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  1. Case report

    Primary pulmonary artery sarcoma masquerading as pulmonary thromboembolism: a rare diagnosis unveiled

    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 thrombo...

    Abhishek Mahajan, Bharat Rekhi, Siddhartha Laskar, Jyoti Bajpai, Lekshmy Jayasree and Meenakshi H. Thakur

    Clinical Sarcoma Research 2017 7:13

    Published on: 1 July 2017

  2. Research

    Overexpressed PRAME is a potential immunotherapy target in sarcoma subtypes

    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 ...

    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 and Anthony P. Conley

    Clinical Sarcoma Research 2017 7:11

    Published on: 15 June 2017

  3. Case Report

    Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease

    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. Des...

    Enrico Pinotti, Marta Sandini, Simone Famularo, Marta Jaconi, Fabrizio Romano, Luca Nespoli and Luca Gianotti

    Clinical Sarcoma Research 2017 7:12

    Published on: 6 June 2017

  4. Research

    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)

    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 ne...

    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 and Christine Chevreau

    Clinical Sarcoma Research 2017 7:10

    Published on: 10 May 2017

  5. Research

    IDH1 or -2 mutations do not predict outcome and do not cause loss of 5-hydroxymethylcytosine or altered histone modifications in central chondrosarcomas

    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 activi...

    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 and Judith V. M. G. Bovée

    Clinical Sarcoma Research 2017 7:8

    Published on: 4 May 2017

  6. Research

    Histone 3.3 hotspot mutations in conventional osteosarcomas: a comprehensive clinical and molecular characterization of six H3F3A mutated cases

    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 presentat...

    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…

    Clinical Sarcoma Research 2017 7:9

    Published on: 4 May 2017

  7. Methodology

    How a Clinical Trial Unit can improve independent clinical research in rare tumors: the Italian Sarcoma Group experience

    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 require...

    Emanuela Marchesi, Celeste Cagnazzo, Irene Quattrini, Martina Piccinni Leopardi, Chiara Villa, Giovanni Grignani, Lorenzo D’Ambrosio, Silvia Stacchiotti, Paolo Giovanni Casali and Piero Picci

    Clinical Sarcoma Research 2017 7:4

    Published on: 28 February 2017

  8. Research

    Osteosarcoma follow-up: chest X-ray or computed tomography?

    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 valid...

    Anna Paioli, Michele Rocca, Luca Cevolani, Eugenio Rimondi, Daniel Vanel, Emanuela Palmerini, Marilena Cesari, Alessandra Longhi, Abate Massimo Eraldo, Emanuela Marchesi, Piero Picci and Stefano Ferrari

    Clinical Sarcoma Research 2017 7:3

    Published on: 14 February 2017

    The Erratum to this article has been published in Clinical Sarcoma Research 2017 7:7

  9. Research

    Central venous access related adverse events after trabectedin infusions in soft tissue sarcoma patients; experience and management in a nationwide multi-center study

    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 ...

    Michiel C. Verboom, Jan Ouwerkerk, Neeltje Steeghs, Jacob Lutjeboer, J. Martijn Kerst, Winette T. A. van der Graaf, Anna K. L. Reyners, Stefan Sleijfer and Hans Gelderblom

    Clinical Sarcoma Research 2017 7:2

    Published on: 31 January 2017

  10. Case report

    Radiotherapy in the management of gist: state of the art and new potential scenarios

    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...

    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 and M. A. Pantaleo

    Clinical Sarcoma Research 2017 7:1

    Published on: 10 January 2017

  11. Research

    Response to anti-PD1 therapy with nivolumab in metastatic sarcomas

    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 diseas...

    L. Paoluzzi, A. Cacavio, M. Ghesani, A. Karambelkar, A. Rapkiewicz, J. Weber and G. Rosen

    Clinical Sarcoma Research 2016 6:24

    Published on: 30 December 2016

  12. Case report

    Metastatic meningioma presenting as a malignant soft tissue tumour

    Extracranial metastasis of malignant meningioma to soft tissues is extremely rare and its clinical, radiological and pathological features are not well-characterised.

    Catherine McCarthy, Monika Hofer, Marianna Vlychou, Robar Khundkar, Paul Critchley, Simon Cudlip, Olaf Ansorge and Nick A. Athanasou

    Clinical Sarcoma Research 2016 6:23

    Published on: 30 December 2016

  13. Research

    Clinical implications of repeated drug monitoring of imatinib in patients with metastatic gastrointestinal stromal tumour

    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 clin...

    Ivar Hompland, Øyvind Sverre Bruland, Kumari Ubhayasekhera, Jonas Bergquist and Kjetil Boye

    Clinical Sarcoma Research 2016 6:21

    Published on: 15 December 2016

  14. Case report

    Pazopanib: a novel treatment option for aggressive fibromatosis

    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 resectio...

    Gulcan Bulut, Anil Ozluk, Atike Pınar Erdogan, Ruchan Uslu, Nevra Elmas and Burcak Karaca

    Clinical Sarcoma Research 2016 6:22

    Published on: 1 December 2016

  15. Review

    UK guidelines for the management of soft tissue sarcomas

    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...

    Adam Dangoor, Beatrice Seddon, Craig Gerrand, Robert Grimer, Jeremy Whelan and Ian Judson

    Clinical Sarcoma Research 2016 6:20

    Published on: 15 November 2016

  16. Research

    Outcome and the effect of age and socioeconomic status in 1318 patients with synovial sarcoma in the English National Cancer Registry: 1985–2009

    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 syno...

    Bernadette Brennan, Charles Stiller, Robert Grimer, Nicola Dennis, John Broggio and Matthew Francis

    Clinical Sarcoma Research 2016 6:18

    Published on: 14 October 2016

  17. Research

    High nuclear expression of proteasome activator complex subunit 1 predicts poor survival in soft tissue leiomyosarcomas

    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 invol...

    Sha Lou, Arjen H. G. Cleven, Benjamin Balluff, Marieke de Graaff, Marie Kostine, Inge Briaire-de Bruijn, Liam A. McDonnell and Judith V. M. G. Bovée

    Clinical Sarcoma Research 2016 6:17

    Published on: 1 October 2016

  18. Review

    Current status and unanswered questions on the use of Denosumab in giant cell tumor of bone

    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. Denosum...

    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 and Piotr Rutkowski

    Clinical Sarcoma Research 2016 6:15

    Published on: 14 September 2016

  19. Case Report

    Solitary fibrous tumour presenting with a single bone metastasis: report of six cases and literature review

    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 di...

    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 and Silvia Stacchiotti

    Clinical Sarcoma Research 2016 6:16

    Published on: 1 September 2016

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