to IM in IM-resistant advanced chordoma...

..A previous analysis showed that myxoid liposarcomas (MLS) are particularly sensitive to the drug. We report on the long-term efficacy of trabectedin in a subgroup of that series...

..To explore the effect of preoperative imatinib mesylate (IM) in patients with unresectable or locally advanced primary gastrointestinal stromal tumor (GIST)...

..The burden indicators were estimated and are provided in this chapter...

..Among STS patients, 9% had objective responses. In this unselected patient series, premedication with dexamethasone strongly reduced drug-induced hepatotoxicity and myelosuppression...

..To explore prognostic factors in surgically treated aggressive fibromatosis (extra-abdominal desmoid tumor)...

..To explore the prognostic effect of microscopic marginal status after surgery for extremity soft tissue sarcomas...

..Choi criteria were better predictors than RECIST in patients who received preoperative chemotherapy alone. Cancer 2012. © 2012 American Cancer Society...

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..CONCLUSIONS: Methotrexate plus vinblastine given every 7-10 days for several months is associated with prolonged stable disease in a substantial subset of patients with advanced (inoperable) aggressive fibromatosis...

..The project Surveillance of Rare Cancers in Europe (RARECARE) provides estimates of the incidence, prevalence and survival of rare cancers in Europe based on a new and comprehensive list of these diseases...

..The application of molecular simulations allowed us to quantify the interactions between the mutated receptors and imatinib, and to propose a molecular rationale for this type of drug resistance...

..In 2 patients (0.2%) a possible cardiotoxic effect of imatinib could not fully be excluded. The current analysis of a large randomised prospective study could not confirm previous suggestions of imatinib induced cardiac toxicity...

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..This study is based on the European Organisation for Research and Treatment of Cancer-Italian Sarcoma Group-Australasian Gastrointestinal Trials Group randomized trial comparing two doses of imatinib in advanced disease...

..A multivariate risk calculator that can be used in the clinic for individual patients is proposed...

..Our findings suggest the need for differential treatment of patients with GISTs, with KIT exon 9 mutant patients benefiting the most from the 800 mg daily dose of the drug...

..Imatinib is approved worldwide for use in gastrointestinal stromal tumours (GIST). We aimed to assess dose dependency of response and progression-free survival with imatinib for metastatic GIST...

..1% of patients were still alive and progression free one year after cross-over. We conclude that a cross-over to high-dose imatinib is feasible and safe in GIST patients who progress on low-dose therapy...

..Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients...

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..New York, NY) is a multitarget inhibitor of tyrosine kinases for the treatment of some human cancers. A myxedematous coma in a patient treated with sunitinib for a gastrointestinal stromal tumor was unexpectedly observed...