Long-Term Outcome of Single-Session, Ultrasound-Guided, Radiofrequency Ablation for Symptomatic Small, Lower Limb, Venous Malformations.

Objectives: To analyze the long-term clinical outcome of percutaneous, ultrasound-guided radiofrequency ablation (RFA) of peripheral low-flow vascular malformations (VM). Materials & Methods: Adolescent patients presenting with symptomatic VMs and referred for percutaneous management using RFA between January 2010 and January 2015 were identified by a search in the institutional interventional radiology database. Clinical and radiological follow-up up to April 2021 was based on retrospective analysis of patients' electronic medical records and imaging files. This retrospective study was approved by the institutional ethics committee. Results: Four female patients (median age 16 years) presented with lower extremity pain, swelling and functional disability related to VM as confirmed by magnetic resonance imaging. Two patients underwent percutaneous sclerotherapy previously. Clinical follow-up (mean of 8,5 years) showed complete and sustained resolution of the symptoms in all patients; in one patient a persistent decrease in muscular strength of the treated limb was noted. Conclusion: Percutaneous, ultrasound-guided RFA is relatively safe and efficient with durable symptom relief in the management of small, lower limb VMs in adolescent patients.

Radium-223 in patients with prostate specific antigen (PSA) progression and without clinical metastases following maximal local therapy: A pilot study.

BACKGROUND: Despite the curative intent of radical prostatectomy (RP) (+/- radiotherapy (RT)), 30% of the clinically localized prostate cancer (CaP) patients will develop rising PSA (prostate specific antigen). In absence of clinical recurrence, there is a lack of effective treatment strategies in order to control the disease at its earliest (micro)metastatic stage. The aim of this study was to assess safety, tolerability, and biochemical response of off-label Radium-223 (Xofigo) treatment in CaP patients with PSA relapse following maximal local therapy. METHODS: We conducted a prospective, single arm, single center open-label, pilot study with Radium-223 in CaP patients with rising PSA (>0.2 ng/ml) following RP + adjuvant/salvage RT. Negative staging with 68Ga-PSMA-11 PET/CT and whole-body MRI was mandatory at time of inclusion. Patients were eligible if they exhibited adverse clinico-pathological features predictive of significant recurrence. Safety, tolerability, biochemical progression (defined as PSA increase >50% from PSA nadir) and clinical recurrence were assessed. RESULTS: In total, 23 patients were screened of whom 8 patients were included is the study. Radium-223 treatment was safe with no serious treatment related adverse events. One patient developed grade 3 lymphopenia. All patients rapidly developed PSA progression (median PSA progression-free survival: 5.5 months). Eventually all patients experienced clinical recurrence (median clinical recurrence-free survival 11.0 months) of whom only 2 patients developed skeletal recurrence. CONCLUSIONS: Radium-223 in patients with PSA relapse following maximal local treatment without clinical metastases is safe. However, the clinical benefit of Ra-223 in this setting is doubtful as significant oncological benefit is lacking.

Ossifying Subperiosteal Hematoma Caused by a Plexiform Neurofibroma.

Teaching Point: Subperiosteal haemorrhage is a rare complication of a plexiform neurofibroma which may mimic a malignant peripheral nerve sheath tumour.

Whole-body diffusion-weighted magnetic resonance imaging for the detection of bone metastases and their prognostic impact in metastatic renal cell carcinoma patients treated with angiogenesis inhibitors.

Background: Metastatic renal cell carcinoma (mRCC) patients with bone metastases (BM) are at high risk for skeletal related events and have a poorer outcome when treated with vascular endothelial growth factor receptor-tyrosine kinase inhibitors (VEGFR-TKIs). Computed tomography (CT) lacks sensitivity to detect BM in mRCC. We aimed to determine the added value of whole body diffusion-weighted magnetic resonance imaging (WB-DWI/MRI) to CT for the detection of BM in mRCC and to estimate the prognostic impact of the number of BM in mRCC patients treated with VEGFR-TKIs.Material and methods: We conducted a prospective study including consecutive mRCC patients treated with a first-line VEGFR-TKI in the metastatic setting. All patients underwent a pretreatment thoracic-abdominal-pelvic CT and WB-DWI/MRI. CT and WB-DWI/MRI were compared for the detection of BM. The number of detected BM was correlated with response rate (RR), progression-free survival (PFS) and overall survival (OS) after start of the VEGFR-TKI.Results: Ninety-two patients were included. BM were found in 55% of the patients by WB-DWI/MRI and in 43% of the patients by CT (p = .003). Mean number of BM discovered per patient was 6.8 by WB-DWI/MRI versus 1.9 by CT (p = .006). The cutoff of ≤5 versus >5 BM on WB-DWI/MRI had the highest discriminative power for all outcome measures. Patients with >5 BM had a lower RR (10% versus 42%), more frequently early progressive disease (43% versus 13%, p = .003), shorter PFS (4 versus 10 months, p = .006) and shorter OS (10 versus 35 months, p < .0001) compared to patients with ≤5 BM.Conclusion: WB-DWI/MRI detects significantly more BM in mRCC patients than CT, allowing better estimation of the prognostic impact of BM in mRCC patients treated with VEGFR-TKIs. The prognostic impact should now be validated in patients treated with immune checkpoint inhibitors.

Bone metastases and age are associated with earlier dose reductions in metastatic clear-cell renal cell carcinoma patients treated with angiogenesis inhibitors.

Background: Metastatic clear-cell renal cell carcinoma (m-ccRCC) patients with bone metastases (BM) treated with vascular endothelial growth factor receptor (VEGFR) tyrosine kinase inhibitors (TKI) have a poorer outcome compared to patients without BM. We aimed to investigate whether an increased incidence of VEGFR-TKI treatment interruptions and/or dose reductions in patients with BM could explain this difference in outcome. Patients and methods: Retrospective study on m-ccRCC patients treated in first-line with VEGFR-TKI. Analysis of the incidence of treatment interruptions and dose reductions and time-to-event analysis. Study of the correlation with the presence of BM at start of first-line VEGFR-TKIs. Results: Two-hundred-and-five patients were included. In patients with BM, median time-to-dose-reduction was significantly shorter (3 versus 5 cycles; p = 0.005) than in patients without BM. 63% of the total number of cycles was administered at reduced dose, compared to 41% in patients without BM. Age at start of VEGFR-TKI (≤ versus >70 years) was significantly associated with median time-to-dose-reduction (5 versus 3 cycles; p = 0.007). On multivariate analysis, the presence of BM (p = 0.004; HR 1.82, 95%CI 1.21-2.73) and age at start of VEGFR-TKIs (p = 0.017; HR 1.65, 95%CI 1.10-2.50) were independently associated with time-to-dose-reduction. Conclusion: In m-ccRCC patients treated with VEGFR-TKIs, dose reductions occurred earlier in patients with BM compared to patients without BM and in elderly patients.

Voriconazole-Induced Periostitis After Allogeneic Stem Cell Transplantation.

A 34-year-old man with history of Hodgkin lymphoma presented 7 months after allogeneic stem cell transplantation with an unexplained severe musculoskeletal pain syndrome. A Tc-MDP bone SPECTCT showed multiple foci with moderate to intense bone uptake across the axial and appendicular skeleton consistent with periostitis. The patient had been on voriconazole daily for 4 months to treat an Aspergillus pneumonia, and in the absence of other causes, a drug-induced periostitis was suspected. Voriconazole was changed to posaconazole with complete resolution of the musculoskeletal symptoms within 3 weeks.

The characteristics of 76 atypical neurofibromas as precursors to neurofibromatosis 1 associated malignant peripheral nerve sheath tumors.

Background: Neurofibromatosis 1 (NF1) leads to the development of benign and malignant peripheral nerve sheath tumors (MPNST). MPNST have been described to develop in preexisting benign plexiform neurofibromas (PN) and have a poor prognosis. Atypical neurofibromas (ANF) were recently described as precursor lesions for MPNST, making early detection and management of ANF a possible strategy to prevent MPNST. We aimed to clinically characterize ANF and identify management approaches. Methods: We analyzed clinical, imaging, and pathology findings of all patients with NF1 and ANF at 3 institutions. Results: Sixty-three patients had 76 ANF (32M/31F; median age 27.1 y). On MRI, most ANF appeared as distinct nodular lesions and were 18F-fluorodeoxyglucose (FDG) avid. Forty-six ANF were associated with pain, 19 with motor weakness, 45 were palpable or visible, and 13 had no clinical signs. Completely resected ANF (N = 57) have not recurred (median follow-up, 4.1 y; range, 0-14 y). Four ANF transformed into MPNST and 17 patients had a history of MPNST in a different location than was their ANF. Conclusions: Growth of distinct nodular lesions, pain, and FDG-PET avidity should raise concern for ANF in NF1. Patients with ANF are at greater risk for development of MPNST. Complete resection of ANF may prevent development of MPNST.

Imaging features of chondromyxoid fibroma: report of 15 cases and literature review.

OBJECTIVE:: Chondromyxoid fibroma (CMF) is a rare benign bony tumour. Our objectives are three-fold: first, comparing MRI, conventional radiography (CR) and CT characteristics of CMF; second, providing a literature review; and third, summarizing the role of imaging landmarks in the differential diagnosis with other bony lesions. METHODS:: 15 patients with histopathologically proven CMF were retrospectively included. MR images were reviewed for typical findings and compared with imaging features on CR and CT. RESULTS:: All lesions were isointense on T1 weighted images with a low intensity rim in nine cases corresponding to the perilesional sclerosis on CR and CT. Internal trabeculations were more prominent on CR than on CT due to corrugation and scalloping at the tumour's edge. CT was superior to CR in analysing the expansion of the lesion, cortical breakthrough and internal mineralization. T2 short tau inversion recovery or T2 weighted fat saturation images showed an intermediate to hyperintense signal in all lesions, mostly heterogeneous. Some lesions contained small internal cysts, and one lesion demonstrated a low signal intensity centrally, corresponding to internal mineralization on CT. Intense contrast enhancement was present in all lesions. Minimal bone and soft tissue oedema were seen in, respectively, six and three patients. CONCLUSION:: The diagnosis of CMF is difficult because of overlap of characteristics with other bony lesions. Our comparative study puts forward advantages and limitations of different imaging modalities in the diagnosis of CMF. ADVANCES IN KNOWLEDGE:: For the first time, imaging features of CMF are analysed and compared on CR, CT and MRI.

Role of diffusion-weighted magnetic resonance imaging in the evaluation of scalp dermatofibrosarcoma protuberans.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare tumor that grows with tentacle-like projections. In particular, on the scalp this could explain the frequent incomplete resections and the high rate of multiple local recurrences. Adequate pre- and postoperative imaging methods to visualize these prolongations are required to determine the extent of the tumor and to intercept recurrences in a short time base. METHODS: A retrospective study was performed on two cases of multiple recurrent scalp DFSP. The use of diffusion-weighted imaging (DWI) for pre- and postoperative assessment of DFSP was evaluated. RESULTS: Diffusion-weighted imaging magnetic resonance imaging was able to visualize the horizontal extent of tumor prolongations in the periosteum. Also, residual tumor tissue was distinguished from scar tissue in a large area of previous surgical interventions. CONCLUSION: We found DWI magnetic resonance imaging a valuable tool in planning the multidisciplinary therapeutic approach and follow-up of DFSP on the scalp.

Blau syndrome: cross-sectional data from a multicentre study of clinical, radiological and functional outcomes.

OBJECTIVE: To report baseline articular, functional and ocular findings of the first international prospective cohort study of Blau syndrome (BS). METHODS: Three-year, multicentre, observational study on articular, functional (HAQ, Childhood HAQ and VAS global and pain), ophthalmological, therapeutic and radiological data in BS patients. RESULTS: Baseline data on the first 31 recruited patients (12 females and 19 males) from 18 centres in 11 countries are presented. Of the 31 patients, 11 carried the p.R334W NOD2 mutation, 9 the p.R334Q and 11 various other NOD2 missense mutations; 20 patients were sporadic and 11 from five BS pedigrees. Median disease duration was 12.8 years (1.1-57). Arthritis, documented in all but one patient, was oligoarticular in 7, polyarticular in 23. The median active joint count was 21. Functional capacity was normal in 41%, mildly impaired in 31% and moderate-severe in 28% of patients. The most frequently involved joints at presentation were wrists, ankles, knees and PIPs. On radiographs, a symmetrical non-erosive arthropathy was shown. Previously unknown dysplastic bony changes were found in two-thirds of patients. Ocular disease was documented in 25 of 31 patients, with vitreous inflammation in 64% and moderate-severe visual loss in 33%. Expanded manifestations (visceral, vascular) beyond the classic clinical triad were seen in 52%. CONCLUSION: BS is associated with severe ocular and articular morbidity. Visceral involvement is common and may be life-threatening. Bone dysplastic changes may show diagnostic value and suggest a previously unknown role of NOD2 in bone morphogenesis. BS is resistant to current drugs, suggesting the need for novel targeted therapies.

Postoperative evolution of thickness and echogenicity of cutis and subcutis of patients with and without breast cancer-related lymphedema.

BACKGROUND: Compared to volume/circumference measurements to diagnose lymphedema, thickening and disturbance of echogenicity of cutis and subcutis associated with the development of lymphedema is measured directly by ultrasonography. The aim of the present study was to investigate the evolution of thickness and echogenicity of cutis and subcutis of the lower and upper arm after axillary dissection for breast cancer and compare patients with and without arm lymphedema. METHODS AND RESULTS: Immediately after the surgery and at 6 and 12 months post-surgery, 42 patients received an ultrasonography of both arms, at the wrist, ventral and dorsal side of the lower arm, and biceps and triceps side of the upper arm. Thickness and echogenicity of cutis and subcutis were determined. Lymphedema was defined as 5.0% or more increase compare to the preoperative value. Eighteen patients had developed lymphedema of the lower arm, and 24 patients of the upper arm. Thickness of the cutis decreased significantly during the postoperative period on the healthy and affected side as well. The difference of cutis thickness between affected and healthy side increased postoperatively in patients with lymphedema and decreased in patients without lymphedema at the wrist and dorsal side of the lower arm. Patients with lymphedema had more frequent disturbance of echogenicity of the cutis at all measurement points. In exception of the triceps, thickness and echogenicity of the subcutis is less obvious disturbed. Sensitivity to detect arm lymphedema by ultrasonography was acceptable for increased subcutis thickness at the ventral lower arm and triceps and disturbed cutis echogenicity at the wrist (67%-100%). Sensitivity for the other measurements was bad. Specificity was for all measurements acceptable (59%-100%). CONCLUSION: Ultrasonography may be useful to diagnose lymphedema developing after axillary dissection for breast cancer. However, it cannot be used as a separate diagnostic test for lymphedema.

Atlantoaxial instability in monozygotic twin sisters: degenerative or congenital disease?

The authors report on two 70-year-old monozygotic twin sisters who both suffered severe nontraumatic atlantoaxial instability. If either had been a solitary case, degenerative atlantoaxial instability would have been the most straightforward diagnosis. In this case report the authors attempt to answer the question of whether an underlying congenital predisposition might be involved.

Botulinum toxin type A injections in the psoas muscle of children with cerebral palsy: muscle atrophy after motor end plate-targeted injections.

UNLABELLED: MEP targeting during BoNT-A injections has been demonstrated to improve outcome. Two injection techniques of the psoas muscle - proximal MEP targeting versus a widely used more distal injection technique - are compared using muscle volume assessment by digital MRI segmentation as outcome measure. METHOD: 7 spastic diplegic children received injections in both psoas muscles: two different injection techniques randomly in 5 patients, in 2 patients bilateral MEP targeting. MRI images of the psoas were taken before, after 2 months and in 3 patients after 6 months. RESULTS: Average post injection volume (in relation to pre-injection volume) for the MEP targeted muscles (9) is 79.5% versus 107.8% in the 5 distal injected psoas muscles (p=0.0033). In all 5 asymmetric injected patients the MEP targeted psoas had a larger volume reduction than the more distal injected psoas muscle. This atrophy remains even 6 months after the injection. This is the first study were a longitudinal follow-up by MRI demonstrates muscle atrophy after BoNT-A in children with CP. Injections in the MEP zone of the muscle, which is the more proximal part of the psoas muscle, cause atrophy in contrary to more distal injections were this atrophy is not observed.

Validation of a novel spinal posture monitor: comparison with digital videofluoroscopy.

PURPOSE: A novel, minimally invasive posture monitor which can monitor lumbar postures outside the laboratory has demonstrated excellent reliability, as well as concurrent validity compared to a surface marker-based motion analysis system. However, it is unclear if this device reflects underlying vertebral motion. METHODS: Twelve participants performed full range sagittal plane lumbo-pelvic movements during sitting and standing. Their posture was measured simultaneously using both this device (BodyGuard™) and digital videofluoroscopy. RESULTS: Strong correlations were observed between the two methods (all r (s) > 0.88). Similarly, the coefficients of determination were high (all r (2) > 0.78). The maximum mean difference between the measures was located in the mid-range of motion and was approximately 3.4° in sitting and 3.9° in standing. CONCLUSION: The BodyGuard™ appears to be a valid method for analysing vertebral motion in the sagittal plane and is a promising tool for long-term monitoring of spinal postures in laboratory and clinical settings in people with low back pain.

Perineural spread of endometriosis along the obturator nerve into the adductor thigh compartment.

We report the case of a young woman with recurrent unilateral hip pain. A polylobular cystic mass was found in the right adductor space. Magnetic resonance imaging (MRI) revealed a polynodular mass migrating from the intrapelvic region along the obturator nerve. Because of a history of cyclic pain and the characteristics on MRI (hypointense rim and spots of spontaneous hyperintense signal on T1-weighted images), an endometrioma was suspected. The diagnosis of endometriosis was later confirmed through ultrasound-guided biopsy.

Breast cancer in pregnancy: recommendations of an international consensus meeting.

PURPOSE: To provide guidance for clinicians about the diagnosis, staging and treatment of breast cancer occurring during an otherwise uncomplicated pregnancy. METHODS: An international expert Panel convened to address a series of questions identified by a literature review and personal experience. Issues relating to the diagnosis and management of breast cancer after delivery were outside the scope. RESULTS: There is a paucity of large and/or randomized studies. Based on cohort studies, case series and case reports, the recommendations represent the best available evidence, albeit of a lower grade than is optimal. RECOMMENDATIONS: In most circumstances, serious consideration should be given to the option of treating breast cancer whilst continuing with the pregnancy. Each woman should ideally be referred to a centre with sufficient expertise, given a clear explanation of treatment options. Most diagnostic and staging examinations can be performed adequately and safely during pregnancy. Treatment should however be adapted to the clinical presentation and the trimester of the pregnancy: surgery can be performed during all trimesters of pregnancy; radiotherapy can be considered during the first and second trimester but should be postponed during the third trimester; and standard chemotherapies can be used during the second and third trimester. Since neonatal morbidity mainly appears to be related to prematurity, delivery should not be induced before 37 weeks, if at all possible. CONCLUSIONS: The treatment of breast cancer in pregnancy should be executed by experienced specialists in a multidisciplinary setting and should adhere as closely as possible to standard protocols.