Ewing's sarcoma and primary osseous lymphoma : spectrum of imaging appearances

Ewing's sarcoma (ES) is a rare, highly malignant anaplastic stem cell tumor. Histologically, the tumor consists of uniform densely packed small monomorphic cells with round nuclei. The typical appearance at hematoxylin and eosin (H&E) staining is small blue round cells without any matrix formation. On conventional radiography, ES typically presents as a permeative lesion in the diaphysis of a long bone in a child. A large soft tissue component is another characteristic feature, best depicted by magnetic resonance imaging. Primary osseous lymphomas are most commonly highly malignant B-cell lymphomas. At H&E histologic staining, the tumor stroma consists of diffuse round-cell infiltrates that resembles the appearance of ES. Although there is no typical imaging appearance of an osseous lymphoma, it should be considered in an adult presenting with a Lodwick grade II or III lesion in the metaphysis or diaphysis of a large long bone, the pelvis, or the vertebral column. Histologic confirmation is mandatory

Soft Tissue Tumors in Adults: ESSR-Approved Guidelines for Diagnostic Imaging

Soft tissue sarcomas are rare, but early, accurate diagnosis with subsequent appropriate treatment is crucial for the clinical outcome. The ESSR guidelines are intended to help radiologists in their decision-making and support discussion among clinicians who deal with patients with suspected or proven soft tissue tumors. Potentially malignant lesions recognized by ultrasound should be referred for magnetic resonance imaging (MRI), which also serves as a preoperative local staging modality, with specific technical requirements and mandatory radiological report elements. Radiography may add information about matrix calcification and osseous involvement. Indeterminate lesions, or lesions in which therapy is dependent on histology results, should be biopsied. For biopsy, we strongly recommend referral to a specialist sarcoma center, where an interdisciplinary tumor group, with a specialized pathologist, radiologist, and the surgeon are involved. In sarcoma, a CT scan of the chest is mandatory. Additional staging modalities are entity-specific. There are no evidence-based recommendations for routine follow-up in surgically treated sarcomas. However, we would recommend regular follow-up with intervals dependent on tumor grade, for 10 years after the initial diagnosis

Soft tissue tumor imaging in adults : European Society of Musculoskeletal Radiology-Guidelines 2023\u2014overview, and primary local imaging: how and where?

Abstract: ObjectivesEarly, accurate diagnosis is crucial for the prognosis of patients with soft tissue sarcomas. To this end, standardization of imaging algorithms, technical requirements, and reporting is therefore a prerequisite. Since the first European Society of Musculoskeletal Radiology (ESSR) consensus in 2015, technical achievements, further insights into specific entities, and the revised WHO-classification (2020) and AJCC staging system (2017) made an update necessary. The guidelines are intended to support radiologists in their decision-making and contribute to interdisciplinary tumor board discussions.Materials and methodsA validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries. Statements were scored online by level of agreement (0 to 10) during two iterative rounds. Either "group consensus," "group agreement," or "lack of agreement" was achieved.ResultsEight sections were defined that finally contained 145 statements with comments. Overall, group consensus was reached in 95.9%, and group agreement in 4.1%. This communication contains the first part consisting of the imaging algorithm for suspected soft tissue tumors, methods for local imaging, and the role of tumor centers.ConclusionUltrasound represents the initial triage imaging modality for accessible and small tumors. MRI is the modality of choice for the characterization and local staging of most soft tissue tumors. CT is indicated in special situations. In suspicious or likely malignant tumors, a specialist tumor center should be contacted for referral or teleradiologic second opinion. This should be done before performing a biopsy, without exception.Clinical relevanceThe updated ESSR soft tissue tumor imaging guidelines aim to provide best practice expert consensus for standardized imaging, to support radiologists in their decision-making, and to improve examination comparability both in individual patients and in future studies on individualized strategies.Key Points center dot Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors.center dot MRI is the modality of choice for the characterization and local staging of soft tissue tumors in most cases; CT is indicated in special situations. Suspicious or likely malignant tumors should undergo biopsy.center dot In patients with large, indeterminate or suspicious tumors, a tumor reference center should be contacted for referral or teleradiologic second opinion; this must be done before a biopsy.Key Points center dot Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors.center dot MRI is the modality of choice for the characterization and local staging of soft tissue tumors in most cases; CT is indicated in special situations. Suspicious or likely malignant tumors should undergo biopsy.center dot In patients with large, indeterminate or suspicious tumors, a tumor reference center should be contacted for referral or teleradiologic second opinion; this must be done before a biopsy.Key Points center dot Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors.center dot MRI is the modality of choice for the characterization and local staging of soft tissue tumors in most cases; CT is indicated in special situations. Suspicious or likely malignant tumors should undergo biopsy. center dot In patients with large, indeterminate or suspicious tumors, a tumor reference center should be contacted for referral or teleradiologic second opinion; this must be done before a biopsy

The management of acute venous thromboembolism in clinical practice - study rationale and protocol of the European PREFER in VTE Registry

Background: Venous thromboembolism (VTE) is a major health problem, with over one million events every year in Europe. However, there is a paucity of data on the current management in real life, including factors influencing treatment pathways, patient satisfaction, quality of life (QoL), and utilization of health care resources and the corresponding costs. The PREFER in VTE registry has been designed to address this and to understand medical care and needs as well as potential gaps for improvement. Methods/design: The PREFER in VTE registry was a prospective, observational, multicenter study conducted in seven European countries including Austria, France Germany, Italy, Spain, Switzerland, and the UK to assess the characteristics and the management of patients with VTE, the use of health care resources, and to provide data to estimate the costs for 12 months treatment following a first-time and/or recurrent VTE diagnosed in hospitals or specialized or primary care centers. In addition, existing anticoagulant treatment patterns, patient pathways, clinical outcomes, treatment satisfaction, and health related QoL were documented. The centers were chosen to reflect the care environment in which patients with VTE are managed in each of the participating countries. Patients were eligible to be enrolled into the registry if they were at least 18 years old, had a symptomatic, objectively confirmed first time or recurrent acute VTE defined as either distal or proximal deep vein thrombosis, pulmonary embolism or both. After the baseline visit at the time of the acute VTE event, further follow-up documentations occurred at 1, 3, 6 and 12 months. Follow-up data was collected by either routinely scheduled visits or by telephone calls. Results: Overall, 381 centers participated, which enrolled 3,545 patients during an observational period of 1 year. Conclusion: The PREFER in VTE registry will provide valuable insights into the characteristics of patients with VTE and their acute and mid-term management, as well as into drug utilization and the use of health care resources in acute first-time and/or recurrent VTE across Europe in clinical practice. Trial registration: Registered in DRKS register, ID number: DRKS0000479

Reduced Cancer Incidence in Huntington's Disease: Analysis in the Registry Study

Background: People with Huntington's disease (HD) have been observed to have lower rates of cancers. Objective: To investigate the relationship between age of onset of HD, CAG repeat length, and cancer diagnosis. Methods: Data were obtained from the European Huntington's disease network REGISTRY study for 6540 subjects. Population cancer incidence was ascertained from the GLOBOCAN database to obtain standardised incidence ratios of cancers in the REGISTRY subjects. Results: 173/6528 HD REGISTRY subjects had had a cancer diagnosis. The age-standardised incidence rate of all cancers in the REGISTRY HD population was 0.26 (CI 0.22-0.30). Individual cancers showed a lower age-standardised incidence rate compared with the control population with prostate and colorectal cancers showing the lowest rates. There was no effect of CAG length on the likelihood of cancer, but a cancer diagnosis within the last year was associated with a greatly increased rate of HD onset (Hazard Ratio 18.94, p < 0.001). Conclusions: Cancer is less common than expected in the HD population, confirming previous reports. However, this does not appear to be related to CAG length in HTT. A recent diagnosis of cancer increases the risk of HD onset at any age, likely due to increased investigation following a cancer diagnosis