Search

"Acral FibroChondroMyxoid tumor" (AFCMT) is a recently described distinctive subtype of acral soft tissue tumor that typically arises on the fingers and toes. We herein present the unreported imaging features of AFCMT in a 44-year-old woman. This otherwise healthy patient was referred for a painful, slow-growing, soft tissue mass in the middle finger of her right hand. Initial radiographs and computed tomography showed a small lesion centered in the soft tissue of the ulnar aspect of the proximal phalanx, associated with scalloping of the underlying bone. Magnetic resonance imaging confirmed the presence of a well-circumscribed soft tissue tumor that exhibited relatively high T2-weighted signal intensity and marked enhancement after contrast administration. Subsequent excisional biopsy was performed. Histologically, the tumor was characterized by an abundant stroma displaying fibrous, chondroid, and myxoid areas. By immunohistochemistry, tumor cells stained for CD34, ERG, and focally S100 protein. RNA-sequencing allowed detection of THBS1-ADGFR5 gene fusion which confirmed the diagnosis of AFCMT. At 2-year follow-up, the patient remains free of recurrence. AFCMT is a previously unrecognized entity that may mimic chondroma and should be considered in the differential diagnosis of soft tissue tumors with cartilaginous or myxoid stroma in the extremities.

We report an anatomico-radiologic study of humerus, femur, and tibia from a case of total congenital afibrinogenemia. Juxtatrabecular hemorrhages occur mainly in metaphyses and seem to be related to normal lines of stress. They may lead to the formation of intraosseous cysts and to a remodelling of bone trabeculae. The radiologic lesions in a second case, diagnosed as congenital dysfibrinogenemia, are similar to those found in Case 1 (femoral trabeculae remodelling) but also resemble some alterations described in hemophilia (pseudotumor of the right iliac bone). Anatomic study of the lesions in Case 2 was not possible. The significance of these observations could be better defined by a more extended skeletal study (radiologic and when feasible anatomic) of patients with congenital clotting defects and especially with inherited disorders of the fibrinogen molecule. It would also be worthwhile investigating manifest or latent hemostatic disorders (particularly at the fibrinogen level) in patients with solitary or aneurysmal bone cysts, and even with bone infarct or unexplained trabecular remodelling. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the usefulness of dynamic gadolinium-enhanced magnetic resonance imaging (MRI) for assessing the viability of the proximal pole of the scaphoid in patients with acute scaphoid fractures., Methods: Eighteen consecutive patients with acute scaphoid fracture who underwent dynamic gadolinium-enhanced MRI 7 days or less before surgery were prospectively included between August 2011 and December 2012. All patients underwent MR imaging with unenhanced images, enhanced images, and dynamic enhanced images. A radiologist first classified the MRI results as necrotic or viable based on T1- and T2-weighted images only, followed by a second blinded interpretation, this time including analysis of pre- and post-gadolinium administration images and a third blinded interpretation based on the time-intensity curve of the dynamic enhanced study. The standard of reference was the histologic assessment of a cylindrical specimen of the proximal pole obtained during surgery in all patients. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for unenhanced, enhanced, and dynamic gadolinium-enhanced MRI studies., Results: The sensitivity, specificity, PPV, and NPV were 67, 67, 50, and 80 % for unenhanced images, 83, 100, 100, and 92 for enhanced images, and 83, 92, 83, and 92 for dynamic contrast-enhanced images., Conclusions: Our data are consistent with previously reported data supporting contrast-enhanced MRI for assessment of viability, and showing that dynamic imaging with time-intensity curve analysis does not provide additional predictive value over standard delayed enhanced imaging for acute scaphoid fracture.

The following White Paper will discuss the appropriateness of gadolinium administration in MRI for musculoskeletal indications. Musculoskeletal radiologists should consider the potential risks involved and practice the judicious use of intravenous contrast, restricting administration to cases where there is demonstrable added value. Specific nuances of when contrast is or is not recommended are discussed in detail and listed in table format. Briefly, contrast is recommended for bone and soft tissue lesions. For infection, contrast is reserved for chronic or complex cases. In rheumatology, contrast is recommended for early detection but not for advanced arthritis. Contrast is not recommended for sports injuries, routine MRI neurography, implants/hardware, or spine imaging, but is helpful in complex and post-operative cases. [ABSTRACT FROM AUTHOR]

Extra-skeletal osteosarcoma is a rare malignant soft tissue sarcoma which can cause a diagnostic challenge due to its non-specific presentation and soft tissue mineralisation, thus potentially mimicking conditions such as myositis ossificans. This review will outline the demographics, clinical presentation, key imaging features, differential diagnosis, management and outcomes of extra-skeletal osteosarcoma and serve as a reference to radiologists and other clinicians involved in the care of patients with soft tissue tumours and tumour-like lesions. [ABSTRACT FROM AUTHOR]

Low-grade central osteosarcoma (LGCOS), which arises from the intramedullary cavity of the metaphysis of long bones, occasionally exhibits extraosseous spread. Approximately 10-30% of patients with LGCOS exhibit dedifferentiation, but it is rare to experience a primary tumor with a dedifferentiated component. A 38-year-old female patient presented with right knee pain for two months. Imaging studies revealed a bone mass with extraosseous involvement. Wide resection was performed, and pathologic examination led to the diagnosis of LGCOS with a dedifferentiated extraosseous lesion. A single defect in the bone cortex constituted the boundary between the low- and high-grade components. The extraosseous high-grade component included more tumor cells with p53 overexpression and more murine double minute 2 (MDM2) copies compared with the low-grade component. These genetic mutations and copy number alterations can be associated with malignant transformation of LGCOS., (© 2024. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Objective: Denosumab is an established targeted systemic therapy for treatment of giant cell tumor of bone (GCTB). We sought to determine whether treatment response could be quantified from radiomics analysis of radiographs taken longitudinally during treatment. Materials and methods: Pre- and post-treatment radiographs of 10 GCTB tumors from 10 patients demonstrating histologic response after treatment with denosumab were analyzed. Intensity- and texture-based radiomics features for each manually segmented tumor were calculated. Radiomics features were compared pre- and post-treatment in tumors. Results: Mean intensity (p = 0.033) significantly increased while skewness (p = 0.028) significantly decreased after treatment. Post-treatment increases in fractal dimensions (p = 0.057) and abundance (p = 0.065) approached significance. A potential linear correlation in mean (p = 0.005; ΔMean = 0.022 * duration − 0.026) with treatment duration was observed. Conclusion: Radiomics analysis of plain radiographs quantifies time-dependent matrix mineralization and trabecular reconstitution that mark positive response of giant cell tumors of bone to denosumab. [ABSTRACT FROM AUTHOR]

The term edema-like marrow signal intensity (ELMSI) represents a general term describing an area of abnormal signal intensity at MRI. Its appearance includes absence of clear margins and the possibility of exceeding well-defined anatomical borders (for example, physeal scars). We can define "ELMSI with unknown cause" an entity where the characteristic MR appearance is associated with the absence of specific signs of an underlying condition. However, it is more often an important finding indicating the presence of an underlying disease, and we describe this case as "ELMSI with known cause." It presents a dynamic behavior and its evolution can largely vary. It initially corresponds to an acute inflammatory response with edema, before being variably replaced by more permanent marrow remodeling changes such as fibrosis or myxomatous connective tissue that can occur over time. It is important to study ELMSI variations over time in order to evaluate the activity state and therapeutic response of an inflammatory chronic joint disease, the resolution of a trauma, and the severity of an osteoarthritis. We propose a narrative review of the literature dealing with various subjects about this challenging topic that is imaging, temporal evolution, etiology, differential diagnoses, and possible organization, together with a pictorial essay. [ABSTRACT FROM AUTHOR]

Osteosarcoma is the commonest primary malignant bone tumour in children and adolescents, the majority of cases being conventional intra-medullary high-grade tumours affecting the appendicular skeleton. Treatment is typically with a combination of neo-adjuvant chemotherapy, tumour resection with limb reconstruction and post-operative chemotherapy. The current article reviews the role of magnetic resonance imaging (MRI) in the pre-operative assessment of high-grade central conventional osteosarcoma. [ABSTRACT FROM AUTHOR]

Fibrous dysplasia (FD) is a benign fibro-osseous disease of the bone that may be solitary or multicentric. It is important to distinguish this type of lesion from low-grade osteosarcomas (LGOS) and from secondary sarcomas, because malignant transformation has rarely been reported. It is classically described as having a ground-glass appearance, endosteal scalloping, and thinning of the cortex. Cortical disruption is considered evidence of malignancy, but it can also be present in benign FD with aggressive behavior. We present an unusual case of aggressive FD of the 7th left rib, already diagnosed more than 22 years ago, where cortical and costo-vertebral joint disruption and 7th thoracic vertebral body involvement were not evidence of malignant behavior. From a histological perspective, FD and LGOS are similar; even if histology is of fundamental importance, the diagnosis has to be made based on the clinical and radiological aspects as well, although at imaging, differentiation between FD and LGOS can be difficult. In the present case, even though the histological examination suggested a benign lesion, the radiological examination instead consistently suggests malignancy. It is for this reason that there should be a high index of suspicion during follow-up and a new biopsy should be scheduled in case any changes occur during follow-up. [ABSTRACT FROM AUTHOR]

This case reports a 25-year-old woman initially diagnosed with adjacent benign notochordal cell tumors (BNCTs) of L3 and L4 based on needle biopsy of L3 and stable imaging over a 3-year period who was ultimately found to have a chordoma arising from a BNCT at L3. It illustrates the potential relationship between benign and malignant notochordal tumors and the difficulty in distinguishing them by clinical, radiological, and even histopathological means. [ABSTRACT FROM AUTHOR]

The article offers an overview of the diagnostic challenges in central low-grade osteosarcoma. To help in the resolution of the diagnosis, a discussion of recent molecular data is also provided. It offers a discussion of the radiological and pathological features of osteosarcoma which make diagnosis on imaging challenging.