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Clinical Sarcoma Research

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https://www.readbyqxmd.com/read/27891213/uk-guidelines-for-the-management-of-soft-tissue-sarcomas
#1
REVIEW
Adam Dangoor, Beatrice Seddon, Craig Gerrand, Robert Grimer, Jeremy Whelan, Ian Judson
Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues, and can occur almost anywhere in the body. Their rarity, and the heterogeneity of subtype and location means that developing evidence-based guidelines is complicated by the limitations of the data available. However, this makes it more important that STS are managed by teams, expert in such cases, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further data and knowledge...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27822356/efficacy-of-pazopanib-and-sunitinib-in-advanced-axial-chordoma-a-single-reference-centre-case-series
#2
Astrid Lipplaa, Sander Dijkstra, Hans Gelderblom
BACKGROUND: Chordomas are rare malignant tumours of the axial skeleton and skull base supposed to arise from cellular remnants of the notochord. These tumours have the potential to metastasize (30-40 %), usually in the later course of the disease. However, the greatest morbidity is usually a result of loco-regional recurrence with infiltration and destruction of surrounding bone and soft tissue. Patients with unresectable or metastatic chordoma are faced with a poor prognosis since cytotoxic chemotherapy or other systemic therapies have not proven their efficacy yet...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27777717/outcome-and-the-effect-of-age-and-socioeconomic-status-in-1318-patients-with-synovial-sarcoma-in-the-english-national-cancer-registry-1985-2009
#3
Bernadette Brennan, Charles Stiller, Robert Grimer, Nicola Dennis, John Broggio, Matthew Francis
BACKGROUND: The role of age as a prognostic factor has been examined in single institutional studies and in larger data sets from the SEER database, showing a survival advantage for younger versus adult patients with synovial sarcoma (SS). To further assess the role of age, socioeconomic status and other prognostic factors on outcome for SS, we analysed a contemporary all-age population-based cohort of patients with SS registered in England. METHODS: The data on 1318 synovial sarcomas diagnosed in England between 1985 and 2009 were retrospectively analysed for incidence, and the effect of age, patient characteristics and deprivation on outcome using both univariate and multivariate analysis...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27733900/high-nuclear-expression-of-proteasome-activator-complex-subunit-1-predicts-poor-survival-in-soft-tissue-leiomyosarcomas
#4
Sha Lou, Arjen H G Cleven, Benjamin Balluff, Marieke de Graaff, Marie Kostine, Inge Briaire-de Bruijn, Liam A McDonnell, Judith V M G Bovée
BACKGROUND: Previous studies on high grade sarcomas using mass spectrometry imaging showed proteasome activator complex subunit 1 (PSME1) to be associated with poor survival in soft tissue sarcoma patients. PSME1 is involved in immunoproteasome assembly for generating tumor antigens presented by MHC class I molecules. In this study, we aimed to validate PSME1 as a prognostic biomarker in an independent and larger series of soft tissue sarcomas by immunohistochemistry. METHODS: Tissue microarrays containing leiomyosarcomas (n = 34), myxofibrosarcomas (n = 14), undifferentiated pleomorphic sarcomas (n = 15), undifferentiated spindle cell sarcomas (n = 4), pleomorphic liposarcomas (n = 4), pleomorphic rhabdomyosarcomas (n = 2), and uterine leiomyomas (n = 7) were analyzed for protein expression of PSME1 using immunohistochemistry...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27651889/current-status-and-unanswered-questions-on-the-use-of-denosumab-in-giant-cell-tumor-of-bone
#5
REVIEW
Czar Louie Gaston, Robert J Grimer, Michael Parry, Silvia Stacchiotti, Angelo Paolo Dei Tos, Hans Gelderblom, Stefano Ferrari, Giacomo G Baldi, Robin L Jones, Sant Chawla, Paolo Casali, Axel LeCesne, Jean-Yves Blay, Sander P D Dijkstra, David M Thomas, Piotr Rutkowski
Denosumab is a monoclonal antibody to RANK ligand approved for use in giant cell tumour (GCT) of bone. Due to its efficacy, Denosumab is recommended as the first option in inoperable or metastatic GCT. Denosumab has also been used pre-operatively to downstage tumours with large soft tissue extension to allow for less morbid surgery. The role of Denosumab for conventional limb GCT of bone is yet to be defined. Further studies are required to determine whether local recurrence rates will be decreased with the adjuvant use of Denosumab along with surgery...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27588167/solitary-fibrous-tumour-presenting-with-a-single-bone-metastasis-report-of-six-cases-and-literature-review
#6
Vittoria Colia, Salvatore Provenzano, Carlo Morosi, Paola Collini, Salvatore Lorenzo Renne, Paolo G Dagrada, Claudia Sangalli, Angelo Paolo Dei Tos, Andrea Marrari, Paolo G Casali, Silvia Stacchiotti
BACKGROUND: Solitary fibrous tumour (SFT) is a rare soft tissue sarcoma with a low metastatic potential. A higher metastatic rate is observed in the high-grade/dedifferentiated variant. The most common expected site of distant spread are the lungs and the liver. Bone involvement is generally viewed as a late stage of disease spread. We report on a retrospective series of SFT patients relapsing with a single distant bone recurrence as first metastatic event, without evidence of other organ involvement...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27525058/primary-intraosseous-meningioma-an-osteosclerotic-bone-tumour-mimicking-malignancy
#7
M Vlychou, Y Inagaki, R Stacey, N A Athanasou
BACKGROUND: Sclerotic tumours of the calvarial bones are rare and may be due to primary and secondary bone tumours as well as extradural tumours of meningeal origin. CASE PRESENTATION: We report a case of primary intraosseous meningioma (PIM) which arose in the frontal bone of a 63 year old woman who complained of progressive pain and thickening of the right skull. Radiology showed a large osteosclerotic lesion in the right frontal bone. Histology showed an intraosseous lesion containing dense fibrous tissue in which there were scattered cells that expressed epithelial membrane antigen and progesterone receptor...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27482375/dendritic-and-mast-cell-involvement-in-the-inflammatory-response-to-primary-malignant-bone-tumours
#8
Y Inagaki, E Hookway, K A Williams, A B Hassan, U Oppermann, Y Tanaka, E Soilleux, N A Athanasou
BACKGROUND: A chronic inflammatory cell infiltrate is commonly seen in response to primary malignant tumours of bone. This is known to contain tumour-associated macrophages (TAMs) and lymphocytes; dendritic cells (DCs) and mast cells (MCs) have also been identified but whether these and other inflammatory cells are seen commonly in specific types of bone sarcoma is uncertain. METHODS: In this study we determined the nature of the inflammatory cell infiltrate in 56 primary bone sarcomas...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27437097/predictors-of-survival-in-patients-with-sarcoma-admitted-to-the-intensive-care-unit
#9
Rohan Gupta, Neda Heshami, Chouhan Jay, Naveen Ramesh, Juhee Song, Xiudong Lei, Erfe Jean Rose, Kristen Carter, Dejka M Araujo, Robert S Benjamin, Shreyaskumar Patel, Joseph L Nates, Vinod Ravi
BACKGROUND: Advances in treatment of sarcoma patients has prolonged survival but has led to increased disease- or treatment-related complications resulting in greater number of admissions to the intensive care unit (ICU). Survival and long-term outcome information about such critically ill patients with sarcoma is unknown. METHODS: The primary objective of the study was to determine the ICU and post-ICU survival rates of critically ill sarcoma patients. Secondary objectives included determining the modifiable and non-modifiable predictors of poor survival...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27403280/patterns-of-care-for-patients-with-advanced-soft-tissue-sarcoma-experience-from-australian-sarcoma-services
#10
Susie Bae, Philip Crowe, Raghu Gowda, Warren Joubert, Richard Carey-Smith, Paul Stalley, Jayesh Desai
BACKGROUND: There is a paucity of data on the current management of patients with advanced soft tissue sarcoma (STS) in the Australian health care setting. This study utilised the Australian sarcoma database to evaluate the patterns of care delivered to patients with advanced STS at Australian sarcoma services. METHODS: Prospectively collected data from six sarcoma centres in Australia were sourced to identify patients diagnosed with advanced STS between 1 January 2010 and 31 December 2012...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27358721/phosphorylated-insulin-growth-factor-i-receptor-p-igf1r-and-metalloproteinase-3-mmp3-expression-in-advanced-gastrointestinal-stromal-tumors-gist-a-geis-19-study
#11
Joan Maurel, Antonio López-Pousa, Silvia Calabuig, Silvia Bagué, Xavier Garcia Del Muro, Xavier Sanjuan, Jordi Rubió-Casadevall, Miriam Cuatrecasas, Javier Martinez-Trufero, Carlos Horndler, Joaquin Fra, Claudia Valverde, Andrés Redondo, Andrés Poveda, Isabel Sevilla, Nuria Lainez, Michele Rubini, Xabier García-Albéniz, Javier Martín-Broto, Enrique de Alava
BACKGROUND: Most GISTs have mutations in KIT or PDGFRA. Patients with advanced GIST with KIT exon 9, PDGFRA mutation or WT for KIT and PDGFRA have a worse progression-free survival (PFS) compared to patients with KIT exon 11 mutated tumors. We evaluated the immunohistochemical (IHC) expression of p-IGF1R (Y1316) and MMP3 as predictors of PFS or overall survival (OS). METHODS: Ninety-two advanced GIST patients included in GEIS-16 study with KIT and PDGFRA mutational information were examined for p-IGF1R (Y1316) and MMP3 expression in a tissue micro-array...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27315524/the-encca-wp7-eurosarc-eec-provabes-euramos-3rd-european-bone-sarcoma-networking-meeting-joint-workshop-of-eu-bone-sarcoma-translational-research-networks-vienna-austria-september-24-25-2015-workshop-report
#12
Leo Kager, Jeremy Whelan, Uta Dirksen, Bass Hassan, Jakob Anninga, Lindsey Bennister, Judith V M G Bovée, Bernadette Brennan, Javier M Broto, Laurence Brugières, Anne-Marie Cleton-Jansen, Christopher Copland, Aurélie Dutour, Franca Fagioli, Stefano Ferrari, Marta Fiocco, Emmy Fleuren, Nathalie Gaspar, Hans Gelderblom, Craig Gerrand, Joachim Gerß, Ornella Gonzato, Winette van der Graaf, Stefanie Hecker-Nolting, David Herrero-Martín, Stephanie Klco-Brosius, Heinrich Kovar, Ruth Ladenstein, Carlo Lancia, Marie-Cecile LeDeley, Martin G McCabe, Markus Metzler, Ola Myklebost, Michaela Nathrath, Piero Picci, Jenny Potratz, Françoise Redini, Günther H S Richter, Denise Reinke, Piotr Rutkowski, Katia Scotlandi, Sandra Strauss, David Thomas, Oscar M Tirado, Franck Tirode, Gilles Vassal, Stefan S Bielack
This report summarizes the results of the 3rd Joint ENCCA-WP7, EuroSarc, EEC, PROVABES, and EURAMOS European Bone Sarcoma Network Meeting, which was held at the Children's Cancer Research Institute in Vienna, Austria on September 24-25, 2015. The joint bone sarcoma network meetings bring together European bone sarcoma researchers to present and discuss current knowledge on bone sarcoma biology, genetics, immunology, as well as results from preclinical investigations and clinical trials, to generate novel hypotheses for collaborative biological and clinical investigations...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27274393/targeted-antiangiogenic-agents-in-combination-with-cytotoxic-chemotherapy-in-preclinical-and-clinical-studies-in-sarcoma
#13
REVIEW
Kieuhoa T Vo, Katherine K Matthay, Steven G DuBois
Sarcomas are a heterogeneous group of mesenchymal malignancies. In recent years, studies have demonstrated that inhibition of angiogenic pathways or disruption of established vasculature can attenuate the growth of sarcomas. However, when used as monotherapy in the clinical setting, these targeted antiangiogenic agents have only provided modest survival benefits in some sarcoma subtypes, and have not been efficacious in others. Preclinical and early clinical data suggest that the addition of conventional chemotherapy to antiangiogenic agents may lead to more effective therapies for patients with these tumors...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27175275/a-korean-single-center-real-world-retrospective-study-of-first-line-weekly-paclitaxel-in-patients-with-metastatic-angiosarcoma
#14
Seonggyu Byeon, Haa-Na Song, Hee Kyung Kim, Jun Soo Ham, Su Jin Lee, Jeeyun Lee, Se Hoon Park
BACKGROUND: Angiosarcoma is a rare subgroup of soft tissue sarcomas associated with poor prognosis, but paclitaxel has been shown to be active in pretreated metastatic disease. We investigated the efficacy and safety of weekly paclitaxel as first-line chemotherapy in adult patients with metastatic angiosarcoma. METHODS: A retrospective study using the Samsung Medical Center (Seoul, Korea) cancer chemotherapy registry was performed on 21 consecutive patients with angiosarcoma who were treated with weekly paclitaxel as first-line therapy for metastatic disease between Oct...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27148438/uk-guidelines-for-the-management-of-bone-sarcomas
#15
REVIEW
Craig Gerrand, Nick Athanasou, Bernadette Brennan, Robert Grimer, Ian Judson, Bruce Morland, David Peake, Beatrice Seddon, Jeremy Whelan
This document is an update of the British Sarcoma Group guidelines published in 2010. The aim is to provide a reference standard for the clinical care of patients in the UK with bone sarcomas. Recent recommendations by the European Society of Medical Oncology, The National Comprehensive Cancer Network and The National Institute for Health and Care Excellence have been incorporated, and the literature since 2010 reviewed. The standards represent a consensus amongst British Sarcoma Group members in 2015. It is acknowledged that these guidelines will need further updates as care evolves...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27127605/zoledronic-acid-in-metastatic-osteosarcoma-encouraging-progression-free-survival-in-four-consecutive-patients
#16
Robert M Conry, Michael G Rodriguez, Joseph G Pressey
BACKGROUND: Zoledronic acid (ZA) is a third-generation bisphosphonate in widespread clinical use to reduce pain and skeletal events in patients from a variety of malignancies with bone metastases. Pre-clinical studies indicate that ZA inhibits osteosarcoma through direct anti-proliferative effects, immune activation and anti-angiogenic activity. METHODS: The purpose of this study was to evaluate the antitumor efficacy of ZA at standard dose until progression in patients with stage IV osteosarcoma lacking a standard of care treatment option proven to influence survival...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27047656/proof-of-principle-for-bevacizumab-activity-in-desmoid-type-fibromatosis
#17
Viktor Grünwald, Florian Länger, H J Raatschen, Andreas Beilken
BACKGROUND: Desmoid-type fibromatosis (DF) is a rare disease, which often occurs in young adults. Medical treatment is an important option in the treatment algorithm of DF. Different chemotherapeutic regimens showed clinical activity in DF, but overall treatment tolerability remains poor for this patient cohort. Novel approaches investigated tyrosine kinase inhibitors in DF, but tolerability remained an issue. CASE PRESENTATION: We treated a patient with progressive DF after failure of chemotherapy for 1 year with singe agent bevacizumab...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/26998222/primary-kaposi-s-sarcoma-of-the-nasal-cavity-a-case-report-and-review-of-the-literature
#18
Karima Mouden, Mouna Khmou, Saida Loughmari, Afaf Semmar, Hanan El Kacemi, Basma El Khannoussi, Tayeb Kebdani, Sanaa Elmajjaoui, Noureddine Benjaafar
BACKGROUND: Kaposi sarcoma is a neoplastic vascular disorder. It usually present on the skin of the upper and lower extremities, rarely in the mucosa of the head and neck. The most common sites reported are within the oral cavity, particularly on the palate. Other mucosal sites are rare. We present an unusual case where the primary manifestation of the Kaposi's sarcoma was in the nasal mucosa. CASE PRESENTATION: A 56-year-old female of Mediterranean descent presented with a 1 year history of swelling on the left side of her nose, nasal obstruction and occasional minor epistaxes...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/26913180/metastatic-mesenteric-dedifferentiated-leiomyosarcoma-a-case-report-and-a-review-of-literature
#19
Mercy Varghese, Oyvind Bruland, Anne Marit Wiedswang, Ingvild Lobmaier, Bård Røsok, Robert S Benjamin, Kirsten Sundby Hall
BACKGROUND: Abdominal leiomyosarcoma arising from the mesentery is a rare malignancy. It is an aggressive entity with an overall 5 year survival rate between 20 and 30 %. Surgical resection is the cornerstone of primary treatment and may be curative for localized disease. However, patients often develop intra-abdominal relapse and/or metastatic disease. If surgical resection is not feasible, palliative chemotherapy is the treatment of choice. However, there are no clear guidelines regarding chemotherapy; neither in the adjuvant nor advanced setting...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/26909140/tumor-volume-is-a-better-predictor-of-post-operative-wound-complications-compared-to-tumor-size-in-soft-tissue-sarcomas-of-the-proximal-lower-extremity
#20
Michael Ziegele, David M King, Manpreet Bedi
BACKGROUND: Wide local excision with or without radiation therapy (RT) and chemotherapy is widely accepted as appropriate management for soft tissue sarcomas (STS) of the extremity. Although survival and local control rates are comparable to amputation, post-operative wound complications (WC) following limb salvage can result in significant morbidity for the patient. Certain risk factors such as location, pre-operative RT, and age have been shown to increase the risk of WCs. Somewhat surprisingly, size has not consistently been shown to impact WC rates...
2016: Clinical Sarcoma Research
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