Use of a simple form to facilitate communication on long-term consequences of treatment in sarcoma survivors
To 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 challe...Clinical Sarcoma Research 2020 10:2Toxicity management of regorafenib in patients with gastro-intestinal stromal tumour (GIST) in a tertiary cancer centre
Regorafenib is a multi-kinase inhibitor approved as third line treatment for metastatic GIST. Dose limiting toxicities are frequently seen and many patients require dose reductions. This study aimed to evaluat...Clinical Sarcoma Research 2020 10:1A clinico-genomic analysis of soft tissue sarcoma patients reveals CDKN2A deletion as a biomarker for poor prognosis
Sarcomas are a rare, heterogeneous group of tumors with variable tendencies for aggressive behavior. Molecular markers for prognosis are needed to risk stratify patients and identify those who might benefit fr...Clinical Sarcoma Research 2019 9:12The challenge of sarcomas: the patient advocacy group perspective
The patient advocacy agenda covers every aspect of cancer treatment and care. This inevitably means that this review covers almost everything that patient advocates are involved with, whether locally, national...Clinical Sarcoma Research 2019 9:11A rare presentation of carcinosarcoma of the bone in a young female; response with gemcitabine and docetaxel
Sarcomatoid carcinoma, or carcinosarcoma, is a neoplasm that contains both sarcomatous and carcinomatous elements. It is an extremely rare cancer most often arising from visceral organs. Here we report the sev...Clinical Sarcoma Research 2019 9:10Radiotherapy resistance in chondrosarcoma cells; a possible correlation with alterations in cell cycle related genes
Conventional chondrosarcomas are malignant cartilage tumors considered radioresistant. Nevertheless, retrospective series show a small but significant survival benefit for patients with locally advanced diseas...Clinical Sarcoma Research 2019 9:9Immunophenotypic expression of UCP1 in hibernoma and other adipose/non adipose soft tissue tumours
Uncoupling protein 1 (UCP1) is a mitochondral protein transporter that uncouples electron transport from ATP production. UCP1 is highly expressed in brown adipose tissue (BAT), including hibernomas, but its ex...Clinical Sarcoma Research 2019 9:8Correction 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:Clinical Sarcoma Research 2019 9:7The original article was published in Clinical Sarcoma Research 2019 9:4Perspectives on treatment side effects in patients with metastatic gastrointestinal stromal tumour: a qualitative study
This study aims to explore how patients with metastatic gastrointestinal stromal tumour (GIST) experience the adverse effects of treatment, as expressed by the individuals themselves.Clinical Sarcoma Research 2019 9:6The challenge of finding new therapeutic avenues in soft tissue sarcomas
Soft 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 ver...Clinical Sarcoma Research 2019 9:5Survival 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 use of imatinib, sunitinib, and regorafenib has transformed the treatment of advanced GIST. Sunitinib and regorafenib improve progression free-survival in the second (2L) and third (3L) line, respectively,...Clinical Sarcoma Research 2019 9:4The Correction to this article has been published in Clinical Sarcoma Research 2019 9:7Genomic aberrations in cell cycle genes predict progression of KIT-mutant gastrointestinal stromal tumors (GISTs)
Activating mutations of the receptor tyrosine kinase KIT are early events in the development of most gastrointestinal stromal tumors (GISTs). Although GISTs generally remain dependent on oncogenic KIT during tumo...Clinical Sarcoma Research 2019 9:3Genome-wide methylation profiling and copy number analysis in atypical fibroxanthomas and pleomorphic dermal sarcomas indicate a similar molecular phenotype
Atypical fibroxanthomas (AFX) and pleomorphic dermal sarcomas (PDS) are lesions of the skin with overlapping histologic features and unspecific molecular traits. PDS behaves aggressive compared to AFX. Thus, a...Clinical Sarcoma Research 2019 9:2Long-term cure of soft tissue sarcoma with pegylated-liposomal doxorubicin after doxorubicin and ifosfamide failure
Doxorubicin is one of the most active drugs available for the treatment of sarcoma. Pegylated-liposomal doxorubicin (PLD) is a formulation of doxorubicin in which the doxorubicin is encapsulated in liposomes c...Clinical Sarcoma Research 2019 9:1Dedifferentiated chondrosarcoma of the pelvis: clinical outcomes and current treatment
Dedifferentiated chondrosarcomas (CS) are a high-grade variant of CS that confers a 5-year survival of around 10–24%. Dedifferentiated CS arising from the pelvis confers an even worse prognosis.Clinical Sarcoma Research 2018 8:23Extraskeletal myxoid chondrosarcoma with massive pulmonary metastases
Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant mesenchymal neoplasm of uncertain differentiation characterized by rearrangements of the NR4A3 gene. EMC often affects adults around the age of 50 ...Clinical Sarcoma Research 2018 8:20A phase II study of temsirolimus and liposomal doxorubicin for patients with recurrent and refractory bone and soft tissue sarcomas
Relapsed and refractory sarcomas continue to have poor survival rates. The cancer stem cell (CSC) theory provides a tractable explanation for the observation that recurrences occur despite dramatic responses t...Clinical Sarcoma Research 2018 8:21Descending thoracic aortic aneurysm revealing metastasis of a soft tissue fibrosarcoma: a case report and review of the literature
Review of the first documented case of aortic wall metastasis from a limb sarcoma.Clinical Sarcoma Research 2018 8:22Preoperative radiotherapy of soft-tissue sarcomas: surgical and radiologic parameters associated with local control and survival
Preoperative radiotherapy is often used to facilitate excision of soft-tissue sarcomas. We aimed define factors that affect local tumour control and patient survival.Clinical Sarcoma Research 2018 8:19Periostin expression in neoplastic and non-neoplastic diseases of bone and joint
Periostin is a matricellular protein that is expressed in bone and joint tissues. To determine the expression of periostin in primary bone tumours and to assess whether it plays a role in tumour progression, w...Clinical Sarcoma Research 2018 8:18Low-grade central fibroblastic osteosarcoma may be differentiated from its mimicker desmoplastic fibroma by genetic analysis
We studied two cases of rare fibrous bone tumors, namely desmoplastic fibroma (DF) and low-grade central osteosarcoma (LGCOS) resembling desmoplastic fibroma (DF-like LGCOS). As the clinical presentation, imag...Clinical Sarcoma Research 2018 8:16Report 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 repres...Clinical Sarcoma Research 2018 8:17PDGF/PDGFR effects in osteosarcoma and the “add-on” strategy
New 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 developme...Clinical Sarcoma Research 2018 8:15Tenosynovial giant cell tumor: case report of a patient effectively treated with pexidartinib (PLX3397) and review of the literature
Tenosynovial giant cell tumors (TGCTs) or giant cell tumors of tendon sheath are neoplasms that arise in the synovium. They can be categorized as nodular (localized) or diffuse type (D-TGCT). Historically, sur...Clinical Sarcoma Research 2018 8:14Regional 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
Induction 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 co...Clinical Sarcoma Research 2018 8:12Reversible rituximab-induced rectal Kaposi’s sarcoma misdiagnosed as ulcerative colitis in a patient with HIV-negative follicular lymphoma
Kaposi’s sarcoma is a low-grade mesenchymal angioproliferative tumor, most commonly observed in immunocompromised individuals, such as HIV-infected patients. Iatrogenic Kaposi’s sarcoma occurs in patients unde...Clinical Sarcoma Research 2018 8:11Non-canonical WNT6/WNT10A signal factor expression in EBV+ post-transplant smooth muscle tumors
Post-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)+ smoo...Clinical Sarcoma Research 2018 8:10Aggressive fibromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response
One of the commonly used systemic agents for the treatment of aggressive fibromatosis is the anti-oestrogen drug tamoxifen. However, data on efficacy and optimum methods of response assessment are limited, con...Clinical Sarcoma Research 2018 8:13Treatment patterns and survival among older adults in the United States with advanced soft-tissue sarcomas
To describe patient and tumor characteristics, treatments, and survival among older adults in the United States with advanced soft-tissue sarcoma (STS), across and by categories of specifically defined histolo...Clinical Sarcoma Research 2018 8:8Smoking is predictive of poorer distant metastasis-free and progression free-survival in soft tissue sarcoma patients treated with pre-operative radiotherapy or chemoradiotherapy
Soft tissue sarcomas (STS) are often treated with pre-operative radiation (RT), with or without chemotherapy, followed by wide local excision. Prognosis for these patients involves an interplay of tumor and pa...Clinical Sarcoma Research 2018 8:7Retrospective audit of 957 consecutive 18F-FDG PET–CT scans compared to CT and MRI in 493 patients with different histological subtypes of bone and soft tissue sarcoma
The use of 18F-FDG PET–CT (PET–CT) is widespread in many cancer types compared to sarcoma. We report a large retrospective audit of PET–CT in bone and soft tissue sarcoma with varied grade in a single multi-disci...Clinical Sarcoma Research 2018 8:9Unicentric epithelioid hemangioendothelioma of the calcaneus: a case report and review of literature
This review of the literature combined with a clinical case will allow the illustration of a favorable outcome of this variable low grade malignancy, display a role for limb salvage surgery with intralesional tre...Clinical Sarcoma Research 2018 8:5Survival is influenced by approaches to local treatment of Ewing sarcoma within an international randomised controlled trial: analysis of EICESS-92
Two national clinical trial groups, United Kingdom Children’s Cancer and Leukaemia Group (CCLG) and the German Paediatric Oncology and Haematology Group (GPOH) together undertook a randomised trial, EICESS-92,...Clinical Sarcoma Research 2018 8:6The use of whole exome sequencing and murine patient derived xenografts as a method of chemosensitivity testing in sarcoma
Soft tissue and bone sarcoma represent a broad spectrum of different pathology and genetic variance. Current chemotherapy regimens are derived from randomised trials and represent empirical treatment. Chemosen...Clinical Sarcoma Research 2018 8:4Different quality of treatment in retroperitoneal sarcomas (RPS) according to hospital-case volume and surgeon-case volume: a retrospective regional analysis in Italy
Retroperitoneal sarcomas (RPS) should be surgically managed in specialized sarcoma centers. However, it is not clearly demonstrated if clinical outcome is more influenced by Center Case Volume (CCV) or by Surg...Clinical Sarcoma Research 2018 8:3Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour
Liposarcoma is an extremely rare primary bone sarcoma.Clinical Sarcoma Research 2018 8:2Intraosseous papillary intralymphatic angioendothelioma (PILA): one new case and review of the literature
Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and ...Clinical Sarcoma Research 2018 8:1Response to isolated limb perfusion and chemotherapy with epirubicin plus ifosfamide in a metastatic malignant ossifying fibromyxoid tumor
Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain lineage and intermediate biological potential. It is more common in middle-aged men, usually arising from the deep tissues of the ...Clinical Sarcoma Research 2017 7:20Giant cell tumour of the distal radius/ulna: response to pre-operative treatment with short-term denosumab
Treatment of giant cell tumour of bone (GCTB) of the distal radius/ulna poses a surgical challenge, as complex reconstructive surgery may be required. This study evaluates the clinical, radiological and pathol...Clinical Sarcoma Research 2017 7:19A retrospective cohort study of treatment patterns among patients with metastatic soft tissue sarcoma in the US
Since treatment patterns in metastatic soft tissue sarcoma (mSTS) have not been studied subsequent to US approval of pazopanib in 2012, this study sought to examine mSTS treatment patterns by line of therapy, ...Clinical Sarcoma Research 2017 7:18Utility of VS38c in the diagnostic and prognostic assessment of osteosarcoma and other bone tumours/tumour-like lesions
VS38c is a monoclonal antibody that recognises a rough endoplasmic reticulum (rER) intracellular antigen termed cytoskeleton-linking membrane protein 63. rER is typically found in viable tumour cells and is ab...Clinical Sarcoma Research 2017 7:17Activity of anthracycline- and ifosfamide-based chemotherapy in a series of patients affected by advanced myxofibrosarcoma
We report on the activity of anthracycline-based and high-dose prolonged-infusion ifosfamide chemotherapy in a retrospective series of patients affected by advanced myxofibrosarcoma treated at Istituto Naziona...Clinical Sarcoma Research 2017 7:16Radiation induced angiosarcoma of the breast: outcomes from a retrospective case series
Radiation induced angiosarcoma (RIAS) of the breast is a rare and aggressive complication of radiotherapy. Due to the rarity of this disease, much of the evidence for its management is based on case reports or...Clinical Sarcoma Research 2017 7:15Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy
Clear cell sarcoma (CCS) is a rare, aggressive soft tissue sarcoma thought to derive from neural crest and characterized by a 12;22 translocation. The resulting fusion protein directly activates expression of ...Clinical Sarcoma Research 2017 7:14Primary 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...Clinical Sarcoma Research 2017 7:13Overexpressed 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 ...Clinical Sarcoma Research 2017 7:11Disseminated 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...Clinical Sarcoma Research 2017 7:12Antiangiogenic 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...Clinical Sarcoma Research 2017 7:10Histone 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...Clinical Sarcoma Research 2017 7:9IDH1 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...Clinical Sarcoma Research 2017 7:8
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