Transarticular epithelioid hemangioma of the ankle-a case of a rare vascular neoplasm.

Epithelioid hemangioma of the bone is a rare kind of vascular neoplasm posing a diagnostic challenge because of its ability to mimic malignant tumors. We report a case of a fast-growing, talofibular joint-involving epithelioid hemangioma, which was suspectedly initiated by vascular damage due to trauma and arthroscopy. The ankle mass appeared as a lytic lesion on the CT images and as a T1 hypo-, T2 mildly hyperintense, lobulated structure on the MRI scans. The contrast enhancement pattern was typical to vascular neoplasms. Histologically the lesion consisted of well-formed vessels lined with epithelioid cells with a slightly atypical nuclear morphology, inflammation with a significant number of eosinophils, and low mitotic rate. Immunohistochemistry analysis showed the presence of vascular markers but no rearrangements characteristic of soft tissue sarcomas were registered by the next-generation sequencing. The surgical treatment was curative. The report presents current imaging methods and summarizes the imaging findings of transarticular spreading tumors. The paper also highlights that for the differential diagnosis of vascular tumors showing signs of aggressivity, the pathological analysis is inevitable. Correct diagnosis of the epithelioid hemangioma is essential, as the treatment of more malignant entities is substantially different. An added value of the report is that to the best of our knowledge, a transarticular spreading epithelioid hemangioma of the ankle has never been described before.

Correction to: Triple elastofibromas located in the supra- and infrascapular regions-a case report.

The original version of this article unfortunately contained mistakes. The authornames of Matías de Albert, Manuel Perez, and Rosa Dominguez Oronoz as well as the affiliation of Dr. Perez are now corrected in this article.

Aggressive Angiomyxoma: Imaging Findings in 3 Cases With Clinicopathological Correlation and Review of the Literature.

Aggressive angiomyxoma is a rare mesenchymal tumor with a typical presentation as a slowly growing perineal soft tissue mass in paravulvar and pararectal region in young adult women. We present 3 cases of aggressive angiomyxoma with clinicopathological correlation and describe their main imaging features with emphasis on magnetic resonance imaging, adding useful information about their behavior on dynamic contrast-enhanced sequences and diffusion-weighted imaging and including a comprehensive review of the existing literature.

Medium-term outcome of mosaicplasty for grade III-IV cartilage defects of the knee.

PURPOSE: To evaluate the medium-term outcome of mosaicplasty for full-thickness cartilage defects of the knee joint in 17 patients. METHODS: Records of 12 men and 5 women aged 16 to 57 (mean, 35) years who underwent mosaicplasty for grade III/IV osteochondral defects in the lateral (n=14) or medial (n=3) femoral condyle were reviewed. 12 of the patients had undergone knee surgeries. The mean size of the defects was 3.4 (range, 1-4) cm(2). Three patients had defects of >2 cm(2). All operations were performed by a single surgeon using mini-arthrotomy. The lateral edge of the trochlea was the donor site. Graft integration and the presence of any abnormality at the articular surface were assessed using magnetic resonance imaging (MRI). In addition, patients were evaluated using the International Knee Documentation Committee (IKDC) rating scale, the SF-36 health questionnaire, visual analogue scale (VAS) score for pain. RESULTS: Two of the 17 patients developed necrosis and cystic degeneration of the grafts and underwent conversion to unicompartmental knee arthroplasty within 2 years. They were older than 45 years and had defects of >2 cm(2). Respectively in years 4 and 7, one and 4 patients were lost to follow-up, the mean IKDC score was 75% and 88%, the SF-36 score was 83% and 90%, and the VAS score was ≤3 in 13 of 14 patients at year 4 and in all 11 patients at year 7. At the 7-year follow-up, patient satisfaction with mosaicplasty was excellent in 8 patients, good in 3, and poor in 2 (who underwent unicompartmental knee arthroplasty). At year 4, MRI showed integration of the cartilage repair tissue and incorporation of the osseous portion of the graft into the bone in 13 of the 14 patients. The remaining patient had osteoarthritis at the graft donor site. At year 7, MRI showed good integration of the implant in all 11 available patients, but fissures were seen on the cartilage surface in 3 patients. CONCLUSION: The medium-term outcome of autologous mosaicplasty for symptomatic osteochondral defects in the femoral condyle is good. Longer follow-up is needed to determine the structural and functional integrity of the graft over time.

Synthetic resorbable scaffolds for the treatment of isolated patellofemoral cartilage defects in young patients: magnetic resonance imaging and clinical evaluation.

BACKGROUND: Surgical management of patellar cartilage defects remains controversial. The ideal technique to regenerate hyaline cartilage is not yet defined. However, a synthetic resorbable osteochondral scaffold plug (TruFit CB) seems to offer a treatment option with good results at short-term follow-up, at least in the condylar setting. HYPOTHESIS: A synthetic implant provides a simple and efficacious means of treating the cartilage defects of the patellofemoral joint in young patients. STUDY DESIGN: Case series; Level of evidence, 4. METHODS: A study was designed to evaluate prospectively short- and medium-term results in patients with osteochondral patellar defects treated with synthetic reabsorbable scaffolds. Patient outcome scores (Short Form 36 [SF-36] and Knee injury and Osteoarthritis Outcome Score [KOOS]), demographics, prior surgeries, and data from a physical examination were collected at baseline (before implantation) and at 6, 12, and 24 months after surgery. Defect characteristics were collected during implantation. Diagnosis and monitoring were performed by magnetic resonance imaging. RESULTS: Ten patients with a mean age of 33.3 years (range, 16-49 years) were evaluated prospectively at 24 months' follow-up. The number of plugs used for each patient ranged from 1 to 4. At 1-year follow-up, the results were satisfactory in 8 of 10 patients, and poor in 2, according to clinical assessment (KOOS, visual analog scale, and SF-36). At 18 months of follow-up, all patients except one complained of pain and knee swelling. Reoperation rate for implant failure at final follow-up was 70%. Magnetic resonance imaging at final follow-up showed a cylindrical cavity of fibrous tissue instead of subchondral bone restoration. CONCLUSION: A synthetic implant can improve symptoms and joint function, especially for small lesions, only for a short period of time. However, 2 years of monitoring has shown its failure in restoring the subchondral bone despite the formation of predominant hyaline cartilage from synthetic resorbable scaffolds. Under current conditions and according to our experience, we do not recommend TruFit synthetic implants for osteochondral patellar defects in active patients.

Soft tissue solitary fibrous tumor. Imaging findings in a series of nine cases.

OBJECTIVE: Few articles have been published regarding the imaging characteristics of soft tissue solitary fibrous tumors (SFT). The aim of this study is to describe the radiological features in a series of nine patients and to compare these results with pathological and clinical outcome. SUBJECTS AND METHODS: Nine cases of soft tissue SFT, confirmed by an experienced tumor pathologist, were studied with imaging techniques (US and MRI). Tumor location, size, morphology, local invasion, vascularity, MRI signal intensities, and dynamic contrast-enhancement patterns were recorded. Tumors were subclassified into cellular, fibrous, and giant cell forms histologically. RESULTS: The most common findings were a well-defined, polylobulated mass that tended to displace adjacent structures. The extremities were the most frequent site of presentation. The tumors showed high vascularity in all imaging studies. The radiological features that correlated better with malignant criteria were tumor size, heterogeneous signal intensity, and heterogeneous uptake of contrast on MRI. On dynamic contrast-enhanced (DCE) sequences, three tumors showed a biphasic curve (type IV) and one a progressive uptake curve (type II). The latter recurred twice after extensive local surgery and was classified as a giant cell variant. CONCLUSION: In this study a retrospective review of nine new cases of soft tissue SFT was carried out. The presence of a large solid, highly vascularized mass with a prominent vascular pedicle that displaces adjacent structures may suggest the diagnosis of an SFT. DCE sequences might help identify those tumors with worse clinical outcome, but a study with a larger series of patients is needed.

Generalized muscle pseudo-hypertrophy and stiffness associated with the myotilin Ser55Phe mutation: a novel myotilinopathy phenotype?

Myotilinopathies are a group of muscle disorders caused by mutations in the MYOT gene. It was first described in two families suffering from limb girdle muscle dystrophy type 1 (LGMD 1A), and later identified in a subset of dominant or sporadic patients suffering from myofibrillar myopathy, as well as in a family with spheroid body myopathy. Disease phenotypes associated with MYOT mutations are clinically heterogeneous and include pure LGMD forms as well as late-onset distal myopathies. We report here on a 53-year-old male suffering from a unique clinical profile characterized by generalized symmetrical increase in muscle bulk leading to a Herculean appearance. Muscle weakness and stiffness in the lower extremities were the patient's main complaints. Muscle MRI showed extensive fatty infiltration in the thigh and leg muscles and a muscle biopsy showed a myofibrillar myopathy with prominent protein aggregates. Gene sequencing revealed a Ser55Phe missense mutation in the myotilin gene. The mutation was identified in his older brother, who presented a mild hypertrophic appearance and had a myopathic pattern in EMG, despite not presenting any of the complaints of the proband and having normal muscle strength. This finding, and his deceased father and paternal aunt's similar gait disorders, suggest that this is in fact a new autosomal dominant kindred. The present observations further expand the spectrum of clinical manifestations associated with mutations in the myotilin gene.

No-reflow phenomenon in cardiac MRI: diagnosis and clinical implications.

OBJECTIVE: The purposes of this study were to depict the first-pass, delayed contrast enhancement and regional myocardial wall motion abnormalities of no-reflow phenomenon MRI and to review the major mechanisms and significance of this phenomenon in the clinical setting. CONCLUSION: Contrast-enhanced MRI is a useful noninvasive technique for determining the presence of microvascular obstruction. No-reflow phenomenon has important prognostic implications, and knowledge of the physiologic mechanism is important to understanding the distribution patterns of enhancement in correlation with the underlying pathologic process.

Lesions of the cardiophrenic space: findings at cross-sectional imaging.

Cross-sectional imaging techniques allow excellent visualization of the cardiophrenic space. Under normal conditions, the cardiophrenic space is occupied by fat, the amount of which is usually increased in overweight individuals. It has been suggested that this fat accumulation correlates with the risk of cardiovascular disease. Several alterations originating above or below the diaphragm can affect the cardiophrenic space. Inflammatory lesions such as pericardial fat necrosis and tumoral masses are sometimes seen. Lymphoma is a major but not exclusive cause of cardiophrenic adenopathy and must be differentiated from lymphatic seeding of supradiaphragmatic and infradiaphragmatic malignancies. In patients with portal hypertension, cardiophrenic space varices are not uncommon. Other masses or pseudomasses occurring in this region include pericardial cysts, mediastinal tumors, and diaphragmatic hernia. Computed tomography and magnetic resonance imaging of the thorax are helpful in characterizing cardiophrenic lesions initially identified at plain radiography. Such characterization helps narrow the differential diagnosis when lesions are detected in this location.