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Clinical Sarcoma Research

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https://www.readbyqxmd.com/read/28725344/complete-response-of-mediastinal-clear-cell-sarcoma-to-pembrolizumab-with-radiotherapy
#1
Samuel Marcrom, Jennifer F De Los Santos, Robert M Conry
BACKGROUND: Clear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma thought to derive from neural crest and characterized by a 12;22 translocation. The resulting fusion protein directly activates expression of the melanocyte master transcription factor and drives the same down-stream pathways in CCS and melanoma leading to significant clinical parallels between these malignancies. Striking success of immune checkpoint blockade in melanoma has promoted interest in immunotherapy of CCS...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28680558/primary-pulmonary-artery-sarcoma-masquerading-as-pulmonary-thromboembolism-a-rare-diagnosis-unveiled
#2
Abhishek Mahajan, Bharat Rekhi, Siddhartha Laskar, Jyoti Bajpai, Lekshmy Jayasree, Meenakshi H Thakur
BACKGROUND: Primary pulmonary artery sarcomas are rare malignant vascular tumors and carry a very poor prognosis. Due to overlapping clinical and radiological features, the differentiation between pulmonary artery thromboembolism and pulmonary artery sarcoma can be challenging. CASE PRESENTATION: We herein present clinical, radiological and pathological features of primary pulmonary artery high grade sarcoma (angiosarcoma) in a 59-year-old male. The patient presented with a history of breathlessness on exertion of 2-months duration and was misdiagnosed as massive pulmonary thromboembolism on initial CT imaging...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28630682/overexpressed-prame-is-a-potential-immunotherapy-target-in-sarcoma-subtypes
#3
Jason Roszik, Wei-Lien Wang, John A Livingston, Christina L Roland, Vinod Ravi, Cassian Yee, Patrick Hwu, Andrew Futreal, Alexander J Lazar, Shreyaskumar R Patel, Anthony P Conley
BACKGROUND: PRAME (preferentially expressed antigen in melanoma), a member of the cancer-testis antigen family, has been shown to have increased expression in solid tumors, including sarcoma, and PRAME-specific therapies are currently in development for other cancers such as melanoma. METHODS: To map the landscape of PRAME expression in sarcoma, we used publicly available data from The Cancer Genome Atlas (TCGA) and the Cancer Cell Line Encyclopedia (CCLE) projects and determined which sarcoma subtypes and subsets are associated with increased PRAME expression...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28593036/disseminated-and-late-metastatic-disease-from-nasal-pit-leiomyosarcoma-after-radical-surgical-resection-case-report-of-a-singular-presentation-of-a-rare-disease
#4
Enrico Pinotti, Marta Sandini, Simone Famularo, Marta Jaconi, Fabrizio Romano, Luca Nespoli, Luca Gianotti
BACKGROUND: Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Despite international guidelines are available for soft tissue sarcomas, no recommendations are specifically endorsed for leiomyosarcoma of the head and neck, due to the rarity of its presentation and consequently scarcity of data on long-term outcome. CASE PRESENTATION: A 50-year old woman, operated 10 years before for leiomyosarcoma of the nasal pit and with negative 5-years follow-up, was admitted to our ward for impairment of the hepatic function...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28491276/antiangiogenic-effects-in-patients-with-progressive-desmoplastic-small-round-cell-tumor-data-from-the-french-national-registry-dedicated-to-the-use-of-off-labeled-targeted-therapy-in-sarcoma-outc-s
#5
Sarah Bétrian, Christophe Bergeron, Jean-Yves Blay, Emmanuelle Bompas, Philippe A Cassier, Laure Chevallier, Jérome Fayette, Magali Girodet, Cécile Guillemet, Axel Le Cesne, Perrine Marec-Berard, Isabelle Ray-Coquard, Christine Chevreau
BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a very rare mesenchymal tumor that mainly affects teenagers and young adults with a mean age at diagnosis around 20-25 years. Although initial management still needs standardization, many centers will use multimodal treatment including intensive chemotherapy, extensive surgical resection followed by radiotherapy. Despite this, prognosis remains very poor and the median overall survival is 25 months. Recurrent disease is mainly treated by chemotherapy...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28484590/histone-3-3-hotspot-mutations-in-conventional-osteosarcomas-a-comprehensive-clinical-and-molecular-characterization-of-six-h3f3a-mutated-cases
#6
Christian Koelsche, Daniel Schrimpf, Lars Tharun, Eva Roth, Dominik Sturm, David T W Jones, Eva-Kristin Renker, Martin Sill, Annika Baude, Felix Sahm, David Capper, Melanie Bewerunge-Hudler, Wolfgang Hartmann, Andreas E Kulozik, Iver Petersen, Uta Flucke, Hendrik W B Schreuder, Reinhard Büttner, Marc-André Weber, Peter Schirmacher, Christoph Plass, Stefan M Pfister, Andreas von Deimling, Gunhild Mechtersheimer
BACKGROUND: Histone 3.3 (H3.3) hotspot mutations in bone tumors occur in the vast majority of giant cell tumors of bone (GCTBs; 96%), chondroblastomas (95%) and in a few cases of osteosarcomas. However, clinical presentation, histopathological features, and additional molecular characteristics of H3.3 mutant osteosarcomas are largely unknown. METHODS: In this multicentre, retrospective study, a total of 106 conventional high-grade osteosarcomas, across all age groups were re-examined for hotspot mutations in the H3...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28484589/idh1-or-2-mutations-do-not-predict-outcome-and-do-not-cause-loss-of-5-hydroxymethylcytosine-or-altered-histone-modifications-in-central-chondrosarcomas
#7
Arjen H G Cleven, Johnny Suijker, Georgios Agrogiannis, Inge H Briaire-de Bruijn, Norma Frizzell, Attje S Hoekstra, Pauline M Wijers-Koster, Anne-Marie Cleton-Jansen, Judith V M G Bovée
BACKGROUND: Mutations in isocitrate dehydrogenase (IDH)1 or -2 are found in ~50% of conventional central chondrosarcomas and in up to 87% of their assumed benign precursors enchondromas. The mutant enzyme acquires the activity to convert α-ketoglutarate into the oncometabolite d-2-hydroxyglutarate (d-2-HG), which competitively inhibits α-ketoglutarate dependent enzymes such as histone- and DNA demethylases. METHODS: We therefore evaluated the effect of IDH1 or -2 mutations on histone modifications (H3K4me3, H3K9me3 and H3K27me3), chromatin remodeler ATRX expression, DNA modifications (5-hmC and 5-mC), and TET1 subcellular localization in a genotyped cohort (IDH, succinate dehydrogenase (SDH) and fumarate hydratase (FH)) of enchondromas and central chondrosarcomas (n = 101) using immunohistochemistry...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28473911/erratum-to-osteosarcoma-follow-up-chest-x-ray-or-computed-tomography
#8
Anna Paioli, Michele Rocca, Luca Cevolani, Eugenio Rimondi, Daniel Vanel, Emanuela Palmerini, Marilena Cesari, Alessandra Longhi, Massimo Eraldo Abate, Emanuela Marchesi, Piero Picci, Stefano Ferrari
[This corrects the article DOI: 10.1186/s13569-017-0067-5.].
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28465823/uk-clinical-practice-guidelines-for-the-management-of-gastrointestinal-stromal-tumours-gist
#9
Ian Judson, Ramesh Bulusu, Beatrice Seddon, Adam Dangoor, Newton Wong, Satvinder Mudan
BACKGROUND: Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues. Gastrointestinal stromal tumour (GIST) is the commonest STS and arises within the wall of the gastrointestinal (GI) tract. While most GISTs occur in the stomach they do occur in all parts of the GI tract. As with other STS, it is important that GISTs are managed by expert teams, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further knowledge of the disease...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28286642/two-years-survival-of-primary-cardiac-leiomyosarcoma-managed-by-surgical-and-adjuvant-therapy
#10
K Behi, M Ayadi, E Mezni, K Meddeb, A Mokrani, Y Yahyaoui, F Ksontini, H Rais, N Chrait, A Mezlini
BACKGROUND: Cardiac tumors are a very rare entity. Leiomyosarcoma represents less than 1% of cases. CASE PRESENTATION: a 51-year-old woman diagnosed with primary left atrium leiomyosarcoma. She was treated by optimal surgery and adjuvant chemotherapy. She is still alive after a follow-up of 24 months without evidence of local or distant recurrence. CONCLUSIONS: Cardiac leiomyosarcoma is a rare tumor with a dismal prognosis. Surgery is the mainstay of treatment...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28261443/how-a-clinical-trial-unit-can-improve-independent-clinical-research-in-rare-tumors-the-italian-sarcoma-group-experience
#11
Emanuela Marchesi, Celeste Cagnazzo, Irene Quattrini, Martina Piccinni Leopardi, Chiara Villa, Giovanni Grignani, Lorenzo D'Ambrosio, Silvia Stacchiotti, Paolo Giovanni Casali, Piero Picci
BACKGROUND: The Italian Sarcoma Group (ISG) is a nonprofit group of professionals established in 1997 aimed to improve the quality of care and promote the independent research in sarcomas. The increased regulatory requirements, the chance to increase the number of trials with other cooperative groups and an interest from pharmaceutical companies in supporting independent research, generated the need of an internal service for research management. METHODS AND RESULTS: In 2010, ISG implemented in its organization a Clinical Trial Unit (CTU)...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28228934/osteosarcoma-follow-up-chest-x-ray-or-computed-tomography
#12
Anna Paioli, Michele Rocca, Luca Cevolani, Eugenio Rimondi, Daniel Vanel, Emanuela Palmerini, Marilena Cesari, Alessandra Longhi, Abate Massimo Eraldo, Emanuela Marchesi, Piero Picci, Stefano Ferrari
BACKGROUND: In patients with relapsed osteosarcoma, the surgical excision of all metastases, defined as second complete remission (CR-2), is the factor that mainly influences post-relapse survival (PRS). Currently a validated follow-up policy for osteosarcoma is not available, both chest X-ray and computed tomography (CT) are suggested for lung surveillance. The purpose of this study is to evaluate whether the type of imaging technique used for chest surveillance, chest X-ray or CT, influenced the rate of CR-2 and prognosis in patients with recurrent osteosarcoma...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28163887/central-venous-access-related-adverse-events-after-trabectedin-infusions-in-soft-tissue-sarcoma-patients-experience-and-management-in-a-nationwide-multi-center-study
#13
Michiel C Verboom, Jan Ouwerkerk, Neeltje Steeghs, Jacob Lutjeboer, J Martijn Kerst, Winette T A van der Graaf, Anna K L Reyners, Stefan Sleijfer, Hans Gelderblom
BACKGROUND: Trabectedin has shown efficacy against soft tissue sarcomas (STS) and has manageable toxicity. Trabectedin is administered through central venous access devices (VAD), such as subcutaneous ports with tunneled catheters, Hickman catheters and PICC lines. Venous access related adverse events are common, but have not yet been reported in detail. METHODS: A retrospective analysis of patient files of STS patients receiving trabectedin monotherapy between 1999 and 2014 was performed in all five STS referral centers in the Netherlands...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28078078/radiotherapy-in-the-management-of-gist-state-of-the-art-and-new-potential-scenarios
#14
L Gatto, M Nannini, M Saponara, V Di Scioscio, G Beltramo, G P Frezza, G Ercolani, A D Pinna, A Astolfi, M Urbini, G Brandi, G Biasco, M A Pantaleo
BACKGROUND: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal neoplasm of the gastrointestinal tract. The main treatment for localized gastrointestinal stromal tumors is surgical resection. Unresectable or advanced GIST are poorly responsive to conventional cytotoxic chemotherapy but the introduction of tyrosine kinase inhibitors (TKIs) marked a revolutionary step in the treatment of these patients, radically improving prognosis and clinical benefit. Historically GIST has been considered radiation-resistant, and the role of radiotherapy in the management of patients with GIST is currently restricted to symptomatic palliation in current treatment guidelines...
2017: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28042471/response-to-anti-pd1-therapy-with-nivolumab-in-metastatic-sarcomas
#15
L Paoluzzi, A Cacavio, M Ghesani, A Karambelkar, A Rapkiewicz, J Weber, G Rosen
BACKGROUND: Manipulation of immune checkpoints such as CTLA4 or PD-1 with targeted antibodies has recently emerged as an effective anticancer strategy in multiple malignancies. Sarcomas are a heterogeneous group of diseases in need of more effective treatments. Different subtypes of soft tissue and bone sarcomas have been shown to express PD-1 ligand. METHODS: We retrospectively analyzed a cohort of patients (pts) with relapsed metastatic/unresectable sarcomas, who were treated with nivolumab provided under a patient assistance program from the manufacturer...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/28042470/metastatic-meningioma-presenting-as-a-malignant-soft-tissue-tumour
#16
Catherine McCarthy, Monika Hofer, Marianna Vlychou, Robar Khundkar, Paul Critchley, Simon Cudlip, Olaf Ansorge, Nick A Athanasou
BACKGROUND: Extracranial metastasis of malignant meningioma to soft tissues is extremely rare and its clinical, radiological and pathological features are not well-characterised. CASE PRESENTATION: We report a case of a 58 year old man who presented with a mobile mass within the left trapezius muscle. The patient had previously undergone surgery for a right frontal lobe high grade anaplastic meningioma. Histology of the soft tissue lesion showed metastatic anaplastic meningioma with clumps of pleomorphic tumour cells which expressed epithelial membrane antigen, cytokeratin and P63 but were negative for other epithelial and mesenchymal markers...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27999655/clinical-implications-of-repeated-drug-monitoring-of-imatinib-in-patients-with-metastatic-gastrointestinal-stromal-tumour
#17
Ivar Hompland, Øyvind Sverre Bruland, Kumari Ubhayasekhera, Jonas Bergquist, Kjetil Boye
BACKGROUND: Imatinib mesylate (IM) is the preferred treatment for the majority of patients with metastatic gastrointestinal stromal tumour (GIST). Low trough IM concentration (Cmin) values have been associated with poor clinical outcomes in GIST patients. However, there are few studies of repeated measurements of IM levels, and therapeutic drug monitoring is not yet a part of routine clinical practice. This study was conducted to reveal clinical scenarios where plasma concentration measurement of IM trough level (Cmin) is advantageous...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27957320/pazopanib-a-novel-treatment-option-for-aggressive-fibromatosis
#18
Gulcan Bulut, Anil Ozluk, Atike Pınar Erdogan, Ruchan Uslu, Nevra Elmas, Burcak Karaca
BACKGROUND: Aggressive fibromatosis (AF), also known as desmoid tumor, is an uncommon soft tissue neoplasm. AF does not metastasize, but it is locally invasive and its propensity for recurrence after conservative resection is well documented. No effective cytotoxic treatment has been reported, hence there is a need for novel treatment strategies. CASE PRESENTATION: We present the case of an AF successfully treated with an oral tyrosine kinase inhibitor, pazopanib, with mild side effects...
2016: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/27891213/uk-guidelines-for-the-management-of-soft-tissue-sarcomas
#19
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
#20
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
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