Soft tissue tumor imaging in adults: whole-body staging in sarcoma, non-malignant entities requiring special algorithms, pitfalls and special imaging aspects. Guidelines 2024 from the European Society of Musculoskeletal Radiology (ESSR).

OBJECTIVES: The revised European Society of Musculoskeletal Radiology (ESSR) consensus guidelines on soft tissue tumor imaging represent an update of 2015 after technical advancements, further insights into specific entities, and revised World Health Organization (2020) and AJCC (2017) classifications. This second of three papers covers algorithms once histology is confirmed: (1) standardized whole-body staging, (2) special algorithms for non-malignant entities, and (3) multiplicity, genetic tumor syndromes, and pitfalls. MATERIALS AND METHODS: A 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 that had undergone interdisciplinary revision were scored online by the level of agreement (0 to 10) during two iterative rounds, that could result in 'group consensus', 'group agreement', or 'lack of agreement'. RESULTS: The three sections contain 24 statements with comments. Group consensus was reached in 95.8% and group agreement in 4.2%. For whole-body staging, pulmonary MDCT should be performed in all high-grade sarcomas. Whole-body MRI is preferred for staging bone metastasis, with [18F]FDG-PET/CT as an alternative modality in PET-avid tumors. Patients with alveolar soft part sarcoma, clear cell sarcoma, and angiosarcoma should be screened for brain metastases. Special algorithms are recommended for entities such as rhabdomyosarcoma, extraskeletal Ewing sarcoma, myxoid liposarcoma, and neurofibromatosis type 1 associated malignant peripheral nerve sheath tumors. Satisfaction of search should be avoided in potential multiplicity. CONCLUSION: Standardized whole-body staging includes pulmonary MDCT in all high-grade sarcomas; entity-dependent modifications and specific algorithms are recommended for sarcomas and non-malignant soft tissue tumors. CLINICAL RELEVANCE STATEMENT: These updated ESSR soft tissue tumor imaging guidelines aim to provide support in decision-making, helping to avoid common pitfalls, by providing general and entity-specific algorithms, techniques, and reporting recommendations for whole-body staging in sarcoma and non-malignant soft tissue tumors. KEY POINTS: An early, accurate, diagnosis is crucial for the prognosis of patients with soft tissue tumors. These updated guidelines provide best practice expert consensus for standardized imaging algorithms, techniques, and reporting. Standardization can improve the comparability examinations and provide databases for large data analysis.

Standardized reporting of spine and sacroiliac joints in axial spondyloarthritis MRI: from the ESSR-Arthritis Subcommittee.

OBJECTIVES: Apply a modified Delphi-based approach and produce a practical, radiology-specific set of definitions for interpretation and standardization of the multiple MRI findings in axial spondyloarthritis (ax-SpA), specifically to aid the general radiologist with a musculoskeletal interest, working with gold standard basic MRI protocols. MATERIALS AND METHODS: We report the results of a modified Delphi-based consensus of 35 experts from 13 countries in the Arthritis Subcommittee of the European Society of Musculoskeletal Radiology (ESSR). Seventeen definitions were created (i.e., nine for the spine and eight for the sacroiliac joint) and two Delphi rounds were conducted on an electronic database, collated and revised by the project leader with agreement. Group leads were appointed for each definition following the first round. Final definitions included only those that reached a consensus > 80%; if > 50% agreed on exclusion consensus, definitions were excluded. Final results have been shared during the Arthritis meeting at the Annual ESSR Congress. RESULTS: Fourteen definitions, eight for the spine and six for the sacroiliac joint were agreed for standardized reporting. Andersson's, anterior corner sclerotic and costovertebral joint inflammatory lesions of the spine, with active and non-active erosions, and fat metaplasia of the sacroiliac joint reaching the highest consensus (≥ 95%). More than 50% of the experts agreed to exclude joint space inflammation in the sacroiliac joint and tissue backfill. Syndesmophytes reached 76% agreement. CONCLUSIONS: Agreed definitions by expert radiologists using a modified Delphi process, should allow standardized actionable radiology reports and clarity in reporting terminology of ax-SpA. CLINICAL RELEVANCE STATEMENT: The proposed definitions will support reporting from musculoskeletal and general radiologists working with gold-standard basic MRI, improve confidence in lesion assessment, and standardize terminology to provide actionable reports on MRI in patients with ax-SpA. KEY POINTS: Experts applied a modified Delphi method to optimize the definitions of MRI findings of ax-SpA. After two Delphi rounds and one in-person meeting, fourteen definitions reached the agreement threshold. These consensus-based definitions will aid in actionable reporting specifically for the general radiologist with a musculoskeletal interest.

Update on Rheumatic Diseases in Clinical Practice: Recent Concepts and Developments.

Diagnostic imaging is essential in the diagnostic process of rheumatic diseases. Given the heterogeneity of this group of diseases and the tremendous impact of novel therapeutic options, guidelines and recommendations regarding the optimal choice of the most appropriate technique/s are continuously revised and radiologists should always be up-to-date. Last, because of the continuous technological innovations, we will assist to the progressive application of advanced techniques and tools in rheumatology.

How to Approach the Imaging Differential Diagnosis of Rheumatic Diseases by Anatomic Location.

The purpose of this review is to present an approach to differential diagnosis based on the particular features of involvement of the most common rheumatological conditions focused on anatomic location (by joint). The most common radiological signs and how they are demonstrated in different modalities, as well as the typical patterns of involvement are analyzed, with the aim to facilitate the differential diagnosis. Early and adequate adjustment of treatment has an effect on outcome, and on this basis, early diagnosis and characterization are paramount to appropiately manage patients.

Interventional Radiology for Rheumatic Diseases.

Imaging plays a key role in the management of rheumatological pathologies, also as guidance for diagnostic and therapeutic interventional procedures, as it can provide better accuracy and safety compared to palpation-guided interventions. Inflammatory and degenerative conditions nonresponsive to systemic treatment may benefit from intra-articular and periarticular administration of drugs, with therapeutic and symptomatic actions or providing a bridge for surgery. Desired effects include reduction of inflammation and pain and improvement of physical function of patients. Training and knowledge of indications, appropriate procedures, contraindications, and side effects are necessary to obtain maximum accuracy and safety in performing interventional procedures.

DXA beyond bone mineral density and the REMS technique: new insights for current radiologists practice.

Osteoporosis is the most prevalent skeletal disorder, a condition that is associated with significant social and healthcare burden. In the elderly, osteoporosis is commonly associated with sarcopenia, further increasing the risk of fracture. Several imaging techniques are available for a non-invasive evaluation of osteoporosis and sarcopenia. This review focuses on dual-energy X-ray absorptiometry (DXA), as this technique offers the possibility to evaluate bone mineral density and body composition parameters with good precision and accuracy. DXA is also able to evaluate the amount of aortic calcification for cardiovascular risk estimation. Additionally, new DXA-based parameters have been developed in recent years to further refine fracture risk estimation, such as the Trabecular Bone Score and the Bone Strain Index. Finally, we describe the recent advances of a newly developed ultrasound-based technology known as Radiofrequency Echographic Multi-Spectrometry, which represent the latest non-ionizing approach for osteoporosis evaluation at central sites.

Radiological diagnosis of prevalent osteoporotic vertebral fracture on radiographs: an interim consensus from a group of experts of the ESSR osteoporosis and metabolism subcommittee.

When a low-energy trauma induces an acute vertebral fracture (VF) with clinical symptoms, a definitive diagnosis of osteoporotic vertebral fracture (OVF) can be made. Beyond that, a "gold" radiographic standard to distinguish osteoporotic from non-osteoporotic VFs does not exist. Fracture-shaped vertebral deformity (FSVD) is defined as a deformity radiographically indistinguishable from vertebral fracture according to the best of the reading radiologist's knowledge. FSVD is not uncommon among young populations with normal bone strength. FSVD among an older population is called osteoporotic-like vertebral fracture (OLVF) when the FSVD is likely to be associated with compromised bone strength. In more severe grade deformities or when a vertebra is collapsed, OVF diagnosis can be made with a relatively high degree of certainty by experienced readers. In "milder" cases, OVF is often diagnosed based on a high probability rather than an absolute diagnosis. After excluding known mimickers, singular vertebral wedging in older women is statistically most likely an OLVF. For older women, three non-adjacent minimal grade OLVF (< 20% height loss), one minimal grade OLVF and one mild OLVF (20-25% height loss), or one OLVF with ≥ 25% height loss, meet the diagnosis of osteoporosis. For older men, a single OLVF with < 40% height loss may be insufficient to suggest the subject is osteoporotic. Common OLVF differential diagnoses include X-ray projection artifacts and scoliosis, acquired and developmental short vertebrae, osteoarthritic wedging, oncological deformities, deformity due to high-energy trauma VF, lateral hyperosteogeny of a vertebral body, Cupid's bow, and expansive endplate, among others.

Sarcopenic Obesity in Cervical Carcinoma: A Strong and Independent Prognostic Factor beyond the Conventional Predictors (ESTHER Study-AFRAID Project).

Locally advanced cervical cancer represents a significant treatment challenge. Body composition parameters such as body mass index, sarcopenia, and sarcopenic obesity, defined by sarcopenia and BMI ≥ 30 kg/m2, have been identified as potential prognostic factors, yet their overall impact remains underexplored. This study assessed the relationship between these anthropometric parameters alongside clinical prognostic factors on the prognosis of 173 cervical cancer patients. Survival outcomes in terms of local control (LC), distant metastasis-free survival (DMFS), disease-free survival (DFS), and overall survival (OS) were analyzed using Kaplan regression methods-Meier and Cox. Older age, lower hemoglobin levels, higher FIGO (International Federation of Gynecology and Obstetrics) stages, and lower total radiation doses were significantly associated with worse outcomes. Univariate analysis showed a significant correlation between BMI and the outcomes examined, revealing that normal-weight patients show higher survival rates, which was not confirmed by the multivariate analysis. Sarcopenia was not correlated with any of the outcomes considered, while sarcopenic obesity was identified as an independent negative predictor of DFS (HR: 5.289, 95% CI: 1.298-21.546, p = 0.020) and OS (HR: 2.645, 95% CI: 1.275-5.488, p = 0.009). This study highlights the potential of sarcopenic obesity as an independent predictor of clinical outcomes. These results support their inclusion in prognostic assessments and treatment planning for patients with advanced cervical cancer.

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

OBJECTIVES: Early, 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 METHODS: A 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. RESULTS: Eight 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. CONCLUSION: Ultrasound 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 RELEVANCE: The 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: • Ultrasound remains the best initial triage imaging modality for accessible and small suspected soft tissue tumors. • 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. • 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.

Aneurysmal bone cyst-like changes developed in melorheostosis with epiphyseal osteopoikilosis.

Aneurysmal bone cyst (ABC) is a rare and usually painful condition, representing about 1% of all bone tumors. A geographical lytic, expansile, and septated radiological pattern, with fluid-fluid levels on MRI, is classically displayed. ABC can be a primary bone lesion (70% of patients) or can arise in an underlying condition and is subsequently named "ABC-like changes" (30%). ABC-like changes are more frequently encountered in skeletal segments affected by chondroblastoma, fibrous dysplasia, giant cell tumor, osteoblastoma, non-ossifying fibroma, and osteosarcoma. In this article, we describe the first case of ABC-like changes developed in association with an ultra-rare sclerosing bone disease: melorheostosis. Melorheostosis is characterized by recognizable patterns on radiological studies with a pathological increased bone density and a cortical thickening within the periosteal or endosteal space, usually with a "dripping candle wax" appearance. More rarely, other different radiological patterns can be observed, such as "osteopatia striata-like," "osteoma-like," "myositis ossificans-like," and mixed patterns. Pain and limb hypotrophy are the most common clinical manifestations. We report the case of a Caucasian male with a clinic-radiological diagnosis of melorheostosis (with epiphyseal osteopoikilosis) since the age of twelve. At the age of nineteen, he suffered from increased pain in the proximal right thigh, and the radiological control revealed an expansive septated lesion at the right proximal femoral bone. The diagnosis of ABC-like changes developed in melorheostosis was obtained after CT-guided bone biopsy and confirmed by open-incisional biopsy.

NTRK rearranged sarcoma of the bone. Role for larotrectinib in the neoadjuvant setting of an ultra-rare tumor: a case report.

BACKGROUND: Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearrangements. This evidence recently led to the agnostic approval of these drugs, and evidence (confirmation) of their activity in a broader disease setting is emerging. Here, we report the case of a patient affected by EML4-NTRK3 rearranged undifferentiated spindle cell bone sarcoma treated with larotrectinib, and we argue (discuss about) the incidence and clinical presentation of NTRK gene-fusion positive bone sarcomas, the potential use of upfront treatment with NTRK inhibitors in neoadjuvant setting, and the role of a multidisciplinary tumor board. Despite the rarity of these rearrangements in patients with primitive bone sarcomas, the therapy with NTRK inhibitors represents a highly effective strategy to be pursued in selected cases even in neoadjuvant settings. The management of these very rare cancers should always be discussed in a multidisciplinary board of reference centers.

Current Role of Conventional Radiography of Sacroiliac Joints in Adults and Juveniles with Suspected Axial Spondyloarthritis: Opinion from the ESSR Arthritis and Pediatric Subcommittees.

This opinion article by the European Society of Musculoskeletal Radiology Arthritis and Pediatric Subcommittees discusses the current use of conventional radiography (CR) of the sacroiliac joints in adults and juveniles with suspected axial spondyloarthritis (axSpA). The strengths and limitations of CR compared with magnetic resonance imaging (MRI) and computed tomography (CT) are presented.Based on the current literature and expert opinions, the subcommittees recognize the superior sensitivity of MRI to detect early sacroiliitis. In adults, supplementary pelvic radiography, low-dose CT, or synthetic CT may be needed to evaluate differential diagnoses. CR remains the method of choice to detect structural changes in patients with suspected late-stage axSpA or established disease and in patients with suspected concomitant hip or pubic symphysis involvement. In children, MRI is the imaging modality of choice because it can detect active as well as structural changes and is radiation free.

History Page: Leaders in MSK Radiology Augusto Pellegrini, 1877-1958.

This history page in the series "Leaders in MSK Radiology" is dedicated to the memory and achievements of the Italian surgeon Augusto Pellegrini, whose name is partially associated with the medical eponym Pellegrini-Stieda disease.

High-resolution peripheral quantitative computed tomography: research or clinical practice?

High-resolution peripheral quantitative CT (HR-pQCT) is a low-dose three-dimensional imaging technique, originally developed for in vivo assessment of bone microarchitecture at the distal radius and tibia in osteoporosis. HR-pQCT has the ability to discriminate trabecular and cortical bone compartments, providing densitometric and structural parameters. At present, HR-pQCT is mostly used in research settings, despite evidence showing that it may be a valuable tool in osteoporosis and other diseases. This review summarizes the main applications of HR-pQCT and addresses the limitations that currently prevent its integration into routine clinical practice. In particular, the focus is on the use of HR-pQCT in primary and secondary osteoporosis, chronic kidney disease (CKD), endocrine disorders affecting bone, and rare diseases. A section on novel potential applications of HR-pQCT is also present, including assessment of rheumatic diseases, knee osteoarthritis, distal radius/scaphoid fractures, vascular calcifications, effect of medications, and skeletal muscle. The reviewed literature seems to suggest that a more widespread implementation of HR-pQCT in clinical practice would offer notable opportunities. For instance, HR-pQCT can improve the prediction of incident fractures beyond areal bone mineral density provided by dual-energy X-ray absorptiometry. In addition, HR-pQCT may be used for the monitoring of anti-osteoporotic therapy or for the assessment of mineral and bone disorder associated with CKD. Nevertheless, several obstacles currently prevent a broader use of HR-pQCT and would need to be targeted, such as the small number of installed machines worldwide, the uncertain cost-effectiveness, the need for improved reproducibility, and the limited availability of reference normative data sets.