Gray-scale and color Doppler ultrasonographic manifestations of papillary thyroid carcinoma: analysis of 51 cases

The purpose of this study is to assess the specific ultrasonic characteristics of papillary thyroid carcinoma and to determine the relative frequency of various patterns of papillary carcinoma on gray-scale ultrasonography (US) and color Doppler ultrasonography (CDU).

Methods

We retrospectively reviewed US features in 51 patients with confirmed papillary thyroid carcinoma. The features were analyzed based on tumor size, echogenicity, echotexture, boundary, margin, shape, and calcification pattern on gray-scale US imaging, and on patterns of vascularity on CDU. We obtained the relative frequency of features and classified these features into three categories: common (≥50% of lesions), less common (>10% but <50%), and uncommon (≤10%). Individual differences and combinations of features were also analyzed.

Results

In total, 67 nodules were enrolled in our study. The sizes of 76% of nodular lesions were <20.0 mm. Common US features of papillary carcinoma included: a homogeneous hypoechoic solid picture; a poorly defined boundary; an irregular margin; the absence of halo; the absence of calcifications or microcalcifications; and mixed perinodular and intranodular blood flow patterns. Less common features included: a heterogeneous hypoechoic or very hypoechoic picture; microcalcifications; a well-defined boundary; a regular margin; a halo with uneven thickness or an incomplete halo; and a taller-than-wide shape. Uncommon features included: an isoechoic picture; solid with cystic components; coarse calcifications; mixed coarse calcifications and microcalcifications; “inferno”-type blood flow; and absence of blood flow. On average, each nodule had 4.9 US features considered common, 1.8 US features considered less common, and 0.4 US feature considered uncommon. Features such as predominantly cystic composition, hyperechoic texture, and hypoechoic halo with even thickness were never found in our study. The top two common manifestations of papillary carcinoma were solid architecture and mixed perinodular and intranodular blood flow signals.

Conclusion

All lesions in our series had a predominantly solid characteristic on gray-scale US.     

Brain MR Imaging Findings of Cardiac-Type Fabry Disease with an IVS4+919G>A Mutation

BACKGROUND AND PURPOSE:A high incidence of cardiac-type Fabry disease with an α-galactosidase A mutation, IVS4 + 919 G>A, has been identified in the Taiwanese population. The neurologic manifestation has not been understood in this specific cardiac variant. This study aimed to investigate the typical imaging features of classic Fabry disease in patients with IVS4 Fabry disease.MATERIALS AND METHODS:Twenty-six patients with IVS4-type Fabry disease (20 men and 6 women; age range, 43–71 years; median age, 61 years) and 26 age- and sex-matched healthy controls (age range, 44–68 years; median age, 60 years) were analyzed for white matter hyperintensities, the pulvinar sign, and basilar artery diameter. The volumes of white matter hyperintensities were calculated by comparison with an in-house data base of 276 controls.RESULTS:Infarctions were found in 9 patients with IVS4 Fabry disease (35%) and in none of the healthy controls (P = .001). A pulvinar sign was found in 8 patients with IVS4 Fabry disease (30%) and in none of the healthy controls (P = .002). No significant difference was found in Fazekas scale scores for white matter hyperintensities; however, white matter hyperintensity volume in the deep white matter was higher in patients with IVS4 Fabry disease than in those from the healthy control data base (P = .004).CONCLUSIONS:Along with its involvement of the cardiac system, IVS4-type Fabry disease has features similar to those of classic Fabry disease and a higher frequency of deep white matter hyperintensities and a higher incidence of infarctions and pulvinar signs than in healthy controls.

Classic Fabry disease is a multisystem X-linked lysosomal disorder due to lysosomal α-galactosidase A (GLA) deficiency, which subsequently leads to accumulation of glycosphingolipids, primarily globotriaosylceramide, throughout the body.¹ The disease results in severe renal, cardiac, and central nervous system complications in adulthood. On brain MR imaging, classic Fabry disease is characterized by white matter hyperintensities, infarcts, and dolichoectasia.²In the general population, the incidence of Fabry disease has been reported as 1 in 40,000–117,000 live births. However, our previous studies by using neonate screening had identified a high incidence (approximately 1 in 1600 males) of a cardiac variant resulting from a GLA mutation, IVS4 + 919G>A (IVS4-type).^3,4 Another study revealed that 12 of 10,499 males (1/875) and 24 of the 9564 females (1/399) had the IVS4 + 919G>A mutation in neonate screening.⁵ The natural course of the IVS4-type Fabry disease is still largely unknown. The intronic mutation (IVS4 + 919G>A) was reported to be a “cardiac-type” Fabry mutation,⁶ which may present with asymptomatic, mild symptomatic as microalbuminuria and retinal vessel tortuosity, to severe cardiac symptoms causing significant morbidity after the fifth decade of life. However, the neurologic symptoms in the specific subtype have never been understood. Therefore, the current study aimed to analyze the degrees of CNS involvement in IVS4-type Fabry disease by retrospectively comparing brain imaging results of this patient population with images from a healthy control data base.     

Valsalva sinus aneurysm of the non-coronary cuspid presenting as cyclops sign on multi-detector computed tomography

An 8-year-old girl with Williams syndrome was found to have a heart murmur. Cardiac CT demonstrated a Valsalva sinus aneurysm (VSA) of the non-coronary cuspid, with left atrium (LA) indentation, resembling the face of Cyclops on a coronal reformatted image. Williams syndrome is related to some congenital disorders with interruption of the tunica media from the aortic root to the annular fibrous ring or aortic valve. However, it rarely presents at birth. The sinus of Valsalva dilates as time goes by, due to the persistent striking force from the left ventricle (LV). However, it is silent until rupture when cardiac tamponade occurs. The typical imaging appearance of VSA is of a saccular shape and originates above the aortic root, but sometimes involves the entire sinus. Although lethal complications of VSA occur without warning, prophylactic surgical intervention remains controversial. Regular imaging follow-up is advised before complications occur.     

Algorithmic approaches to the diagnosis of gallbladder intraluminal lesions on ultrasonography

Ultrasound is a frequently used diagnostic tool for gallbladder diseases. Polypoid lesions are commonly depicted at routine abdominal ultrasonography (US). The characteristics of these lesions vary. Since most early malignant tumors in the gallbladder are asymptomatic, differentiation between malignancy and benignity is crucial. Knowledge of gallbladder polypoid lesions is important so that they can be appropriately included in the differential diagnosis in patients presenting with intra-gallbladder nodules on US. This article summarizes the algorithmic approach to the diagnosis of these lesions and our recent experience with contrast-enhanced US. The clinical and imaging features of gallbladder polypoid lesions are reviewed.     

Evaluation of noncytotoxic DNMT1-depleting therapy in patients with myelodysplastic syndromes

BACKGROUND. Mutational inactivation in cancer of key apoptotic pathway components, such as TP53/p53, undermines cytotoxic therapies that aim to increase apoptosis. Accordingly, TP53 mutations are reproducibly associated with poor treatment outcomes. Moreover, cytotoxic treatments destroy normal stem cells with intact p53 systems, a problem especially for myeloid neoplasms, as these cells reverse the low blood counts that cause morbidity and death. Preclinical studies suggest that noncytotoxic concentrations of the DNA methyltransferase 1 (DNMT1) inhibitor decitabine produce p53-independent cell-cycle exits by reversing aberrant epigenetic repression of proliferation-terminating (MYC-antagonizing) differentiation genes in cancer cells.METHODS. In this clinical trial, patients with myelodysplastic syndrome (n = 25) received reduced decitabine dosages (0.1–0.2 mg/kg/day compared with the FDA-approved 20–45 mg/m²/day dosage, a 75%–90% reduction) to avoid cytotoxicity. These well-tolerated doses were frequently administered 1–3 days per week, instead of pulse cycled for 3 to 5 days over a 4- to 6-week period, to increase the probability that cancer S-phase entries would coincide with drug exposure, which is required for S-phase–dependent DNMT1 depletion.RESULTS. The median subject age was 73 years (range, 46–85 years), 9 subjects had relapsed disease or were refractory to 5-azacytidine and/or lenalidomide, and 3 had received intensive chemoradiation to treat other cancers. Adverse events were related to neutropenia present at baseline: neutropenic fever (13 of 25 subjects) and septic death (1 of 25 subjects). Blood count improvements meeting the International Working Group criteria for response occurred in 11 of 25 (44%) subjects and were highly durable. Treatment-induced freedom from transfusion lasted a median of 1,025 days (range, 186–1,152 days; 3 ongoing), and 20% of subjects were treated for more than 3 years. Mutations and/or deletions of key apoptosis genes were frequent (present in 55% of responders and in 36% of nonresponders). Noncytotoxic DNMT1 depletion was confirmed by serial BM γ-H2AX (DNA repair/damage marker) and DNMT1 analyses. MYC master oncoprotein levels were markedly decreased.CONCLUSION. Decitabine regimens can be redesigned to minimize cytotoxicity and increase exposure time for DNMT1 depletion, to safely and effectively circumvent mutational apoptotic defects.TRIAL REGISTRATION. Clinicaltrials.gov {"type":"clinical-trial","attrs":{"text":"NCT01165996","term_id":"NCT01165996"}}NCT01165996.FUNDING. NIH (R01CA138858, {"type":"entrez-nucleotide","attrs":{"text":"CA043703","term_id":"24344623","term_text":"CA043703"}}CA043703); Department of Defense (PR081404); Clinical and Translational Science Award (CTSA) (UL1RR024989); and the Leukemia and Lymphoma Society (Translational Research Program).     

Feasibility of Robotic Laparoendoscopic Single-Site Partial Nephrectomy for Renal Tumors >4 cm

Background

Laparoendoscopic single-site (LESS) urologic procedures have gained significant interest worldwide in an attempt to further reduce morbidity and minimize scarring associated with conventional laparoscopic surgery. The robotic technology has overcome some of the limitations of manual single-incision surgery relating to lack of triangulation, instrument collision, and surgical exposure. There are no data on robotic LESS partial nephrectomy (PN) for renal tumors >4 cm.

Objectives

To evaluate the feasibility of robotic LESS PN for renal tumors >4 cm.

Design, setting, and participants

Data from 67 consecutive patients who underwent robotic LESS PN were collected between May 2009 to January 2011.

Outcome measurements and statistical analysis

Patients were stratified into two groups: 20 patients with renal tumors >4 cm (group 1) and 47 patients with renal tumors ≤4 cm (group 2). Perioperative data were recorded and comparisons between the two groups were analyzed using the Mann-Whitney U test for continuous variables and Fisher exact test for categorical variables.

Results and limitations

No statistically significant differences were found between the two groups in demographic information, operative complications, pathologic characteristics, mean decline in estimated glomerular filtration rate, estimated blood loss, operative times, conversion rate, or positive surgical margins. However, group 1 had a higher mean nephrometry score (p < 0.01), longer warm ischemia time (p = 0.007), and longer length of stay (p = 0.046). Its retrospective design and being conducted at a single center were the main limitations of this study.

Conclusions

This study demonstrated the feasibility and safety of robotic LESS PN for tumors >4 cm. Patients with tumors >4 cm had a statistically significant, higher mean nephrometry score, longer warm ischemia time, and longer length of stay, but there was no increased risk of adverse outcomes. A long-term study is needed to confirm the durable renal preservation and oncologic outcomes for patients with larger tumor burden.     

Ibutilide to expedite ED therapy for recent-onset atrial fibrillation flutter

OBJECTIVE: Ibutilide is a type III antiarrhythmic agent approved for the pharmacologic conversion of atrial fibrillation (AF) and atrial flutter (AFl). Previous studies conducted outside the ED setting have demonstrated conversion rates of 60% to 80%. This response has been highest in patients with recent-onset AF-AFl. These observations and the 4-hour half-life of ibutilide suggest that it may be an excellent drug with which to treat AF-AFl in the ED. The purpose of the study was to examine the efficacy and safety of ibutilide in terminating AF-AFl in patients who present to the ED with symptoms of less than 3 days' duration, neither angina nor heart failure, and no comorbid conditions that require admission. METHODS: Among 36 enrolled patients, the admission electrocardiogram demonstrated AF in 26 and AFl in 10. Ibutilide 1 mg was administered intravenously for 10 minutes. If sinus rhythm was not present 10 minutes after the infusion concluded, a second infusion of 1 mg was given. Successful conversion was defined as restoration of sinus rhythm within 1 hour after the last dose of ibutilide. RESULTS: Sixteen (61.5%) of 26 patients with AF and 9 (90%) of 10 patients with AFl converted to sinus rhythm (overall conversion rate=69%). The mean time to arrhythmia termination was 19+/-9 minutes. The mean stay in the ED was 16.2 hours. No significant complications occurred. CONCLUSION: We conclude that ibutilide is an excellent therapy option for restoring sinus rhythm in the ED. Its use may obviate the need for admission, avoid the risks and inconveniences of general anesthesia to perform electrical cardioversion, and reduce the ED length of stay in selected patients with recent-onset atrial arrhythmias.