Primary pulmonary artery sarcoma masquerading as pulmonary thromboembolism: a rare diagnosis unveiled
ConclusionGreat similarity with significant degree of overlap in clinical and radiologic presentation makes differentiation of pulmonary artery sarcomas and thromboembolism a diagnostic challenge. Even though they are exceptionally rare, one should always consider it as differential diagnosis especially in cases with atypical clinical or imaging presentation. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - July 1, 2017 Category: Cancer & Oncology Source Type: research

Overexpressed PRAME is a potential immunotherapy target in sarcoma subtypes
ConclusionsWe determined that uterine carcinosarcoma, synovial sarcoma, and leiomyosarcoma patients would potentially benefit from PRAME-specific immunotherapies. Tumor escape through loss of antigen presentation needs to be further studied. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - June 15, 2017 Category: Cancer & Oncology Source Type: research

Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease
ConclusionsDespite adequate primary treatment, distant and disseminated metastatic disease may be not excluded in leiomyosarcoma of the head and neck. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - June 6, 2017 Category: Cancer & Oncology Source Type: 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)
ConclusionsDespite very limited activity of antiangiogenics in our study, prospective collection of cases of these rare tumors together with molecular data should guide therapeutic decision and enhance outcome. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 10, 2017 Category: Cancer & Oncology Source Type: research

Histone 3.3 hotspot mutations in conventional osteosarcomas: a comprehensive clinical and molecular characterization of six H3F3A mutated cases
ConclusionsH3.3 mutations in osteosarcomas may occur inH3F3A at mutational hotspots. They are overall rare, but become more frequent in osteosarcoma patients older than 30  years. Osteosarcomas carryingH3F3A G34W/R mutations are associated with epigenetic dysregulation ofKLLN/PTEN andHIST1H2BB. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 4, 2017 Category: Cancer & Oncology Source Type: research

IDH1 or - 2 mutations do not predict outcome and do not cause loss of 5-hydroxymethylcytosine or altered histone modifications in central chondrosarcomas
ConclusionsIn summary, in central chondrosarcomaIDH1 or -2 mutations do not affect immunohistochemical levels of 5-hmC, 5mC, trimethylation of H3K4, -K9 and K27 and outcome, as compared to wildtype. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 4, 2017 Category: Cancer & Oncology Source Type: research

Erratum to: Osteosarcoma follow-up: chest X-ray or computed tomography?
(Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - May 3, 2017 Category: Cancer & Oncology Source Type: research

UK clinical practice guidelines for the management of gastrointestinal stromal tumours (GIST)
ConclusionsThe guidelines cover aetiology, genetics and underlying molecular mechanisms, diagnosis and initial investigations, staging and risk stratification, surgery, neoadjuvant and adjuvant therapy, the management of advanced disease and follow-up. The importance of mutational analysis in guiding treatment is highlighted, since this can indicate the most effective treatment and avoid administration of ineffective drugs, emphasising the need for management in specialist centres. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - April 21, 2017 Category: Cancer & Oncology Source Type: research

Two years survival of primary cardiac leiomyosarcoma managed by surgical and adjuvant therapy
ConclusionsCardiac leiomyosarcoma is a rare tumor with a dismal prognosis. Surgery is the mainstay of treatment. Adjuvant treatment is still controversial. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - March 8, 2017 Category: Cancer & Oncology Source Type: research

How a Clinical Trial Unit can improve independent clinical research in rare tumors: the Italian Sarcoma Group experience
ConclusionThe contribution of ISG researchers to the international community was striking from the earliest years of the ISG creation. The challenges of the regulatory clinical research scenario, which imposes solid and hard-fast methodology with deep knowledge and expertise, highlighted the need to identify qualified and dedicated experts able to run and follow the multifaceted aspects of trials. Our analysis demonstrated how this model has led to a growth in competitiveness of the group. The collaboration between clinicians and CTU made possible to support the research with high scientific and ethical standards and to in...
Source: Clinical Sarcoma Research - February 27, 2017 Category: Cancer & Oncology Source Type: research

Osteosarcoma follow-up: chest X-ray or computed tomography?
ConclusionsA follow-up strategy with chest CT leads to a higher rate of CR-2 and significantly improves PRS and OS in osteosarcoma, compared to chest X-ray. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - February 13, 2017 Category: Cancer & Oncology Source Type: research

Central venous access related adverse events after trabectedin infusions in soft tissue sarcoma patients; experience and management in a nationwide multi-center study
ConclusionTrabectedin infusion commonly leads to central venous access related adverse events. Sterile inflammation along the catheter trajectory is one of the most common adverse events and can be prevented by placing the catheter deeper under the skin. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - January 30, 2017 Category: Cancer & Oncology Source Type: research

Radiotherapy in the management of gist: state of the art and new potential scenarios
ConclusionsThese and other successful experiences that are progressively accumulating, open up new scenarios of use of radiation therapy in various settings of treatment. GIST is not universally radioresistant and radiotherapy, especially if combined with molecularly targeted therapy, can improve the outcomes for patients diagnosed with GIST. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - January 9, 2017 Category: Cancer & Oncology Source Type: research

Metastatic meningioma presenting as a malignant soft tissue tumour
ConclusionsMetastatic malignant meningioma can very rarely present as a high grade pleomorphic malignant soft tissue tumour and needs to be distinguished from soft tissue sarcomas and metastatic carcinomas that express epithelial antigens. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - December 29, 2016 Category: Cancer & Oncology Source Type: research

Response to anti-PD1 therapy with nivolumab in metastatic sarcomas
ConclusionsThese data provide a rationale for further exploring the efficacy of nivolumab and other checkpoint inhibitors in soft tissue and bone sarcoma. (Source: Clinical Sarcoma Research)
Source: Clinical Sarcoma Research - December 29, 2016 Category: Cancer & Oncology Source Type: research