Striving towards Normality in Daily Life: A Qualitative Study of Patients Living with Metastatic Gastrointestinal Stromal Tumour in Long-Term Clinical RemissionRead the full article
Sarcoma covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.
Sarcoma maintains an Editorial Board of practicing researchers from around the world, to ensure manuscripts are handled by editors who are experts in the field of study.
Latest ArticlesMore articles
Diagnostic Differences in Expert Second-Opinion Consultation Cases at a Tertiary Sarcoma Center
Soft tissue tumors are diagnostically challenging, and it is recommended that these are reported or reviewed by specialist soft tissue pathologists. We present our experience with second-opinion (consultation) cases in a specialist tertiary sarcoma center. The aim of this study was to determine areas of diagnostic difficulty in soft tissue pathology. We assessed 581 second-opinion cases which were reviewed by two experienced pathologists in a period of one year. There was 62% concordance between the original and the second-opinion diagnosis, with diagnostic discrepancy in 38%. The largest group of soft tissue neoplasms received for second opinion was fibroblastic/myofibroblastic tumors, and most major diagnostic problems were encountered in adipocytic and so-called “fibrohistiocytic” tumors. Major diagnostic errors impacting management were found in 148 cases (25%). Morphologic assessment of tumors, judicious use of molecular techniques, newer immunostains and their interpretation, along with importance of knowledge of rarer entities were found to be most useful in avoiding errors.
Time Trends and Prognostic Factors for Overall Survival in Myxoid Liposarcomas: A Population-Based Study
Background. The purpose of this study was to evaluate the overall survival (OS) and associated characteristics for patients with Myxoid Liposarcoma (MLS) over time in The Netherlands. Methods. A population-based study was performed of patients with primary localized (n = 851) and metastatic (n = 50) MLS diagnosed in The Netherlands between 1989 and 2016, based on data from the National Cancer Registry. Results. The median age of the MLS patients was 49 years, and approximately two-thirds was located in the lower limb. An association was revealed between age and the risk of having a Round Cell (RC) tumor. OS rates for primary localized MLS were 93%, 83%, 78%, and 66% after 1, 3, 5, and 10 years, respectively. The median OS for patients with metastatic disease at diagnosis was 10 months. Increasing age (Hazard Ratio (HR) 1.05, ), a tumor size >5 cm (HR 2.18; ), and tumor location (trunk HR 1.29; , upper limb HR 0.83; , and “other” locations HR 2.73; , as compared to lower limb) were independent prognostic factors for OS. The percentage of patients treated with radiotherapy (RT) increased over time, and preoperative RT gradually replaced postoperative RT. In contrast to patients with localized disease, significant improvement of OS was observed in patients with metastatic disease over time. Conclusions. In this large nationwide cohort, tumor size and tumor location were independent prognostic factors for OS. Furthermore, a higher probability of an RC tumor with increasing age was suggested. An increased use of RT over the years did not translate into improved OS for localized MLS.
Pharmacologic Inhibition of Ezrin-Radixin-Moesin Phosphorylation is a Novel Therapeutic Strategy in Rhabdomyosarcoma
Intermediate and high-risk rhabdomyosarcoma (RMS) patients have poor prognosis with available treatment options, highlighting a clear unmet need for identification of novel therapeutic strategies. Ezrin-radixin-moesin (ERM) family members are membrane-cytoskeleton linker proteins with well-defined roles in tumor metastasis, growth, and survival. ERM protein activity is regulated by dynamic changes in the phosphorylation at a conserved threonine residue in their C-terminal actin-binding domain. Interestingly, ERM family member, ezrin, has elevated expression in the RMS tissue. Despite this, the translational scope of targeting ERM family proteins in these tumors through pharmacological inhibition has never been considered. This study investigates the inhibition of ERM phosphorylation using a small molecule pharmacophore NSC668394 as a potential strategy against RMS. Upon in vitro treatment with NSC668394, RMS cells exhibit a dose-dependent decrease in cell viability and proliferation, with induction of caspase-3 cleavage and apoptosis. siRNA-mediated knockdown of individual ERM protein expression revealed that each regulates RMS survival to a different degree. In vivo administration of NSC668394 in RMS xenografts causes significant decrease in tumor growth, with no adverse effect on body weight. Collectively, this study highlights the importance of the active conformation of ERM proteins in RMS progression and survival and supports pharmacologic inhibition of these proteins as a novel therapeutic approach.
Epidemiology of Soft Tissue Sarcoma and Bone Sarcoma inItaly: Analysis of Data from 15 Population-Based Cancer Registries
Sarcomas are a heterogeneous group of rare cancers of mesenchymal origin. In this study, we provide updated, world age-standardised incidence rate (ASR) and European age-standardised incidence rate for malignant soft tissue sarcoma (ICD-O-3 topographic code C47–C49) and bone sarcoma (C40, C41) in Italy, by area (north, centre, and south) and by cancer registry. We also assess morphology in relation to site and area and assess metastases at diagnosis. We analysed 1,112 cases, with incidence 2009–2012, provided by 15 cancer registries (CRs) affiliated to the Association of Italian Cancer Registries (AIRTUM). Overall, ASR was 1.7/100,000/year for soft tissue sarcoma and 0.7 for bone sarcoma. Central Italy had the highest (2.4) ASR and south Italy had the lowest (1.6) ASR for soft tissue sarcoma. Central Italy had the highest (1.1) ASR and north Italy had the lowest (0.7) ASR for bone sarcoma. By CR, ASRs ranged from 1.1 to 2.6 for soft tissue sarcoma and from 0 to 1.4 for bone sarcoma. The most frequent soft tissue sarcomas were sarcoma not otherwise specified (NOS) (29.4%) and liposarcoma (22.2%); the most common bone sarcoma was chondrosarcoma (37.6%). Soft tissue sarcomas occurred most frequently (35.6%) in lower limb connective tissue; bone sarcomas arose mainly (68.8%) in long bones. The frequencies of morphologies arising at different sites varied considerably by Italian area; for example, 20% of hemangiosarcomas occurred in the head and neck in south Italy with 17% at this site in the centre and 6% in the north. For soft tissue sarcoma, the highest ASRs of 2.6 and 2.4 contrast with the lowest ASRs 1.1 and 1.3, suggesting high-risk hot spots that deserve further investigation. The marked variations in morphology distribution with site and geography suggest geographic variation in risk factors that may also repay further investigation particularly since sarcoma etiology is poorly understood.
Angiosarcoma of the Scalp and Face: A Dosimetric Comparison of HDR Surface Applicator Brachytherapy and VMAT
Purpose. Angiosarcoma of the face and scalp is a rare disease with high rates of recurrence. The optimal treatment approach is not well defined. This study presents a dosimetric comparison of high-dose-rate surface applicator (HDR-SA) brachytherapy to volumetric-modulated arc therapy (VMAT). Methods. Between 2011 and 2018, 12 patients with primary or recurrent angiosarcoma of the face or scalp were treated with HDR-SA brachytherapy using CT-based planning at our institution. For comparison, deliverable VMAT plans for each patient were generated, and dose distribution was compared to the delivered HDR-SA brachytherapy plans. Results. Both VMAT and HDR-SA brachytherapy plans delivered good coverage of the clinical target. However, the dose distribution of VMAT was significantly different from HDR-SA brachytherapy across a variety of parameters. Mean doses to the lacrimal gland, orbit, lens, and cochlea were significantly higher with HDR-SA brachytherapy vs. VMAT. Brain Dmax, V80%, and V50% were also significantly higher with HDR-SA brachytherapy. Conclusions. There may be dosimetric advantages to VMAT over HDR-SA brachytherapy for many patients. However, individual tumor location, patient anatomy, and treatment reproducibility may result in HDR-SA brachytherapy being the preferred technique in a subset of patients. Ultimately, a personalized approach is likely to be the optimal treatment plan.
Predicting Pathologic Bone Lesions Using Scout Computed Tomography (CT) Imaging
The purpose of this study is to evaluate the benefit of reviewing scout CT images, obtained for routine oncologic surveillance, for the early identification of pathologic bony lesions. A retrospective review was conducted on patients who previously underwent surgical treatment by two orthopedic oncology surgeons at a tertiary care institution from 2009–2019 for pathologic lesions or fractures of the humerus or femur. Radiographic records were reviewed to identify patients in this cohort who had available scout views from CT imaging prior to official diagnosis of the bony lesion or fracture. CT scout images were assessed by two independent reviewers to identify any pathologic lesions, and radiographic reports were reviewed to identify if the lesions were noted by radiology at the time of the initial scan interpretation. One hundred and forty-four patients were identified, and thirty-nine had an available scout CT image prior to official diagnosis of the lesion. Twenty-five patients (64.1%) had lesions identified by authors on scout CT versus only 9 (23.1%) who had lesions that were documented in the initial CT radiologic report. There was a total of 29 lesions identified by the study authors on scout CT, and 19 (65.5%) were not reported in the initial radiographic interpretation with an average interval between observation by authors and official diagnosis of 202 days. Of the impending fractures, three patients (16.7%) went on to complete fracture prior to referral to orthopedics with an average interval between these missed lesions on scout CT and their presentation with fracture of 68 days. This study advocates for the careful review of all scout CT imaging as an essential part of the work up for metastatic disease and encourages all practitioners to utilize this screening tool for the identification of pathologic bony lesions which may help expedite early treatment to reduce patient morbidity.