Articles

Pazopanib is an active treatment in desmoid tumour/aggressive fibromatosis

Desmoid tumours/aggressive fibromatosis (DT/AF) are infrequent soft-tissue neoplasms. They usually behave as indolent diseases. However, they may grow locally infiltrating or compressing adjacent structures. T...

Authors: Juan Martin-Liberal, Charlotte Benson, Heather McCarty, Khin Thway, Christina Messiou and Ian Judson

Gastrointestinal stromal tumors: a case-only analysis of single nucleotide polymorphisms and somatic mutations

Gastrointestinal stromal tumors are rare soft tissue sarcomas that typically develop from mesenchymal cells with acquired gain-in-function mutations in KIT or PDGFRA oncogenes. These somatic mutations have been w...

Authors: Katie M O’Brien, Irene Orlow, Cristina R Antonescu, Karla Ballman, Linda McCall, Ronald DeMatteo and Lawrence S Engel

The attitudes of people with sarcoma and their family towards genomics and incidental information arising from genetic research

The study aimed to examine attitudes of individuals diagnosed with sarcoma and their family members towards genetics, genomic research and incidental information arising as a result of participating in genetic...

Authors: Mary-Anne Young, Amy Herlihy, Gillian Mitchell, David M Thomas, Mandy Ballinger, Kathy Tucker, Craig R Lewis, Susan Neuhaus and Jane Halliday

Mesenchymal stem cell transformation and sarcoma genesis

MSCs are hypothesized to potentially give rise to sarcomas after transformation and therefore serve as a good model to study sarcomagenesis. Both spontaneous and induced transformation of MSCs have been report...

Authors: Wei Xiao, Alexander B Mohseny, Pancras C W Hogendoorn and Anne-Marie Cleton-Jansen

MicroRNA expression in Epstein-Barr virus-associated post-transplant smooth muscle tumours is related to leiomyomatous phenotype

Epstein-Barr virus (EBV)-associated post-transplant smooth muscle tumours (PTSMT) are rare complications. In our previous molecular analysis, we have evaluated the expression of regulatory microRNA which are k...

Authors: Danny Jonigk, Nicole Izykowski, Lavinia Maegel, Eileen Schormann, Britta Maecker-Kolhoff, Florian Laenger, Hans Kreipe and Kais Hussein

Response to imatinib in villonodular pigmented synovitis (PVNS) resistant to nilotinib

Pigmented villonodular synovitis (PVNS) is a rare locally aggressive tumor. PVNS is characterized in most cases by a specific t(1;2) translocation, which fuses the colony stimulating factor-1 (CSF1) gene to the c...

Authors: Silvia Stacchiotti, Flavio Crippa, Antonella Messina, Silvana Pilotti, Alessandro Gronchi, Jean Y Blay and Paolo G Casali

The role of chemotherapy in advanced solitary fibrous tumors: a retrospective analysis

Patients with advanced solitary fibrous tumors (SFTs) have a poor prognosis; treatment options for recurrent disease are particularly limited. Several novel targeted agents have recently shown promise against ...

Authors: Min S Park, Vinod Ravi, Anthony Conley, Shreyaskumar R Patel, Jonathan C Trent, Dina C Lev, Alexander J Lazar, Wei-Lien Wang, Robert S Benjamin and Dejka M Araujo

Bone metastases in soft tissue sarcoma: a survey of natural history, prognostic value and treatment options

We surveyed the natural history of bone metastases in patients affected by soft tissue sarcoma (STS).

Authors: Bruno Vincenzi, Anna Maria Frezza, Gaia Schiavon, Daniele Santini, Palma Dileo, Marianna Silletta, Delia Delisi, Francesco Bertoldo, Giuseppe Badalamenti, Giacomo Giulio Baldi, Stefania Zovato, Rossana Berardi, Marco Tucci, Franco Silvestris, Angelo Paolo Dei Tos, Roberto Tirabosco…

Dermatofibrosarcoma protuberans (DFSP) successfully treated with sorafenib: case report

DFSP is a locally invasive, slow-growing tumor of the subcutaneous tissue that rarely metastasizes but recurs frequently after surgical excision. We report herein a case of highly recurrent, locally invasive D...

Authors: Francois G Kamar, Victor F Kairouz and Alain N Sabri

Solitary fibrous tumor of all sites: outcome of late recurrences in 14 patients

We explore the pattern of late recurrence (LR) in solitary fibrous tumor (SFT), focusing on histopathologic characteristics, clinical presentation and patients (pts) outcome.

Authors: Giacomo G Baldi, Silvia Stacchiotti, Valentina Mauro, Angelo P Dei Tos, Alessandro Gronchi, Ugo Pastorino, Leonardo Duranti, Salvatore Provenzano, Andrea Marrari, Michela Libertini, Silvana Pilotti and Paolo G Casali

Endometrial stromal tumors: immunohistochemical and molecular analysis of potential targets of tyrosine kinase inhibitors

The systemic treatment of malignant endometrial stromal tumors (EST) is not well established. A few reports describe objective responses to imatinib, which suggest a novel therapeutic strategy for these tumors...

Authors: Ruth Sardinha, Teresa Hernández, Susana Fraile, Francesc Tresserra, August Vidal, Maria Carmén Gómez, Aurora Astudillo, Nieves Hernández, Javier Saenz de Santamaría, Jaume Ordi, Luis Gonçalves, Rafael Ramos, Carmen Balañá and Enrique de Álava

Localized Ewing sarcoma of the tibia

Ewing sarcoma (ES) is a high-grade malignant primary round cell tumour of bone in which there is commonly extension into extraosseous soft tissues at the time of diagnosis. This report details the clinical, ra...

Authors: Takeshi G Kashima, Nimali G Gamage, Uta Dirksen, Christopher L M H Gibbons, Simon J Ostlere and Nicholas A Athanasou

Solitary fibrous tumors of the soft tissues: imaging features with histopathologic correlations

To describe the imaging features of soft tissue solitary fibrous tumors, with histopathological correlations and clinical outcome.

Authors: Zafaria G Papathanassiou, Marco Alberghini, Piero Picci, Eric Staals, Marco Gambarotti, Francesco Giuseppe Garaci and Daniel Vanel

Extensive adipocytic maturation can be seen in myxoid liposarcomas treated with neoadjuvant doxorubicin and ifosfamide and pre-operative radiation therapy

Trabectedin and thioglitazones have been documented to induce adipocytic maturation in myxoid liposarcoma; we have noted this in our experience as well. Intriguingly, we have also encountered this same phenome...

Authors: Wei-Lien Wang, Daniela Katz, Dejka M Araujo, Vinod Ravi, Joseph A Ludwig, Jonathan C Trent, Shreyaskumar R Patel, Patrick P Lin, Ashleigh Guadagnolo, Dolores Lòpez-Terrada, Angelo Paola dei Tos, Valerie O Lewis, Dina Lev, Raphael E Pollock, Gunar K Zagars, Robert S Benjamin…

The CXCR4-CXCL12 axis in Ewing sarcoma: promotion of tumor growth rather than metastatic disease

Chemokine receptor CXCR4, together with its ligand CXCL12, plays critical roles in cancer progression, including growth, metastasis and angiogenesis. Ewing sarcoma is a sarcoma with poor prognosis despite curr...

Authors: Dagmar Berghuis, Marco W Schilham, Susy J Santos, Suvi Savola, Helen J Knowles, Uta Dirksen, Karl-Ludwig Schaefer, Jukka Vakkila, Pancras CW Hogendoorn and Arjan C Lankester

Management of imatinib-associated skin rash in a patient with metastatic gastrointestinal stromal tumor: a case report

Long-term continuous imatinib is recommended for adult patients with unresectable and/or metastatic KIT+ gastrointestinal stromal tumors (GIST) as long as the patient continues to benefit. In the adjuvant sett...

Authors: Jean-Yves Blay

Extraskeletal myxoid chondrosarcoma: tumor response to sunitinib

Extraskeletal myxoid chondrosarcoma (EMCS) is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein EWSR1-CHN (the latter even ...

Authors: Silvia Stacchiotti, Gian Paolo Dagrada, Carlo Morosi, Tiziana Negri, Antonella Romanini, Silvana Pilotti, Alessandro Gronchi and Paolo G Casali

The rule of fives, a simple way to stratify risk for primary gastrointestinal stromal tumors (GIST)

The current AJCC version 7 staging system is a lugubrious means to classify gastrointestinal stromal tumors with respect to their risk and need for adjuvant systemic therapy. The rule of fives, suggested here,...

Authors: Robert G Maki

Mouse models of sarcomas: critical tools in our understanding of the pathobiology

Sarcomas are neoplastic malignancies that typically arise in tissues of mesenchymal origin. The identification of novel molecular mechanisms leading to sarcoma formation and the establishment of new therapies ...

Authors: Sean M Post

Genetically engineered mouse models and human osteosarcoma

Osteosarcoma is the most common form of bone cancer. Pivotal insight into the genes involved in human osteosarcoma has been provided by the study of rare familial cancer predisposition syndromes. Three kindred...

Authors: Alvin JM Ng, Anthony J Mutsaers, Emma K Baker and Carl R Walkley

Sarcoma risk after radiation exposure

Sarcomas were one of the first solid cancers to be linked to ionizing radiation exposure. We reviewed the current evidence on this relationship, focusing particularly on the studies that had individual estimat...

Authors: Amy Berrington de Gonzalez, Alina Kutsenko and Preetha Rajaraman

Malignant peripheral nerve sheath tumours in inherited disease

Malignant peripheral nerve sheath tumours (MPNST) are rare tumours known to occur at high frequency in neurofibromatosis 1 (NF1), but may also occur in other cancer prone syndromes.

Authors: D Gareth R Evans, Susan M Huson and Jillian M Birch

Inherited gastrointestinal stromal tumor syndromes: mutations, clinical features, and therapeutic implications

The discovery of underlying molecular genetic abnormalities in gastrointestinal stromal tumors (GISTs) such as activating mutations in the tyrosine kinase genes, KIT and platelet derived growth factor receptor-al...

Authors: Michael A Postow and Mark E Robson

Sarcomas in hereditary retinoblastoma

Children diagnosed with the hereditary form of retinoblastoma (Rb), a rare eye cancer caused by a germline mutation in the RB1 tumor suppressor gene, have excellent survival, but face an increased risk of bone an...

Authors: Ruth A Kleinerman, Sara J Schonfeld and Margaret A Tucker

The Epidemiology of Sarcoma

Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while maligna...

Authors: Zachary Burningham, Mia Hashibe, Logan Spector and Joshua D Schiffman

Hereditary and environmental epidemiology of sarcomas

Authors: David M Thomas, Sharon A Savage and Gareth L Bond

Interval compressed vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide in patients with advanced Ewing’s and other Small Round Cell Sarcomas

To evaluate tolerability and maintenance of dose intensity of 2 weekly treatment with vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide (VDC/IE) in patients with advanced small ...

Authors: Jeremy Whelan, Atia Khan, Anand Sharma, Christian Rothermundt, Palma Dileo, Maria Michelagnoli, Beatrice Seddon and Sandra Strausss

Recombinant erythropoietin for the anaemia of patients with advanced Gastrointestinal Stromal Tumours (GIST) receiving imatinib: an active agent only in non progressive patients

Recombinant erythropoietin for the anaemia of patients with advanced Gastrointestinal Stromal Tumours (GIST) receiving imatinib : an active agent only in non progressive patients.

Authors: Florence Duffaud, Caroline Even, Isabelle Ray-Coquard, Emmanuelle Bompas, Thanh Khoa-Huynh, Sebastien Salas, Philippe Cassier, Armelle Dufresne, Sylvie Bonvalot, Francoise Ducimetiere, Axel Le Cesne and Jean-Yves Blay

Phenol levels during intralesional curettage and local adjuvant treatment of benign and low-grade malignant bone tumours

Phenol is widely used for years as local adjuvant treatment for bone tumours. Despite its use for a long time, no information is available about the local concentration of phenol that is achieved in an individ...

Authors: Suzan HM Verdegaal, Jan den Hartigh, Pancras CW Hogendoorn, Hugo FG Brouwers and Antonie HM Taminiau

Serum tumor markers in pediatric osteosarcoma: a summary review

Osteosarcoma is the most common primary high-grade bone tumor in both adolescents and children. Early tumor detection is key to ensuring effective treatment. Serum marker discovery and validation for pediatric...

Authors: Yulia A Savitskaya, Genaro Rico-Martínez, Luis Miguel Linares-González, Ernesto Andrés Delgado-Cedillo, René Téllez-Gastelum, Alfonso Benito Alfaro-Rodríguez, Antonio Redón-Tavera and José Clemente Ibarra-Ponce de León

Histone deacetylase inhibitors enhance expression of NKG2D ligands in Ewing sarcoma and sensitize for natural killer cell-mediated cytolysis

Ewing sarcoma patients have a poor prognosis despite multimodal therapy. Integration of combination immunotherapeutic strategies into first-/second-line regimens represents promising treatment options, particu...

Authors: Dagmar Berghuis, Marco W Schilham, Hanneke I Vos, Susy J Santos, Stephan Kloess, Emilie P Buddingh', R Maarten Egeler, Pancras CW Hogendoorn and Arjan C Lankester

The clinical use of biomarkers as prognostic factors in Ewing sarcoma

Ewing Sarcoma is the second most common primary bone sarcoma with 900 new diagnoses per year in Europe (EU27). It has a poor survival rate in the face of metastatic disease, with no more than 10% survival of t...

Authors: Annmeik M van Maldegem, Pancras CW Hogendoorn and Andrew B Hassan

Molecular pathogenesis and targeted therapeutics in Ewing sarcoma/primitive neuroectodermal tumours

Ewing sarcoma/PNET is managed with treatment paradigms involving combinations of chemotherapy, surgery, and sometimes radiation. Although the 5-year survival rate of non-metastatic disease approaches 70%, thos...

Authors: Fergal C Kelleher and David M Thomas

Comprehensive analysis of published phase I/II clinical trials between 1990-2010 in osteosarcoma and Ewing sarcoma confirms limited outcomes and need for translational investment

High grade primary bone sarcomas are rare cancers that affect mostly children and young adults. Osteosarcoma and Ewing sarcoma are the most common histological subtypes in this age group, with current multimod...

Authors: Annemiek M van Maldegem, Aparna Bhosale, Hans J Gelderblom, Pancras CW Hogendoorn and Andrew B Hassan

Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a relatively common soft-tissue tumor. A more aggressive appearing fibrosarcoma may arise in DFSP, changing its biological behavior. CD34 and apolipoprotein-D are high...

Authors: Emanuela Palmerini, Marco Gambarotti, Eric L Staals, Licciana Zanella, Gabriela Sieberova, Alessandra Longhi, Marilena Cesari, Stefano Bonarelli, Piero Picci, Pietro Ruggieri, Marco Alberghini and Stefano Ferrari

Paratesticular desmoplastic small round cell tumour: an unusual tumour with an unusual fusion; cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH

Desmoplastic small round cell tumour is a rare malignant tumour with a male to female ratio of 4:1. It manifests mostly at serosal sites. Here we present a case of a 28-year-old male patient, who presented wit...

Authors: Vincent PM Cliteur, Károly Szuhai, Hans J Baelde, Jurriaan van Dam, Hans Gelderblom and Pancras CW Hogendoorn

Efficacy of first-line doxorubicin and ifosfamide in myxoid liposarcoma

Myxoid liposarcoma (MLS) is a soft tissue sarcoma with adipocytic differentiation characterized by a unique chromosome rearrangement, t(12;16)(q13;p11). The exact efficacy of chemotherapy in MLS has not been c...

Authors: Daniela Katz, Piyaporn Boonsirikamchai, Haeson Choi, Alexander J Lazar, Wei-Lein Wang, Lianchun Xiao, Min S Park, Vinod Ravi, Robert S Benjamin and Dejka M Araujo

An increased incidence of Hodgkin's lymphoma in patients with adult-onset sarcoma

Sarcomas are rare, often fatal malignancies of connective tissues that can occur in genetic predisposition syndromes or result from carcinogen exposure. Hodgkin's lymphoma (HL) is not known to contribute to an...

Authors: Megan E Downing, Gillian S Dite and Mandy L Ballinger

Reclassification and subtyping of so-called malignant fibrous histiocytoma of bone: comparison with cytogenetic features

The diagnostic entity malignant fibrous histiocytoma (MFH) of bone is, like its soft tissue counterpart, likely to be a misnomer, encompassing a variety of poorly differentiated sarcomas. When reviewing a seri...

Authors: Fredrik Mertens, Salvatore Romeo, Judith VMG Bovée, Roberto Tirabosco, Nick Athanasou, Marco Alberghini, Pancras CW Hogendoorn, Angelo P Dei Tos, Raf Sciot, Henryk A Domanski, Kristina Åström, Nils Mandahl and Maria Debiec-Rychter

Vaginal metastasis of a Ewing sarcoma five years after resection of the primary tumor

A 35-year-old female presented with pain and swelling of the distal left radius. A diagnosis of Ewing sarcoma was made and she underwent neoadjuvant chemotherapy and surgery. Macroscopic viable areas remained ...

Authors: Noemie Vanel, Victoire Vierling, Jennifer Kreshak, Marco Gambarotti, Stefania Cocchi, Cristina Tranfaglia and Daniel Vanel

Liposarcoma cells with aldefluor and CD133 activity have a cancer stem cell potential

Aldehyde dehydrogenase (ALDH) has recently been shown to be a marker of cancer stem-like cells (CSCs) across tumour types. The primary goals of this study were to investigate whether ALDH is expressed in lipos...

Authors: Eva W Stratford, Russell Castro, Anna Wennerstrøm, Ruth Holm, Else Munthe, Silje Lauvrak, Bodil Bjerkehagen and Ola Myklebost

Local recurrence and assessment of sentinel lymph node biopsy in deep soft tissue leiomyosarcoma of the extremities

Leiomyosarcoma of deep soft tissues of the extremities is a rare malignant tumour treated primarily by surgery. The incidence of local recurrence and lymph node metastasis is uncertain and it is not known whet...

Authors: Michael J Lamyman, Henk P Giele, Paul Critchley, Duncan Whitwell, Max Gibbons and Nicholas A Athanasou

Guidelines for histopathological specimen examination and diagnostic reporting of primary bone tumours

This review is intended to provide histopathologists with guidelines for clinical assessment, specimen handling and diagnostic reporting of benign and malignant primary bone tumours. Information from radiology...

Authors: D Charles Mangham and Nicholas A Athanasou

Expression of aromatase and estrogen receptor alpha in chondrosarcoma, but no beneficial effect of inhibiting estrogen signaling both in vitro and in vivo

Chondrosarcomas are malignant cartilage-forming tumors which are highly resistant to conventional chemotherapy and radiotherapy. Estrogen signaling is known to play an important role in proliferation and diffe...

Authors: Danielle Meijer, Hans Gelderblom, Marcel Karperien, Anne-Marie Cleton-Jansen, Pancras CW Hogendoorn and Judith VMG Bovée

Frequent activation of EGFR in advanced chordomas

Chordomas are rare neoplasms, arising from notochordal remnants in the midline skeletal axis, for which the current treatment is limited to surgery and radiotherapy. Recent reports suggest that receptor tyrosi...

Authors: Barbara Dewaele, Francesca Maggiani, Giuseppe Floris, Michèle Ampe, Vanessa Vanspauwen, Agnieszka Wozniak, Maria Debiec-Rychter and Raf Sciot

Imaging of hibernomas: A retrospective study on twelve cases

To analyze the imaging features of hibernomas on computed tomography (CT) and magnetic resonance (MRI).

Authors: Zafiria G Papathanassiou, Marco Alberghini, Sophie Taieb, Costantino Errani, Piero Picci and Daniel Vanel

Parosteal osteosarcoma mimicking osteochondroma: A radio-histologic approach on two cases

Parosteal osteosarcoma is a well-differentiated variant of osteosarcoma that affects the surface of the bone. The imaging pattern is very typical. We report two cases mimicking an osteochondroma, radiologicall...

Authors: Zafiria G Papathanassiou, Marco Alberghini, Philippe Thiesse, Marco Gambarotti, Giuseppe Bianchi, Cristina Tranfaglia and Daniel Vanel

A new journal... on spindle cells

Welcome to Clinical Sarcoma Research a new open access, online medical journal providing a forum for clinical knowledge on rare solid cancers - sarcomas. We believe there is a vacuum, which this effort may hope t...

Authors: Paolo G Casali and Angelo P Dei Tos