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Clinical Sarcoma Research

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https://www.readbyqxmd.com/read/30410720/a-phase-ii-study-of-temsirolimus-and-liposomal-doxorubicin-for-patients-with-recurrent-and-refractory-bone-and-soft-tissue-sarcomas
#1
Matteo M Trucco, Christian F Meyer, Katherine A Thornton, Preeti Shah, Allen R Chen, Breelyn A Wilky, Maria A Carrera-Haro, Lillian C Boyer, Margaret F Ferreira, Umber Shafique, Jonathan D Powell, David M Loeb
Background: Relapsed and refractory sarcomas continue to have poor survival rates. The cancer stem cell (CSC) theory provides a tractable explanation for the observation that recurrences occur despite dramatic responses to upfront chemotherapy. Preclinical studies demonstrated that inhibition of the mechanistic target of rapamycin (mTOR) sensitizes the CSC population to chemotherapy. Methods: Here we present the results of the Phase II portion of a Phase I/II clinical trial that aimed to overcome the chemoresistance of sarcoma CSC by combining the mTOR inhibitor temsirolimus (20 mg/m2 weekly) with the chemotherapeutic agent liposomal doxorubicin (30 mg/m2 monthly)...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/30323920/preoperative-radiotherapy-of-soft-tissue-sarcomas-surgical-and-radiologic-parameters-associated-with-local-control-and-survival
#2
Panagiotis Tsagozis, Otte Brosjö, Mikael Skorpil
Background: Preoperative radiotherapy is often used to facilitate excision of soft-tissue sarcomas. We aimed define factors that affect local tumour control and patient survival. Methods: A single institution registry study of 89 patients with non-metastatic soft-tissue sarcomas having preoperative radiotherapy between 1994 and 2014. Radiologic (presence of peritumoural oedema and volume change following radiotherapy) and histopathologic (tumour volume, grade and surgical margin) parameters were recorded...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/30202513/periostin-expression-in-neoplastic-and-non-neoplastic-diseases-of-bone-and-joint
#3
Jennifer M Brown, Akiro Mantoku, Afsie Sabokbar, Udo Oppermann, A Bass Hassan, Akiro Kudo, Nick Athanasou
Background: Periostin is a matricellular protein that is expressed in bone and joint tissues. To determine the expression of periostin in primary bone tumours and to assess whether it plays a role in tumour progression, we carried out immunohistochemistry and ELISA for periostin in a range of neoplastic and non-neoplastic bone and joint lesions. Methods: 140 formalin-fixed paraffin-embedded sections of bone tumours and tumour-like lesions were stained by an indirect immunoperoxidase technique with a polyclonal anti-periostin antibody...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/30159138/low-grade-central-fibroblastic-osteosarcoma-may-be-differentiated-from-its-mimicker-desmoplastic-fibroma-by-genetic-analysis
#4
Wangzhao Song, Eva van den Berg, Thomas C Kwee, Paul C Jutte, Anne-Marie Cleton-Jansen, Judith V M G Bovée, Albert J Suurmeijer
Background: We studied two cases of rare fibrous bone tumors, namely desmoplastic fibroma (DF) and low-grade central osteosarcoma (LGCOS) resembling desmoplastic fibroma (DF-like LGCOS). As the clinical presentation, imaging features and histopathology of DF and DF-like LGOS show much overlap, the objective of this study was to investigate the value of cytogenetic analysis, molecular pathology and immunohistochemistry in discrimination of these two mimickers. Case presentation: A mutation in CTNNB (S45F) and nuclear beta-catenin immunostaining were observed in DF...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/30116519/retrospective-audit-of-957-consecutive-18-f-fdg-pet-ct-scans-compared-to-ct-and-mri-in-493-patients-with-different-histological-subtypes-of-bone-and-soft-tissue-sarcoma
#5
Ruth E Macpherson, Sarah Pratap, Helen Tyrrell, Mehrdad Khonsari, Shaun Wilson, Max Gibbons, Duncan Whitwell, Henk Giele, Paul Critchley, Lucy Cogswell, Sally Trent, Nick Athanasou, Kevin M Bradley, A Bassim Hassan
Background: The use of 18 F-FDG PET-CT (PET-CT) is widespread in many cancer types compared to sarcoma. We report a large retrospective audit of PET-CT in bone and soft tissue sarcoma with varied grade in a single multi-disciplinary centre. We also sought to answer three questions. Firstly, the correlation between sarcoma sub-type and grade with 18 FDG SUVmax, secondly, the practical uses of PET-CT in the clinical setting of staging (during initial diagnosis), restaging (new baseline prior to definitive intervention) and treatment response...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/30083310/pdgf-pdgfr-effects-in-osteosarcoma-and-the-add-on-strategy
#6
REVIEW
Jie Xu, Lu Xie, Wei Guo
New treatment options for advanced osteosarcoma have remained limited. The platelet-derived growth factor (PDGF)/platelet-derived growth factor receptor (PDGFR) pathway plays an important role in the development and metastasis of osteosarcoma, via either direct autocrine stimulation of tumor cells, or paracrine stimulation on tumor stromal cells. It promotes angiogenesis to overcome hypoxia in the tumor microenvironment, and modulates tumor interstitial fluid pressure to control the influx and efflux of other agents...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/30002809/tenosynovial-giant-cell-tumor-case-report-of-a-patient-effectively-treated-with-pexidartinib-plx3397-and-review-of-the-literature
#7
Nicholas Giustini, Nicholas M Bernthal, Susan V Bukata, Arun S Singh
Background: Tenosynovial giant cell tumors (TGCTs) or giant cell tumors of tendon sheath are neoplasms that arise in the synovium. They can be categorized as nodular (localized) or diffuse type (D-TGCT). Historically, surgery has been the mainstay of therapy, but diffuse type disease recurs at a high rate and treatment often requires increasingly morbid procedures. Elucidation of the importance of the colony-stimulating factor (CSF1)/CSF1 receptor (CSF1R) pathway in the pathogenesis of this disease has created significant interest in targeting this pathway as a novel TGCT treatment approach...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29992013/reversible-rituximab-induced-rectal-kaposi-s-sarcoma-misdiagnosed-as-ulcerative-colitis-in-a-patient-with-hiv-negative-follicular-lymphoma
#8
Emilien Billon, Anne-Marie Stoppa, Lena Mescam, Massimo Bocci, Audrey Monneur, Delphine Perrot, François Bertucci
Background: Kaposi's sarcoma is a low-grade mesenchymal angioproliferative tumor, most commonly observed in immunocompromised individuals, such as HIV-infected patients. Iatrogenic Kaposi's sarcoma occurs in patients undergoing immunosuppressive therapies. Rituximab is a chimeric monoclonal antibody targeted against the pan B cell marker CD20. Because of its immunosuppressive effects through reduction of mature B-cells, it may exacerbate Kaposi's sarcoma in HIV-positive patients. Rituximab-related Kaposi's sarcomas have been previously reported in only two HIV-negative patients and were treated surgically...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29988594/regional-chemotherapy-by-isolated-limb-perfusion-prior-to-surgery-compared-with-surgery-and-post-operative-radiotherapy-for-primary-locally-advanced-extremity-sarcoma-a-comparison-of-matched-cohorts
#9
Jens Jakob, Henry G Smith, Michelle J Wilkinson, Tim Pencavel, Aisha B Miah, Joseph M Thomas, Per-Ulf Tunn, Lothar R Pilz, Dirk C Strauss, Peter Hohenberger, Andrew J Hayes
Background: Induction chemotherapy by isolated limb perfusion (ILP) with melphalan and tumour necrosis factor-α is an effective strategy to facilitate limb-conserving surgery in locally advanced extremity sarcoma. In a comparison of cohorts matched for grade, size and surgical resectability, we compared the outcome of patients undergoing induction ILP prior to limb-conserving surgery and selective post-operative radiotherapy with patients undergoing limb-conserving surgery and routine post-operative radiotherapy...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29881541/non-canonical-wnt6-wnt10a-signal-factor-expression-in-ebv-post-transplant-smooth-muscle-tumors
#10
Kristin Teiken, Mark Kuehnel, Jan Rehkaemper, Hans Kreipe, Florian Laenger, Kais Hussein, Danny Jonigk
Post-transplant smooth muscle tumors (PTSMTs) are rare mesenchymal neoplasms which occur after solid organ or haematopoietic stem cell transplantation. PTSMT typically consist of Epstein-Barr-virus (EBV)+ smooth muscle-like cells and show an intermediate malignancy. Their main occurrences are visceral organs, especially the liver, but intracranial appearances are described and associated with a poor prognosis. EBV drives the growth of PTSMT; however, the underlying molecular mechanisms still remain unclear...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29785261/aggressive-fibromatosis-response-to-tamoxifen-lack-of-correlation-between-mri-and-symptomatic-response
#11
M Libertini, I Mitra, W T A van der Graaf, A B Miah, I Judson, R L Jones, K Thomas, E Moskovic, Z Szucs, C Benson, C Messiou
Background: One of the commonly used systemic agents for the treatment of aggressive fibromatosis is the anti-oestrogen drug tamoxifen. However, data on efficacy and optimum methods of response assessment are limited, consisting mainly of small case series and reports. Methods: A retrospective database was used to identify consecutive patients diagnosed with aggressive fibromatosis (AF) and treated with tamoxifen plus/minus non-steroidal anti-inflammatory drugs at our tertiary referral centre between 2007 and 2014...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29744029/treatment-patterns-and-survival-among-older-adults-in-the-united-states-with-advanced-soft-tissue-sarcomas
#12
Rohan C Parikh, Maria Lorenzo, Lisa M Hess, Sean D Candrilli, Steven Nicol, James A Kaye
Background: To describe patient and tumor characteristics, treatments, and survival among older adults in the United States with advanced soft-tissue sarcoma (STS), across and by categories of specifically defined histologic subtypes. Methods: We conducted a retrospective cohort analysis using the SEER. The study population comprised patients ≥ 65 years old with advanced STS (excluding osteosarcoma, Kaposi sarcoma, and gastrointestinal stromal tumors) diagnosed from January 1, 2001 to December 31, 2011...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29686838/smoking-is-predictive-of-poorer-distant-metastasis-free-and-progression-free-survival-in-soft-tissue-sarcoma-patients-treated-with-pre-operative-radiotherapy-or-chemoradiotherapy
#13
Nicholas P Gannon, David M King, Manpreet Bedi
Background: Soft tissue sarcomas (STS) are often treated with pre-operative radiation (RT), with or without chemotherapy, followed by wide local excision. Prognosis for these patients involves an interplay of tumor and patient characteristics. Known prognostic determinants include tumor size, grade, response to therapy, and patient characteristics such as age. While smoking is negatively correlated with outcomes in various malignancies, the impact on STS is unknown. We aimed to assess if smoking impacts overall (OS), distant metastasis-free (DMFS), and progression-free (PFS) survival in patients with STS treated with pre-operative RT...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29632658/unicentric-epithelioid-hemangioendothelioma-of-the-calcaneus-a-case-report-and-review-of-literature
#14
Mark C Plumby, Patrick Bacaj, Brock A Lindsey
Background: This review of the literature combined with a clinical case will allow the illustration of a favorable outcome of this variable low grade malignancy, display a role for limb salvage surgery with intralesional treatment, and offer a clinical example of epithelioid hemangioendothelioma, a rare malignancy. Case presentation: The case report presents a case of solitary epithelioid hemangioendothelioma (EHE) of the calcaneus in a 60-year-old male. Primary vascular tumors of the bone are rare; however, EHE is one of the most common primary malignant vascular tumors to occur in bone...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29610659/survival-is-influenced-by-approaches-to-local-treatment-of-ewing-sarcoma-within-an-international-randomised-controlled-trial-analysis-of-eicess-92
#15
Jeremy Whelan, Allan Hackshaw, Anne McTiernan, Robert Grimer, David Spooner, Jessica Bate, Andreas Ranft, Michael Paulussen, Herbert Juergens, Alan Craft, Ian Lewis
Background: Two national clinical trial groups, United Kingdom Children's Cancer and Leukaemia Group (CCLG) and the German Paediatric Oncology and Haematology Group (GPOH) together undertook a randomised trial, EICESS-92, which addressed chemotherapy options for Ewing's sarcoma. We sought the causes of unexpected survival differences between the study groups. Methods: 647 patients were randomised. Cox regression analyses were used to compare event-free survival (EFS) and overall survival (OS) between the two study groups...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29541442/the-use-of-whole-exome-sequencing-and-murine-patient-derived-xenografts-as-a-method-of-chemosensitivity-testing-in-sarcoma
#16
Nicholas Calvert, Jiansha Wu, Sophie Sneddon, Jennifer Woodhouse, Richard Carey-Smith, David Wood, Evan Ingley
Background: Soft tissue and bone sarcoma represent a broad spectrum of different pathology and genetic variance. Current chemotherapy regimens are derived from randomised trials and represent empirical treatment. Chemosensitivity testing and whole exome sequencing (WES) may offer personalized chemotherapy treatment based on genetic mutations. Methods: A pilot, prospective, non-randomised control experimental study was conducted. Twelve patients with metastatic bone or soft tissue sarcoma that had failed first line chemotherapy treatment were enrolled for this study...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29507712/different-quality-of-treatment-in-retroperitoneal-sarcomas-rps-according-to-hospital-case-volume-and-surgeon-case-volume-a-retrospective-regional-analysis-in-italy
#17
Sergio Sandrucci, Agostino Ponzetti, Claudio Gianotti, Baudolino Mussa, Patrizia Lista, Giovanni Grignani, Marinella Mistrangelo, Oscar Bertetto, Daniela Di Cuonzo, Giovannino Ciccone
Background: Retroperitoneal sarcomas (RPS) should be surgically managed in specialized sarcoma centers. However, it is not clearly demonstrated if clinical outcome is more influenced by Center Case Volume (CCV) or by Surgeon Case Volume (SCV). The aim of this study is to retrospectively explore the relationship between CCV and SCV and the quality of surgery in a wide region of Northern Italy. Methods: We retrospectively collected data about patients M0 surgically treated for RPSs in 22 different hospitals from 2006 to 2011, dividing them in two hospital groups according to sarcoma clinical activity volume (HCV, high case volume or LCV, low case volume hospitals)...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29449935/pleomorphic-liposarcoma-of-bone-a-rare-primary-malignant-bone-tumour
#18
G L Tiemeier, J M Brown, S E Pratap, C McCarthy, A Kastrenopoulou, K Bradley, S Wilson, Z Orosz, C L M H Gibbons, U Oppermann, N A Athanasou
Background: Liposarcoma is an extremely rare primary bone sarcoma. Case presentation: We report a case of primary pleomorphic liposarcoma that arose in an 18 year old male in the metaphysis of the left tibia. Plain radiographs showed a partly sclerotic lesion and MR imaging a heterogeneous tumour predominantly isointense on T1- and high-signal on T2-weighted sequences with focal areas of increased T1 signal that suppressed with fat saturation. PET/CT showed marked FDG uptake (SUV = 17...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29423171/intraosseous-papillary-intralymphatic-angioendothelioma-pila-one-new-case-and-review-of-the-literature
#19
Marco Gambarotti, Alberto Righi, Marta Sbaraglia, Giuseppe Bianchi, Piero Picci, Daniel Vanel, Angelo Paolo Dei Tos
Background: Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and only three cases reported in bone. Case presentation: We describe the case of a 51-year-old male with an intraosseous PILA of the proximal edge of his left clavicle and two other lesions evident on imaging. The patient was treated with marginal resection of the clavicle lesion but was lost to follow-up 1 month after surgery...
2018: Clinical Sarcoma Research
https://www.readbyqxmd.com/read/29299229/response-to-isolated-limb-perfusion-and-chemotherapy-with-epirubicin-plus-ifosfamide-in-a-metastatic-malignant-ossifying-fibromyxoid-tumor
#20
Salvatore Provenzano, Alessandra Raimondi, Rossella M Bertulli, Vittoria Colia, Salvatore L Renne, Paola Collini, Gianpaolo Dagrada, Dario Callegaro, Marco Fiore, Francesca G Greco, Paolo G Casali
Background: Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain lineage and intermediate biological potential. It is more common in middle-aged men, usually arising from the deep tissues of the extremities. It is now established that it is a translocation related tumor, most often marked by translocation of PHF1 gene. Surgery is the mainstay of treatment and proves usually curative, although, in rarer cases the disease shows malignant features and tendency to recur both locally and at distant sites...
2017: Clinical Sarcoma Research
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