Spectrum of Fat-containing Soft-Tissue Masses at MR Imaging: The Common, the Uncommon, the Characteristic, and the Sometimes Confusing.

Fat-containing tumors are the most common soft-tissue tumors encountered clinically. The vast majority of fat-containing soft-tissue masses are benign. Lipomas are the most common benign fat-containing masses and demonstrate a characteristic appearance at magnetic resonance (MR) imaging. Less common benign soft-tissue masses include lipoblastoma, angiolipoma, spindle cell lipoma/pleomorphic lipoma, myolipoma, chondroid lipoma, lipomatosis of nerve, lipomatosis, hibernoma, and fat necrosis. Well-differentiated liposarcomas (WDLPSs)/atypical lipomatous tumors (ALTs) are locally aggressive soft-tissue masses that do not metastasize. Biologically more aggressive liposarcomas include myxoid, pleomorphic, and dedifferentiated liposarcomas. At MR imaging, lipomas typically resemble subcutaneous fat but may contain a few thin septa. The presence of thick, irregular, enhancing septa and nonfatty soft-tissue mass components suggests liposarcoma rather than lipoma. However, benign lipomatous lesions and WDLPS/ALT often have overlapping MR imaging findings. Distinguishing WDLPS/ALT from a benign lipomatous lesion or from fat necrosis at imaging can be challenging and often requires histologic evaluation. We present the spectrum of fat-containing masses, using the World Health Organization classification of adipocytic tumors, with an emphasis on commonly encountered lesions, characteristic MR imaging findings associated with specific tumors, and overlapping MR imaging findings of certain tumors that may require histologic sampling. We also briefly discuss the role of molecular markers in proper characterization and classification of fat-containing soft-tissue masses. (©)RSNA, 2016.

A comprehensive review of reported heritable noggin-associated syndromes and proposed clinical utility of one broadly inclusive diagnostic term: NOG-related-symphalangism spectrum disorder (NOG-SSD).

The NOG gene encodes noggin, a secreted polypeptide that is important for regulating multiple signaling pathways during human development, particularly in cartilage and bone. The hallmark of NOG-related syndromes is proximal symphalangism, defined by abnormal fusion of the proximal interphalangeal joints of the hands and feet. Many additional features secondary to NOG mutations are commonly but inconsistently observed, including a characteristic facies with a hemicylindrical nose, congenital conductive hearing loss due to stapes fixation, and hyperopia. The variable clinical presentations led to the designation of five different autosomal dominant syndromes, all subsequently found to have resulted from NOG mutations. These include (1) proximal symphalangism; (2) multiple synostoses syndrome 1; (3) stapes ankylosis with broad thumbs and toes; (4) tarsal-carpal coalition syndrome; and (5) brachydactyly type B2. Herein, we review the phenotypic features associated with mutations in the NOG gene, demonstrating the overlapping characteristics of these syndromes. Due to the variable phenotypic spectrum within families and among families with the same mutation, we propose a unifying term, NOG-related symphalangism spectrum disorder (NOG-SSD), to aid in the clinical recognition and evaluation of all affected individuals with these phenotypes. These NOG gene variants are available in a new locus-specific database (https://NOG.lovd.nl).

Trigeminocardiac reflex during the percutaneous injection of ethylene vinyl alcohol copolymer (Onyx) into a juvenile nasopharyngeal angiofibroma: a report of two cases.

The trigeminocardiac reflex (TCR) is a rare but well-described phenomenon encountered during invasive procedures involving the face, orbit, and cranial base. The reflex is characterized by the abrupt onset of hypotension, bradycardia, asystole, and dysrhythmias. With temporary cessation of the surgical procedure, vital signs typically stabilize without the need for further investigation, though anticholinergic drugs are often used to prevent prolonged hypotension and bradycardia. Two separate cases of the TCR were encountered during the percutaneous embolization of a juvenile nasopharyngeal angiofibroma with dimethylsulfoxide (DMSO) before the injection of ethylene vinyl alcohol copolymer (Onyx, ev3, Irvine, California, USA). In both cases, the injection of DMSO precipitated approximately 30 s of bradycardia/asystole, which then resolved after halting the procedure and administering anticholinergic drugs. There were no additional occurrences afterward and the patients underwent tumor excision with good recovery.

Potentially inappropriate medication utilization in the emergency department visits by older adults: analysis from a nationally representative sample.

OBJECTIVES: The objectives were to determine the frequency of administration of potentially inappropriate medications (PIMs) to older emergency department (ED) patients and to examine recent trends in the rates of PIM usage. METHODS: The data examined during the study were obtained from the National Hospital Ambulatory Medical Care Survey (NHAMCS). This study utilized the nationally representative ED data from 2000-2006 NHAMCS surveys. Our sample included older adults (age 65 years and greater) who were treated in the ED and discharged home. Estimated frequencies of PIM-associated ED visits were calculated. A multivariable logistic regression model was created to assess demographic, clinical, and hospital factors associated with PIM administration and to assess temporal trends. RESULTS: Approximately 19.5 million patients, or 16.8% (95% confidence interval [CI]=16.1% to 17.4%) of eligible ED visits, were associated with one or more PIMs. The five most common PIMs were promethazine, ketorolac, propoxyphene, meperidine, and diphenhydramine. The total number of medications prescribed or administered during the ED visit was most strongly associated with PIM use. Other covariates associated with PIM use included rural location outside of the Northeast, being seen by a staff physician only (and not by a resident or intern), presenting with an injury, and the combination of female sex and age 65-74 years. There was a small but significant decrease in the proportion of visits associated with a PIM over the study period. CONCLUSIONS: Potentially inappropriate medication administration in the ED remains common. Given rising concerns about preventable complications of medical care, this area may be of high priority for intervention. Substantial regional and hospital type (teaching versus nonteaching) variability appears to exist.