The immune microenvironment of uterine adenosarcomas
ConclusionsIn conclusion, this is the first report characterizing the presence of immune infiltrate and PD-1/PD-L1 expression in UA. CD3+ CD8+ T-cells density may be prognostic. The immune-responsiveness of UA needs to be further investigated in a larger study. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - March 28, 2020 Category: Cancer & Oncology Source Type: research

Accurate 3-gene-signature for early diagnosis of liposarcoma progression
ConclusionsThe identified metabolic signature allows highly accurate differential diagnosis between WD- and DDLPS even in samples containing lipid droplets, a marker of differentiation, which makes it very suitable for the use on biopsies. In respect to the pathogenesis of the disease, our results give a new insight into possible molecular mechanisms involved and support the recent observation that deletion ofPNPLA2 is a novel factor in liposarcoma progression. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - March 5, 2020 Category: Cancer & Oncology Source Type: research

Cancer/testis antigens expression during cultivation of melanoma and soft tissue sarcoma cells
AbstractBackgroundAutologous dendritic cells (DC) loaded with tumor-associated antigens (TAAs) are a promising approach for anticancer immunotherapy. Polyantigen lysates appear to be an excellent source of TAAs for loading onto the patient ’s dendritic cells. Cancer/testis antigens (CTA) are expressed by a wide range of tumors, but are minimally expressed on normal tissues, and could serve as a universal target for immunotherapy. However, CTA expression levels can vary significantly in patients with the same tumor type. We proposed that patients who do not respond to DC-based therapy may have distinct features of the...
Source: Clinical Sarcoma Research - February 4, 2020 Category: Cancer & Oncology Source Type: research

Use of a simple form to facilitate communication on long-term consequences of treatment in sarcoma survivors
AbstractBackgroundTo report on our experience using a simple optional form to facilitate communication on late effects between the patients and the oncologists during outpatient follow-up and to detail on the spectrum of challenges reported by sarcoma survivors.MethodsThe form was presented for the patients to complete before their consultation and covered topics related to late effects and unmet needs that the patient wished to discuss with the medical personnel. Logistic regression analysis examined how the distribution of the topics varied with age, gender, diagnosis and type of treatment received.ResultsThe form was ma...
Source: Clinical Sarcoma Research - January 16, 2020 Category: Cancer & Oncology Source Type: research

Toxicity management of regorafenib in patients with gastro-intestinal stromal tumour (GIST) in a tertiary cancer centre
ConclusionAlthough PD was the main reason for discontinuing treatment, toxicity management and dosing of regorafenib remains critical. Median duration of treatment was longer compared to previous studies suggesting a durable clinical benefit with regorafenib with rigorous toxicity management. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - January 4, 2020 Category: Cancer & Oncology Source Type: research

A clinico-genomic analysis of soft tissue sarcoma patients reveals CDKN2A deletion as a biomarker for poor prognosis
ConclusionOur clinico-genomic profiling of STS shows thatCDKN2A deletion was the most prevalent DNA copy number aberration and was associated with poor prognosis. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - September 11, 2019 Category: Cancer & Oncology Source Type: research

The challenge of sarcomas: the patient advocacy group perspective
ConclusionsAcross many of the challenges which I identify good progress is being made. The importance of the partnership with the professional specialists in treating sarcoma cannot be emphasised too strongly and the leadership of key people, whether patient advocates or professionals, is acknowledged. There are challenges indicated which have yet to be properly addressed. Inevitably some of them have characteristics which make them especially problematic and they tend to drop lower on everyone ’s agendas. This does not mean we should forget them. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 17, 2019 Category: Cancer & Oncology Source Type: research

A rare presentation of carcinosarcoma of the bone in a young female; response with gemcitabine and docetaxel
ConclusionsPrimary carcinosarcoma of the bone is an extremely rare malignancy. Early diagnosis is crucial as localized disease may be curable with resection. As shown in this case, combination chemotherapy with gemcitabine and docetaxel is a potential option in patients with unresectable or metastatic disease. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 2, 2019 Category: Cancer & Oncology Source Type: research

Radiotherapy resistance in chondrosarcoma cells; a possible correlation with alterations in cell cycle related genes
ConclusionsChondrosarcoma cell lines as well as primary tumors are variably radioresistant, particularly in case of a defective Rb pathway. Whether selection for radiotherapy can be based upon an intact Rb pathway should be further investigated. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 28, 2019 Category: Cancer & Oncology Source Type: research

Immunophenotypic expression of UCP1 in hibernoma and other adipose/non adipose soft tissue tumours
ConclusionsUCP1 is strongly expressed in BAT but not WAT and is found in all hibernomas and a few pleomorphic liposarcomas but not in other adipose tumours. UCP1 expression in a few non-adipose soft tissue sarcomas may possibly reflect origin of tumour cells from a common mesenchymal stem cell precursor and/or developmental pathway. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 13, 2019 Category: Cancer & Oncology Source Type: research

Correction to: Survival in advanced GIST has improved over time and correlates with increased access to post-imatinib tyrosine kinase inhibitors: results from Life Raft Group Registry
The legends for Figs.  3 and 5 of the article [1] incorrectly refer to an “all others group”. The corrected legends should read: (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 7, 2019 Category: Cancer & Oncology Source Type: research

Perspectives on treatment side effects in patients with metastatic gastrointestinal stromal tumour: a qualitative study
ConclusionsWe observed a gap between the biomedical perspective on disease that health professionals typically adopt and the individual experiences of patients living with metastatic GIST. For those patients who are living in limbo between having metastatic cancer and offered an effective treatment, a holistic view of health on the part of their healthcare providers seems crucial. A vital goal should hence be to improve communication between healthcare professionals and GIST patients so as to secure an individualised follow-up with guidance on coping with, and adapting to, their new normal.Trial registration The study was ...
Source: Clinical Sarcoma Research - April 30, 2019 Category: Cancer & Oncology Source Type: research

The challenge of finding new therapeutic avenues in soft tissue sarcomas
AbstractSoft tissue sarcomas are rare malignancies of mesenchymal origin comprising about 1% of all adult cancers. Systemic therapies for locally advanced and metastatic disease have been restricted for decades to very few effective and approved agents such as doxorubicin and ifosfamide. However, new therapeutic avenues including new drug developments and registrations such as trabectedin, pazopanib and eribulin as well as numerous clinical trial options have recently enriched the therapeutic armamentarium in the treatment of patients with advanced soft tissue sarcomas. The challenges and pitfalls of finding such new thera...
Source: Clinical Sarcoma Research - April 10, 2019 Category: Cancer & Oncology Source Type: research

Survival in advanced GIST has improved over time and correlates with increased access to post-imatinib tyrosine kinase inhibitors: results from Life Raft Group Registry
ConclusionsIn our analysis, sunitinib and regorafenib significantly improved OS compared with patients who never received these agents. Our data also support the hypothesis that the use of KIT/PDGFRA inhibitors, including non-approved agents, has improved OS for patients with imatinib- and sunitinib-resistant GIST. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - April 2, 2019 Category: Cancer & Oncology Source Type: research

Genomic aberrations in cell cycle genes predict progression of KIT -mutant gastrointestinal stromal tumors (GISTs)
AbstractBackgroundActivating mutations of the receptor tyrosine kinaseKIT are early events in the development of most gastrointestinal stromal tumors (GISTs). Although GISTs generally remain dependent on oncogenicKIT during tumor progression,KIT mutations alone are insufficient to induce malignant behavior. This is evidenced byKIT-mutant micro-GISTs, which are present in up to one-third of normal individuals, but virtually never progress to malignancy.MethodsWe performed whole exome sequencing on 29 tumors obtained from 21 patients with high grade or metastaticKIT-mutant GIST (discovery set). We further validated the frequ...
Source: Clinical Sarcoma Research - March 5, 2019 Category: Cancer & Oncology Source Type: research

Genome-wide methylation profiling and copy number analysis in atypical fibroxanthomas and pleomorphic dermal sarcomas indicate a similar molecular phenotype
ConclusionsOur findings support the concept that AFX and PDS belong to a common tumor spectrum. We could demonstrate the diagnostic value of DNA-methylation profiling to delineating AFX/PDS from potential mimics. However, the assessment of certain histologic features remains crucial for separating PDS from AFX. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - February 14, 2019 Category: Cancer & Oncology Source Type: research

Long-term cure of soft tissue sarcoma with pegylated-liposomal doxorubicin after doxorubicin and ifosfamide failure
ConclusionsThis case illustrates the curative potential of PLD after failure of free doxorubicin and the absence of long term cardiotoxicity with PLD. As with all drugs, individual adjustment of dose and treatment interval is important. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - January 15, 2019 Category: Cancer & Oncology Source Type: research

Dedifferentiated chondrosarcoma of the pelvis: clinical outcomes and current treatment
ConclusionsPelvic dedifferentiated CS often presents at an advanced local or systemic stage and confers a poor prognosis. Achieving wide surgical margins (>  4 mm) provided the highest rate of long-term disease-free survival. Failing to achieve wide margins results in rapid disease recurrence, conferring deleterious consequences. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - December 14, 2018 Category: Cancer & Oncology Source Type: research

Extraskeletal myxoid chondrosarcoma with massive pulmonary metastases
ConclusionWhile surgical management of EMC with or without radiation therapy is still the preferable approach when feasible, small series support the use of tyrosine kinase inhibitors and possible new immunotherapies in selected patients. Basket trials focusing on diseases with unique genomic features such as EMC will hopefully provide a better understanding of new options for care. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - December 5, 2018 Category: Cancer & Oncology Source Type: research

A phase II study of temsirolimus and liposomal doxorubicin for patients with recurrent and refractory bone and soft tissue sarcomas
ConclusionsOur study adds to the literature supporting the addition of mTOR inhibition to chemotherapy agents for the treatment of sarcomas, and proposes that a mechanism by which mTOR inhibition enhances the efficacy of chemotherapy may be through sensitizing the chemoresistant CSC population. Further study, ideally with pre- and post-therapy assessment of ALDH expression in tumor cells, is warranted.Trial registration The trial was registered on clinicaltrials.gov (NCT00949325) on 30 July 2009.http://www.editorialmanager.com/csrj/default.aspx (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - November 5, 2018 Category: Cancer & Oncology Source Type: research

Descending thoracic aortic aneurysm revealing metastasis of a soft tissue fibrosarcoma: a case report and review of the literature
ConclusionThe presence of an aneurysm in a patient with a sarcoma with a high potential for metastasis and poor cardiovascular risk factors should alert physicians. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - October 26, 2018 Category: Cancer & Oncology Source Type: research

Preoperative radiotherapy of soft-tissue sarcomas: surgical and radiologic parameters associated with local control and survival
ConclusionPreoperative radiotherapy of high-risk soft-tissue sarcomas allows for good local control rate at the expense of local wound complications, which are however manageable. Marginal excision is sufficient for local control. Absence of peritumoural oedema on MRI, as well as tumour size reduction following radiotherapy are associated to superior patient survival and can be used ass early prognostic factors. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - October 5, 2018 Category: Cancer & Oncology Source Type: research

Periostin expression in neoplastic and non-neoplastic diseases of bone and joint
ConclusionsIn keeping with its known role in modulating the synthesis of collagen and other extracellular matrix proteins in bone, strong periostin expression was noted in benign and malignant lesions forming an osteoid or osteoid-like matrix. Periostin was also noted in other bone tumours and was found in areas of reactive bone and increased vascularity at the edge of growing tumours, consistent with its involvement in tissue remodelling and angiogenesis associated with tumour progression. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - September 5, 2018 Category: Cancer & Oncology Source Type: research

Low-grade central fibroblastic osteosarcoma may be differentiated from its mimicker desmoplastic fibroma by genetic analysis
ConclusionsKaryotyping and molecular genetic analysis may contribute to a conclusive diagnosis. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 23, 2018 Category: Cancer & Oncology Source Type: research

Report from the 4th European Bone Sarcoma Networking meeting: focus on osteosarcoma
This report summarizes the proceedings of the 4th European Bone Sarcoma Networking Meeting, held in London, England, on 21 June 2017. The meeting brought together scientific and clinical researchers and representatives from sarcoma charities from 19 countries representing five networks across Europe, to present and discuss new developments on bone sarcoma. In view of the challenges is poses, the meeting focussed primarily on osteosarcoma with presentations on developments in our understanding of osteosarcoma genetics and immunology as well as results from preclinical investigations and discussion of recent and ongoing clin...
Source: Clinical Sarcoma Research - August 10, 2018 Category: Cancer & Oncology Source Type: research

PDGF/PDGFR effects in osteosarcoma and the “add-on” strategy
AbstractNew 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. Targeting the PDGF/PDGFR pathway is a promising therapeutic method to overcome drug resistance and ...
Source: Clinical Sarcoma Research - August 2, 2018 Category: Cancer & Oncology Source Type: research

Tenosynovial giant cell tumor: case report of a patient effectively treated with pexidartinib (PLX3397) and review of the literature
ConclusionsThis case illustrates the effectiveness of systemic therapy in controlling a disease associated with high surgical morbidity. This approach may be especially useful in the treatment of extra-articular disease which often invades neurovascular bundles; as the effectiveness in metastatic disease is still unknown. In the future, systemic treatment for TGCT may be appropriate for the neoadjuvant setting to decrease disease burden prior to surgery with the aim of decreasing recurrence rates. However, properly designed prospective studies will need to be carried out to answer these questions. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 10, 2018 Category: Cancer & Oncology Source Type: research

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
AbstractBackgroundInduction 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-ope rative radiotherapy with patients undergoing limb-conserving surgery and routine post-operative radiotherapy.MethodsPatients with primary, grade 2/3 sarcomas of the lower limbs over 10  cm in size were ...
Source: Clinical Sarcoma Research - July 2, 2018 Category: Cancer & Oncology Source Type: research

Reversible rituximab-induced rectal Kaposi ’s sarcoma misdiagnosed as ulcerative colitis in a patient with HIV-negative follicular lymphoma
ConclusionsTo our knowledge, this is the third case of Kaposi ’s sarcoma diagnosed under rituximab in a HIV-negative patient, the first one at the rectal level and the first one that completely regresses after stop of rituximab. This case raises awareness of iatrogenic Kaposi’s sarcoma in HIV-negative patients treated with rituximab, and further highlights the importance of immunosuppression in the pathophysiology of disease. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - June 11, 2018 Category: Cancer & Oncology Source Type: research

Non-canonical WNT6/WNT10A signal factor expression in EBV+ post-transplant smooth muscle tumors
AbstractPost-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 mech anisms still remain unclear. Gene expression analysis of a set of morphologically similar tumors (leiomyomas, leiomyosarcomas, ang...
Source: Clinical Sarcoma Research - June 4, 2018 Category: Cancer & Oncology Source Type: research

Aggressive fibromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response
ConclusionsNo relationship was identified between symptomatic benefit and response by RECIST 1.1 on MRI. Prospective studies in AF should incorporate endpoints focusing on patient symptoms. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 14, 2018 Category: Cancer & Oncology Source Type: research

Treatment patterns and survival among older adults in the United States with advanced soft-tissue sarcomas
ConclusionsAlthough previous studies of younger populations reported anthracycline-based therapy predominated in first line, our study of older advanced STS patients found that docetaxel plus gemcitabine was most commonly used. Despite variation by histologic category, prognosis remains poor for older adult patients with advanced STS. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 3, 2018 Category: Cancer & Oncology Source Type: research

Smoking is predictive of poorer distant metastasis-free and progression free-survival in soft tissue sarcoma patients treated with pre-operative radiotherapy or chemoradiotherapy
ConclusionsCurrent smokers and patients with a significant smoking history demonstrated decreased DMFS and PFS in STS patients treated with pre-operative RT. Smoking may cause immunologic compromise and therefore lead to higher rates of progression and distant metastasis. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - April 16, 2018 Category: Cancer & Oncology Source Type: research

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
ConclusionsPET –CT in high-grade bone and soft tissue sarcoma can add significant benefit to routine CT/MRI staging. Further prospective and multi-centre evaluation of PET–CT is warranted to determine the actual predictive value and cost-effectiveness of PET–CT in directing clinical management of clinically complex and heterogeneous high-grade sarcomas. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - April 9, 2018 Category: Cancer & Oncology Source Type: research

Unicentric epithelioid hemangioendothelioma of the calcaneus: a case report and review of literature
ConclusionThis case has clinical follow-up greater than 2  years post-operatively and could be a guide for treatment of a rare disorder with a substantial paucity of literature. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - April 6, 2018 Category: Cancer & Oncology Source Type: research

Survival is influenced by approaches to local treatment of Ewing sarcoma within an international randomised controlled trial: analysis of EICESS-92
ConclusionsUnexpected differences in EFS and OS occurred between two patient cohorts recruited within an international randomised trial. Failure to select or deliver appropriate local treatment modalities for Ewing ’s sarcoma may compromise chances of cure.Trial registration Supported by Deutsche Krebshilfe (Grants No. DKH M43/92/J ü2 and DKH 70-2551 Jü3), and European Union Biomedicine and Health Programme (Grants No. BMH1-CT92-1341 and BMH4-983956), and Cancer Research United Kingdom. Clinical trial information can be found for the following: NCT0000251 (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - March 30, 2018 Category: Cancer & Oncology Source Type: research