Classification of Fibro-osseous Tumors in the Craniofacial Bones using DNA Methylation and Copy Number Alterations.

Fibro-osseous tumors of the craniofacial bones are a heterogeneous group of lesions comprising cemento-osseous dysplasia (COD), cemento-ossifying fibroma (COF), juvenile trabecular ossifying fibroma (JTOF), psammomatoid ossifying fibroma (PsOF), fibrous dysplasia (FD), and low-grade osteosarcoma (LGOS) with overlapping clinicopathological features. However, their clinical behavior and treatment differ significantly, underlining the need for accurate diagnosis. Molecular diagnostic markers exist for subsets of these tumors, including GNAS mutations in FD, SATB2 fusions in PsOF, mutations involving the RAS-MAPK signaling pathway in COD, and MDM2 amplification in LGOS.

Clinical outcome of curettage in atypical cartilaginous tumors of the long bones: a descriptive cohort study.

Background And Purpose:  Despite evolving management strategies for atypical cartilaginous tumors (ACT)-shifting from radical resection to intralesional curettage and "wait-and-scan" approaches-there remains no universal consensus on optimal treatment. We primarily aimed to evaluate disease-specific and progression-free survival following intralesional curettage and adjuvant phenol treatment of ACTs. Secondary aims included assessing surgical complications, the need for additional interventions, and an overview of long-term follow-up.

Inflammatory Pseudotumor of the Ulnar Nerve Mimicking a Malignant Peripheral Nerve Sheath Tumor: A Radiologic Puzzle.

Inflammatory pseudotumor (IPT) of a peripheral nerve is a rare non-neoplastic tumefactive inflammatory condition, often mimicking malignancy. The etiology of this condition is still unknown. Clinically and radiologically, the lesion can mimic a malignant tumor.

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.

Sacrococcygeal chordoma with spontaneous regression due to a large hemorrhagic component.

Chordoma is a malignant bone tumor originating from notochordal remnants, most commonly occurring at the sacrococcygeal junction. We present a case of a 70-year-old male with chronic pain in the lower lumbar spine. MRI performed elsewhere revealed a large tumor that involved S4, S5, and the coccyx with a presacral soft tissue component.

Small cell osteosarcoma versus fusion-driven round cell sarcomas of bone: retrospective clinical, radiological, pathological, and (epi)genetic comparison with clinical implications.

Small cell osteosarcoma (SCOS), a variant of conventional high-grade osteosarcoma (COS), may mimic fusion-driven round cell sarcomas (FDRCS) by overlapping clinico-radiological and histomorphological/immunohistochemical characteristics, hampering accurate diagnosis and consequently proper therapy. We retrospectively analyzed decalcified formalin-fixed paraffin-embedded (FFPE) samples of 18 bone tumors primarily diagnosed as SCOS by methylation profiling, fusion gene analysis, and immunohistochemistry.In eight cases, the diagnosis of SCOS was maintained, and in 10 cases it was changed into FDRCS, including three Ewing sarcomas (EWSR1::FLI1 in two cases and no identified fusion gene in the third case), two sarcomas with BCOR alterations (KMT2D::BCOR, CCNB3::BCOR, respectively), three mesenchymal chondrosarcomas (HEY1::NCOA2 in two cases and one case with insufficient RNA quality), and two sclerosing epithelioid fibrosarcomas (FUS::CREBL3 and EWSR1 rearrangement, respectively).

Wait-and-scan: an alternative for curettage in atypical cartilaginous tumours of the long bones.

Aims: Due to its indolent clinical behaviour, the treatment paradigm of atypical cartilaginous tumours (ACTs) in the long bones is slowly shifting from intralesional resection (curettage) and local adjuvants, towards active surveillance through wait-and-scan follow-up. In this retrospective cohort study performed in a tertiary referral centre, we studied the natural behaviour of ACT lesions by active surveillance with MRI. Clinical symptoms were not considered in the surveillance programme.

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.

Characterization of denosumab treatment response in giant cell tumors of bone with dynamic contrast-enhanced MRI.

Rationale And Objectives: Denosumab is a monoclonal antibody used neo-adjuvantly in giant cell tumor of bone (GCTB) to facilitate surgery, or long term for axial tumors where surgery comes with high morbidity. Time intervals for treatment effects to occur are unclear and monitoring tools are limited, complicating optimal drug dose titration. We assessed changes in time intensity curve (TIC) - derived perfusion features on DCE-MRI in GCTB during denosumab treatment and evaluated the duration of treatment effects on tumor perfusion.

MRI of diffuse-type tenosynovial giant cell tumour in the knee: a guide for diagnosis and treatment response assessment.

Tenosynovial giant cell tumour (TGCT) is a rare soft-tissue tumour originating from synovial lining of joints, bursae and tendon sheaths. The tumour comprises two subtypes: the localised-type (L-TGCT) is characterised by a single, well-defined lesion, whereas the diffuse-type (D-TGCT) consists of multiple lesions without clear margins. D-TGCT was previously known as pigmented villonodular synovitis.

Updated concepts in treatment of giant cell tumor of bone.

Purpose Of Review: Giant cell tumors of bone (GCTB) are intermediate, locally aggressive primary bone tumors. For conventional GCTB, surgery remains treatment of choice. For advanced GCTB, a more important role came into play for systemic therapy including denosumab and bisphosphonates over the last decade.

Malignant Transformation of Giant Cell Tumor of Bone and the Association with Denosumab Treatment: A Radiology and Pathology Perspective.

Objective: Malignancy in giant cell tumor of bone (mGCTB) is categorized as primary (concomitantly with conventional GCTB) or secondary (after radiotherapy or other treatment). Denosumab therapy has been suggested to play a role in the etiology of secondary mGCTB. In this case series from a tertiary referral sarcoma center, we aimed to find distinctive features for malignant transformation in GCTB on different imaging modalities.

Surgical management of 144 diffuse-type TGCT patients in a single institution: A 20-year cohort study.

Background And Objectives: Surgery is the mainstay of treatment for tenosynovial giant cell tumors (TGCTs). However, achieving a cure through surgery alone remains challenging, especially for the diffuse-type (D-TGCT).

Methods: Our goal was to describe the surgical management of patients with D-TGCT related to large joints, treated between 2000 and 2020.

MRI radiomics-based machine learning classification of atypical cartilaginous tumour and grade II chondrosarcoma of long bones.

Background: Atypical cartilaginous tumour (ACT) and grade II chondrosarcoma (CS2) of long bones are respectively managed with watchful waiting or curettage and wide resection. Preoperatively, imaging diagnosis can be challenging due to interobserver variability and biopsy suffers from sample errors. The aim of this study is to determine diagnostic performance of MRI radiomics-based machine learning in differentiating ACT from CS2 of long bones.

Intraosseous hibernoma of the appendicular skeleton.

Hibernomas are rare lipomatous tumors composed of brown adipocytes. The relative paucity of reported cases involving the bones accounts for the poor understanding of this entity, which is known to affect almost exclusively the axial skeleton. We present a case of intraosseous hibernoma of the humerus, which was found incidentally in a 52-year-old woman and initially misinterpreted as a cartilaginous tumor on magnetic resonance imaging (MRI).

Soft Tissue Sarcomas: The Role of Quantitative MRI in Treatment Response Evaluation.

Background: Although curative surgery remains the cornerstone of the therapeutic strategy in patients with soft tissue sarcomas (STS), neoadjuvant radiotherapy and chemotherapy (NART and NACT, respectively) are increasingly used to improve operability, surgical margins and patient outcome. The best imaging modality for locoregional assessment of STS is MRI but these tumors are mostly evaluated in a qualitative manner.

Objective: After an overview of the current standard of care regarding treatment for patients with locally advanced STS, this review aims to summarize the principles and limitations of (i) the current methods used to evaluate response to neoadjuvant treatment in clinical practice and clinical trials in STS (RECIST 1.

A moderate dose of preoperative radiotherapy may improve resectability in myxoid liposarcoma.

Background: Histotype specific neoadjuvant therapy response data is scarce in soft tissue sarcomas. This study aimed to assess the impact of a moderate radiotherapy (RT) dose on resectability and to correlate MRI parameters to pathological treatment response in Myxoid Liposarcoma (MLS).

Methods: This prospective, multicenter, single-arm, phase 2 trial assessed the radiological effects of 36 Gy of preoperative radiotherapy in primary non-metastatic MLS (n=34).

Radiological Assessment of Giant Cell Tumour of Bone in the Sacrum: From Diagnosis to Treatment Response Evaluation.

Giant cell tumour of bone (GCTB) typically occurs in young adults from 20-40 years old. Although the majority of lesions are located in the epi-metaphyses of the long bones, approximately one third of tumours are located in the axial skeleton, of which only 4% in the sacrum. Sacral tumours tend to be large at the time of presentation, and they present with aggressive features such as marked cortical destruction and an associated soft tissue component.

Imaging of Spinal Bone Tumors: Principles and Practice.

Age, location of the tumor, and detailed patient history can narrow the differential diagnosis of spinal bone lesions, including metastasis and primary benign and malignant bone tumors. Computed tomography and magnetic resonance imaging are both crucial in evaluating the characteristics of spinal bone tumors. Growth speed and Lodwick margin description can differentiate malignant from benign tumors to a certain degree.

Local control and postponement of systemic therapy after modest dose radiotherapy in oligometastatic myxoid liposarcomas.

Introduction: Oligometastatic disease and/or oligoprogression in myxoid liposarcoma(oMLS) triggers discussions on local treatment options and delay of systemic treatments. We hypothesized that satisfactory local control and postponement of systemic therapy could be achieved with a modest radiotherapy(RT) dose in oMLS.

Methods: The DOREMY trial is a multicenter, phase 2 trial evaluating efficacy and toxicity of a modest RT dose in both localized and oMLS; this report presents the data of the oMLS cohort treated with 36 Gy in 12-18 fractions with optional subsequent metastasectomy.