Survival of soft tissue sarcoma patients after completing six cycles of first-line anthracycline containing treatment: an EORTC-STBSG database study
ConclusionThis large retrospective study is the first to report progression free survival and overall survival after completion of 6 cycles of first line doxorubicin containing chemotherapy. These results are important when designing new studies exploring for example maintenance therapy after doxorubicin based chemotherapy. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - September 9, 2020 Category: Cancer & Oncology Source Type: research

Anthracycline-based and gemcitabine-based chemotherapy in the adjuvant setting for stage I uterine leiomyosarcoma: a retrospective analysis at two reference centers
ConclusionsDFS was not the same by using an anthracycline-based or a gemcitabine-based adjuvant chemotherapy for patients with radically resected eULMS. The results of our study are in line with recent prospective controlled evidence in limb and superficial trunk soft tissue sarcomas. The role of anthracycline-based adjuvant chemotherapy should still be viewed as a research issue in eULMS. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 28, 2020 Category: Cancer & Oncology Source Type: research

Long lasting response with trabectedin monotherapy in relapsed metastatic mesenchymal chondrosarcoma
ConclusionThis report aims to present a comprehensive review into available and newer treatment choices for mesenchymal chondrosarcoma, and to highlight trabectedin monotherapy as a possible therapeutic option for mesenchymal chondrosarcoma in the relapsed setting. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 27, 2020 Category: Cancer & Oncology Source Type: research

Paraneoplastic dermatomyositis associated with metastatic leiomyosarcoma of unknown primary
DiscussionParaneoplastic dermatomyositis heralded disease progression after first-line chemotherapy; however, in hindsight, subtle cutaneous features were present at sarcoma diagnosis. The temporal relationship between paraneoplastic dermatomyositis and metastatic leiomyosarcoma is key in this case, as fluctuations in dermatomyositis severity correlated with growth of metastatic disease. Understanding this relationship may provide clues for tumour progression and prompt timely initiation of anti-cancer therapy. It is important to recognise that in addition to the more common cancers associated with paraneoplastic dermatomy...
Source: Clinical Sarcoma Research - August 25, 2020 Category: Cancer & Oncology Source Type: research

Treatment of gastrointestinal tumor (GIST) of the rectum requiring abdominoperineal resection following neoadjuvant imatinib: a cost-effectiveness analysis
ConclusionUAPR is more effective and less costly than CIUP for patients with rectal GIST requiring abdominoperineal resection following neoadjuvant imatinib, and is the strategy of choice in this setting. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 6, 2020 Category: Cancer & Oncology Source Type: research

Successful treatment of lipofibromatosis-like neural tumor of the lumbar spine with an NTRK-fusion inhibitor
ConclusionsThe exceptional treatment response of this patient suggests that NTRK fusions are true drivers of the disease. Thus, all patients should be evaluated for NTRK fusions using sensitive methodologies and treatment with TRK fusion-protein inhibitors should be considered in patients who are not candidates for oncologic resection. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - August 6, 2020 Category: Cancer & Oncology Source Type: research

A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids
ConclusionsThese cases are often misdiagnosed and despite a delay in diagnosis have good outcomes with appropriate multimodality management. This case highlights the unique and typical characteristics of kaposiform haemangioendothelioma. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 30, 2020 Category: Cancer & Oncology Source Type: research

Chemotherapy improves distant control in localized high-grade soft tissue sarcoma of the extremity/trunk
ConclusionIn patients with localized STS  >  5 cm, the high-grade subset had a distant control benefit with the addition of chemotherapy, leading to improved progression free survival. This is more pronounced with the use of MAI and should be considered in patients eligible for this regimen. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 9, 2020 Category: Cancer & Oncology Source Type: research

Good and sustained response to pembrolizumab and pazopanib in advanced undifferentiated pleomorphic sarcoma: a case report
ConclusionsUndifferentiated pleomorphic sarcoma is an immunologically active subtype of soft tissue sarcoma, which is particularly amenable to immune checkpoint inhibitors. Pazopanib with immune checkpoint inhibitors is a well-tolerated, yet hitherto underexplored combination that may offer significant clinical benefit in advanced sarcomas —this finding warrants further evaluation in clinical trials. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 9, 2020 Category: Cancer & Oncology Source Type: research

Real-world experience with doxorubicin and olaratumab in soft tissue sarcomas in England and Northern Ireland
ConclusionsOur results are in keeping with the phase III study findings: response rate, PFS and OS were similar to those reported in the phase III ANNOUNCE trial. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 6, 2020 Category: Cancer & Oncology Source Type: research

Evaluation of the accuracy of algorithms to identify soft tissue sarcoma (STS) in administrative claims
ConclusionsThe algorithms tested in this study sample did not achieve sufficient performance and suggest the ability to accurately identify the STS population in administrative data is problematic. Difficulties are likely due to the origin of STS in a variety of locations, the non-specific symptoms of STS, and the common diagnostic tests recommended to diagnose the disease. Future research applying machine learning to examine timing and patterns of variables that comprise the diagnostic process may further investigate the ability to accurately identify STS cases in claims databases. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 5, 2020 Category: Cancer & Oncology Source Type: research

The anti-neoplastic effect of doxycycline in osteosarcoma as a metalloproteinase (MMP) inhibitor: a systematic review
ConclusionDoxycycline remains a promising chemotherapeutic agent against osteosarcoma via MMP inhibition, showing the need for further in vivo and clinical trials to be carried out in the future. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - April 30, 2020 Category: Cancer & Oncology Source Type: research

Dedifferentiated soft tissue leiomyosarcoma with heterologous osteosarcoma component: case report and review of the literature
We report the case of a 65-year-old male patient who, after initial inadequate surgery of a tumor of the left forearm, developed local recurrence that was treated with neoadjuvant chemotherapy, surgery and postoperative radiation therapy. Histologically the tumor showed an abrupt separation of two different patterns. One component consisted of interlacing fascicles of spindle cells with cigar-shaped nuclei strongly positive for smooth muscle actin, desmin and H-caldesmon. The other component consisted of a high-grade pleomorphic sarcoma with osteoid and chondroid matrix production, which positive for SATB2. Thus, a final d...
Source: Clinical Sarcoma Research - April 5, 2020 Category: Cancer & Oncology Source Type: research

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