Clinical impact of whole-body MRI in staging and surveillance of patients with myxoid liposarcoma: a 14-year single-centre retrospective study.

OBJECTIVE: To assess the clinical impact of regular whole-body magnetic resonance imaging (WBMRI) surveillance in myxoid liposarcoma patients. METHODS: This was a retrospective cohort study of myxoid liposarcoma patients who underwent at least one WBMRI at our institution between October 2006 and December 2020. The effect of WBMRI on clinical management, namely treatment modification or additional diagnostic investigations was studied. A standardised WBMRI surveillance protocol was instituted in 2015. We compared patient outcomes for the metastatic patients who had and had not received regular WBMRI surveillance and performed survival analysis for both subgroups. RESULTS: Of the 56 patients (60.7% male, median age: 48.1 years) who underwent 345 WBMRI, 17 (30.3%) had metastases, and 168 WBMRI were performed in this group. The median imaging follow-up for the entire cohort was 35 months; the metastatic group had a median follow-up of 42 months. WBMRI changed the clinical management in 13 (76.5%) metastatic patients, with 33 instances of treatment modification. Thirty-five lesions were labelled 'indeterminate,' 16 (45.7%) had additional investigations/interventions, and 4 (11.4%) were confirmed to be metastatic. Twenty-one metastatic lesions were missed initially on WBMRI and confirmed on subsequent WBMRI, of which 5 (23.8%) were clinically significant. The 5-year survival since the detection of metastasis was better in the regular surveillance subgroup (85.7% vs. 45%), but this was not statistically significant (p = 0.068). Five patients (8.9%) developed their first metastasis more than 5 years after diagnosing the primary lesion. CONCLUSION: Regular WBMRI surveillance of myxoid liposarcoma patients considerably impacts clinical management by frequently influencing treatment decisions. CLINICAL RELEVANCE STATEMENT: WBMRI has been recently recommended as an imaging option for the staging and surveillance of myxoid liposarcoma patients. Our study highlights the impact of regular WBMRI surveillance on the clinical management of these patients and how it affects their survival.

Atypical femur fracture in a woman with osteogenesis imperfecta and multiple myeloma.

Use of intravenous bisphosphonates has been demonstrated to improve clinical outcomes in children with osteogenesis imperfecta (OI). However, efficacy and safety of bisphosphonates in adults with OI remains unclear. Atypical femur fractures (AFF) are rare insufficiency fractures associated with long-term bisphosphonate use. We report on a 56 year old woman with OI type 1 and long-term bisphosphonate use who was diagnosed with multiple myeloma (MM) following a severe vertebral fracture. During workup, an asymptomatic incomplete AFF of the left femur diaphysis was noted. Multiple factors may have contributed to the occurrence of AFF, including bisphosphonate exposure, bowing of the proximal femur, as well as the intrinsic collagen defect of OI. To reduce the risk of skeletal complications from MM, intravenous pamidronate was administered in addition to chemotherapy, though in reduced dose and frequency. Orthopedic consultant recommended against prophylactic surgery for the AFF. Follow-up radiograph showed no progression of the AFF, though delayed healing was present. This case highlights the importance of close monitoring of patients on long-term bisphosphonate therapy who have additional risk factors for developing AFF, such as underlying genetic bone disorders or lower limb deformities. A multidisciplinary approach is recommended for optimal management of such complex patients.

Early detection of metastases using whole-body MRI for initial staging and routine follow-up of myxoid liposarcoma.

OBJECTIVE: To define the role of whole-body MRI (WBMRI) for initial staging and routine follow-up of myxoid liposarcoma (MLS). MATERIALS AND METHODS: A retrospective review of all the patients with MLS who underwent WBMRI for initial staging and routine follow-up at our institution between October 1, 2006, and September 30, 2016 was performed. Patient demographics, clinical presentation, imaging findings, tumor histology, and occurrence and location of metastatic disease were recorded. Thirty-three patients who underwent a total of 150 WBMRI examinations were included in the study. RESULTS: Nine patients (27%) were diagnosed with metastases between 0 and 60 months (median 10; interquartile range, 7-13) from the diagnosis of the primary tumor. The initial site of metastatic disease was extrapulmonary in all patients. Only two patients developed pulmonary metastases, which were diagnosed by CT chest 9 and 29 months after the diagnosis of extrapulmonary metastases. The first metastasis was diagnosed by WBMRI in seven patients (78%), by thoracic CT in one patient, and by abdominal CT in one patient. Eight of nine patients (89%) were asymptomatic at the time of diagnosis of the metastases. In seven patients (78%), WBMRI demonstrated metastases included within the field of view of, but occult on a contemporaneous CT scan. CONCLUSION: Our 10-year institutional experience demonstrates that WBMRI facilitates early detection of extrapulmonary MLS metastases before the onset of clinical symptoms and pulmonary metastases. WBMRI also depicts extrapulmonary metastases that are occult on CT scans. The current surveillance strategies are insufficient for screening for extrapulmonary MLS metastases.

Solitary fibrous tumor: an update on the spectrum of extrapleural manifestations.

Solitary fibrous tumor (SFT) is a rare tumor initially believed to be a benign localized pleural tumor of mesothelial origin. Over the past few years, the literature on this tumor has grown tremendously. The tumor is now reported in diverse bodily locations, and recognized to have a wider range of clinical and radiological features. The most common extrapleural sites of the tumor are the orbits and the extremities. Tumors are often well-circumscribed masses, and vary in size from 1 cm to over 30 cm. The admixture of histological components in the tumor, namely, fibrous tissue, cellular components, and highly vascularized areas consisting of numerous closely packed small to medium-sized blood vessels, influence the imaging appearances of the tumor. On magnetic resonance imaging (MRI), the diagnosis of solitary fibrous tumor is suggested by a well-circumscribed mass that has smooth margins, and focal or diffuse hypointense signal on T2-weighted imaging due to fibrous content in the tumor. SFTs demonstrate strong focal or diffuse contrast enhancement due to the highly vascularized areas in the tumor.

Angiogenesis in carotid atherosclerotic lesions is associated with timing of ischemic neurological events and presence of computed tomographic cerebral infarction in the ipsilateral cerebral hemisphere.

We studied the association between plaque vascularity, timing of neurological and ocular symptoms, and presence of cerebral infarction in the ipsilateral cerebral hemisphere on preoperative computed tomographic (CT) scan. Consecutive patients undergoing carotid endarterectomy for carotid stenosis were included. All patients underwent preoperative noncontrast cerebral CT. Histological sections were obtained from carotid endarterectomy specimens and stained with an endothelial cell marker (CD34). Microvessel counts were performed in CD34-stained sections and verified through computerized image analysis. Associations between microvessel density in carotid atherosclerotic plaques, preoperative ipsilateral CT evidence of cerebral infarction, and timing of ipsilateral neurological and ocular events were assessed. Seventy-three patients underwent carotid endarterectomy, of whom 17 were symptomatic within 30 days of carotid endarterectomy, 11 were asymptomatic, and a further 45 had a preoperative symptom-free interval of 31-540 days (median = 56). Eighteen patients (24.6%) had CT evidence of cerebral infarction. Significantly higher microvessel counts were observed in patients with CT evidence of cerebral infarction in the appropriate hemisphere compared with patients who did not (p = 0.02). There was an inverse relationship between the microvessel density in atherosclerotic lesions and the timing of ischemic neurological events (odds ratio [OR] = 4.63, 95% confidence interval [CI] 2.95-7.28, p < 0.001). This relationship was independent of patient age (OR = 1.03, 95% CI 0.55-1.99, p = 0.70), sex (OR = 1.18, 95% CI 0.47-2.05, p = 0.56), smoking (OR = 1.07, 95% CI 0.54-2.09, p = 0.84), diabetes (OR = 0.90, 95% CI 0.45-1.79, p = 0.76), and hypercholesterolemia (OR = 0.98, 95% CI 0.68-1.11, p = 0.88). This study confirms the relationship between angiogenesis in carotid atherosclerotic lesions and development and chronology of ipsilateral hemispheric neurological events.

Prediction of the exact degree of internal carotid artery stenosis using an artificial neural network based on duplex velocity measurements.

Duplex ultrasound criteria use a combination of velocity measurements to evaluate internal carotid artery (ICA) stenosis. These evaluations divide ICA stenosis into broad categories. The aim of this study was to design an artificial neural network (ANN) capable of predicting the exact degree of ICA stenosis based on duplex velocity measurements. Consecutive patients with significant carotid atherosclerosis underwent carotid duplex ultrasound and angiography. Peak systolic and end-diastolic velocities in the ICA and common carotid artery were measured. Multilayered perceptron ANNs were constructed and trained to predict the degree of ICA stenosis and band the degree of ICA stenosis into 10% intervals based on these measurements. The accuracy of the ANN models in predicting the degree of ICA stenosis and classifying the ICA stenosis was compared with the angiographic degree of ICA stenosis and duplex velocity criteria. A total of 208 carotid bifurcations were studied. ANNs were able to accurately predict the degree of angiographic ICA stenosis (R2 = 0.9374, p < 0.0001) and band the ICA stenosis into the predefined 10% intervals [sensitivity 97.3% (95% CI 90.7-99.3), specificity 97.7 % (95% CI 93.6-99.2), accuracy 97.5%]. The ANN model was more accurate [discriminant power (DP) = 4.11] in banding the degree of ICA stenosis than duplex velocity criteria (DP = 1.67) (p < 0.05). The accuracy of the ANN in correctly identifying >70% ICA stenosis was 98.4% [sensitivity 96.4% (95% CI 93.8-99.3), specificity 98.7% (95% CI 93.4-99.8), DP = 4.21]. ANNs can accurately predict the degree of ICA stenosis. With further refinement, ANNs could replace velocity criteria in the assessment of ICA stenosis using duplex ultrasound.