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In this study, we sought to determine the rate of progression of atherosclerosis using coronary calcium scores derived from electron beam tomography (EBT). We studied a variety of disease states (hypertension, high cholesterol, tobacco use, diabetes mellitus) followed for 1 to 6.5 years. We evaluated 299 asymptomatic persons (227 men and 72 women) who underwent 2 consecutive EBT scans at least 12 months apart. The average change in the calcium score (Agatston method) for the entire group was 33.2 +/- 9.2%/year. The treated group (receiving statins) demonstrated an average increase in calcium scores of 15 +/- 8%/year compared with 39 +/- 12%/year for untreated patients (p <0.001). Among the 60 patients on statin monotherapy, 37% had a decrease in the calcium score from baseline to follow-up scan. The relative increase in calcium scores did not vary significantly by gender or risk factors, with the exception of statin-treated hypercholesterolemic subjects. Scores of zero on the initial scan portend a low likelihood of significant calcific deposits on repeat scanning. Only 2 of 81 participants (2%) with scores of zero at baseline had scores >10 on repeat study. In this study, statin therapy induced a 61% reduction in the rate of coronary calcium progression. This study demonstrates that EBT may be a useful tool in assessing efficacy of different interventions to retard progression of atherosclerosis, noninvasively, over relatively short time periods. [ABSTRACT FROM AUTHOR]

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma characterized by bland histologic features and a paradoxically aggressive clinical course. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is a closely related tumor characterized by the presence of giant collagen rosettes. Only a single example of a metastasizing HSCT has been reported. A small subset of both LGFMS and HSCT display areas of increased cellularity and atypia which qualify as intermediate- to high-grade sarcoma; the significance of these features has not been definitively assessed. We present the clinicopathologic features of 77 cases of LGFMS and HSCT to determine the degree of overlap of these two lesions, their biologic behavior, and the significance of the occasional presence of intermediate- to high-grade sarcoma within both. The patients (33 female, 40 male) ranged from 3 to 78 years of age (median, 34 yrs). Fourteen cases occurred in patients less than 18 years of age. The tumors measured from 1 to 23 cm (median, 4.5 cm) and occurred predominantly in the trunk and lower extremities in both the deep (66 cases) and superficial (7 cases) soft tissues. In 15 cases, the tumor was present > 1 year before diagnosis. All tumors showed predominantly the typical hypocellularity and bland cytologic features of typical LGFMS; however, areas of hypercellularity and nuclear enlargement and hyperchromatism were identified in 12 of 73 (16%) and 7 of 73 (10%), respectively. Necrosis and mitotic activity >5/50 high-powered fields (HPF) were present in 6 of 73 (8%) and 5 of 73 (7%), respectively. Epithelioid areas were present in 33 of 73 (45%) and rosettes in 22 of 73 (30%). Follow up (54 cases; range, 2-192 mos; median, 24 mos; mean, 38 mos) showed 5 recurrences, 3 metastases, and 1 death. The diagnosis of LGFMS or HSCT was made prospectively in 51 patients; none had metastatic disease. Two of the metastatic tumors were LGFMS and one was a HSCT. LGFMS may occur more often in the pediatric population and show a much wider histologic spectrum than previously thought. A significant number of LGFMS possess inconspicuous collagen rosettes characteristic of HSCT, indicating that these two tumors are ends of a common spectrum rather than distinct entities. HSCT, like LGFMS, are low-grade sarcomas with metastatic potential. The presence of focal areas of intermediate- to high-grade sarcoma does not portend a worse outcome in the short term. The better prognosis reflected in this study compared with previous ones might reflect the fact that all were initially diagnosed as sarcomas and treated with aggressive surgery. The fact that the only three patients to develop metastatic disease were patients whose LGFMS or HSCT was identified retrospectively supports this concept.

Background: Quantitative determination of ejection fraction is predicated on precise measurement of end-diastolic and end-systolic volumes of the left ventricle. Contrast enhanced electron beam tomography (EBT), with excellent temporal and spatial resolution, has the potential for highly accurate measures of ejection fraction., Methods: EBT protocol used a short axis scan of the left ventricle (8-12 levels, apex to base) during infusion of iodinated contrast. To assess the accuracy of the measured left ventricular ejection fraction (LVEF), we compared EBT with first-pass radionuclide angiography (RNA) and cine angiography (CINE)., Results: A total of 41 patients (26 men and 15 women) underwent all three tests within 1 week. Resting ejection fraction using each modality was assessed in a linear regression model to assess inter-test correlation with the other two modalities. Correlation between CINE and EBT was high (r = 0.90, intercept 4.67, p < 0.001). Similarly, correlation of CINE and RNA (r = 0.87, intercept -5.48, p < 0.001) and between EBT and RNA (r = 0.87, intercept -4.6, p < 0.001) were high. In a subset of those patients with LVEF < or = 40%, correlation was consistently high between EBT and CINE. However, correlations were poor for the comparisons between RNA and CINE (r = 0.40), and between the RNA and EBT (r = 0.47). The mean differences of measured ejection fractions between each of the imaging modality were small. However, there was only modest agreement between each of the comparisons as measured using 95% confidence interval (CI) on Bland-Altman plots., Conclusion: These data indicate that the LVEF results are comparable among EBT, RNA, and CINE and can be used interchangeably to assess ventricular function for LVEF > 40%. For LVEF < or = 40%, we demonstrated some disparate results between cine angiography and RNA and between EBT and RNA, indicating that CINE or EBT may provide more accurate assessment.

We report the findings of 19 cases of a previously undescribed spindle cell tumor of soft tissues that resembles a low-grade fibromyxoid sarcoma but contains distinctive rosettelike structures. The tumors occurred principally as a painless, slowly growing, deeply situated mass of the proximal extremities in young to middle-aged adults (age range 14-65 years; mean 38). Although grossly circumscribed, the tumors had infiltrative borders microscopically and were composed of bland spindled cells situated in a hyalinized to myxoid stroma. The most characteristic feature of the tumor was scattered large rosettelike structures that often merged with serpinginous areas of dense hyalinization. The rosettes consisted of a central collagen core surrounded by a rim of rounded cells morphologically and immunophenotypically different from the cells of the spindled stroma. These cells expressed a number of antigens, including S-100 protein, neuron-specific enolase, and leu 22, in contrast to the stroma, which usually lacked these antigens. Of the 12 patients with available follow-up information, one patient treated with simple excision clinically developed local recurrence of the tumor 20 months after initial biopsy. No other recurrences were reported during the limited follow-up period, and no patient developed metastatic disease. However, the favorable prognosis of the patients in our series to date may relate to the limited follow-up period (approximately 3 years), as well as initial treatment by wide excision in nearly half of the patients. We regard the hyalinizing spindle cell tumor with giant rosettes as a distinctive type of low-grade fibroblastic tumor that with time may prove to behave similar to a low-grade fibromyxoid sarcoma and, hence, to represent an unusual variant thereof.

Scores from the Child Behavior Checklist (CBCL; Achenbach, 1991a) and the Client Development Evaluation Report (CDER; California Department of Developmental Services, 1980) for 67 children and adolescents with mental retardation were examined to evaluate the factorial validity of the instruments. Four factor analyses were conducted. The initial factor analysis of CBCL data failed to confirm the presence of the five first-order factors previously reported for the CBCL standardization sample (Achenbach, 1991b). Second, the higher-order factors of Externalizing and Internalizing behaviors, similar to the structure reported for the CBCL standardization sample (Achenbach, 1991b), were confirmed on the present sample. Third, the two CDER factors of Personal Maladaption and Social Maladaption, previously identified by Widaman, Gibbs, and Geary (1987), were also confirmed. Finally, a higher-order factor analysis of the two factor scores from the CBCL and two factor scores from the CDER was conducted to study the congruence between the CBCL Externalizing and CDER Social Maladaption dimensions, and between the CBCL Internalizing and CDER Personal Maladaption factors. Moderate levels of congruence were found. Next, child characteristics, including level of mental retardation, age, and four dimensions of adaptive behavior, were used as predictors of problem behavior. No child characteristics were significantly related to the CBCL Externalizing dimension, but child age and level of mental retardation were significant predictors of the CBCL Internalizing dimension. CDER Cognitive Competence predicted CDER Social Maladaption, and child age predicted CDER Personal Maladaption. The findings are discussed in relation to previous studies of problem behaviors of children and adolescents with mental retardation.

Objective: Our objective was to evaluate, over a 5-year period, the nonsurgical treatment of three patients with suprahepatic caval anastomotic stenosis who had undergone orthotopic liver transplantation., Conclusion: Balloon angioplasty is a safe and effective method for treating chronic caval stenosis occurring in patients who have undergone orthotopic liver transplantation. Caval stenosis identified in the early postoperative period, especially when such stenosis is associated with caval torsion or kinking, may be effectively treated by stent placement.

Laparoscopic surgery frequently requires tattooing of endoscopically identified sites for localization during surgery. Some tattooing agents cause serious tissue injury, which must be recognized in pathologic examination. Seven surgically resected colons were reviewed after injection with methylene blue or India ink at intervals of 1 day to 7 weeks before surgery. Early reactions to India ink included necrosis, edema, and neutrophilic infiltration in the submucosa and muscularis propria. Vessels were inflamed but without fibrinoid necrosis. Early reactions to methylene blue included ischemic ulceration, necrosis, and eosinophilic infiltration in the submucosa as well as fibrinoid necrosis of vessel walls. In the repair of methylene-blue injury, obliterative intimal fibrosis was seen in vessels. Such changes were absent in the colons injected with India ink. The India ink remained remained visible with the naked eye and microscopically 7 weeks after injection. Methylene blue was not grossly visible 7 days after injection, and only microscopic particles of pigment remained in widely scattered macrophages. In light of these findings, the amount of ink injected should be minimized and the injection site should be completely resected at surgery. Methylene blue is a poor tattoo agent, but its occasional use continues, and pathologists should recognize the resulting reaction.

A 28-year-old woman with prior neurosurgery involving the placement of a cadaveric dural graft developed a rapidly progressive neurodegenerative disorder with prominent cerebellar dysfunction that was proven at autopsy to be Creutzfeldt-Jakob disease. She represents the second American to develop Creutzfeldt-Jakob disease in association with a dural graft. The unusual features of the case include the patient's initial clinical presentation with cerebellar ataxia in the absence of dementia, the widespread presence of kuru-type amyloid plaques on a histological examination of the brain, the development of clinical symptoms during pregnancy, and the subsequent delivery of a child who remains healthy at the age of 3 years.

To investigate possible associations of nodular regenerative hyperplasia (NRH) with antitumor chemotherapy, we reviewed 72 partial hepatic resections (55 with metastatic tumor, 12 hepatocellular carcinomas, and five benign neoplasms). Thirty autopsy livers from adults without malignancies served as controls. Studies included hematoxylin and eosin, reticulin, and trichrome stains and immunostaining for proliferating cell nuclear antigen (PCNA). Five of 72 livers (7%) had NRH. All five patients had received chemotherapy, one by intrahepatic artery infusion. Four had received chemotherapy 2 months or less before undergoing partial hepatectomy. These five cases represented 15% of the 33 patients who received chemotherapy. No NRH was seen in autopsy control livers. In contrast to NRH, multiple hyperplastic foci were seen in 28 of 72 livers (39%). This finding did not correlate with chemotherapy. Two of 30 control livers (7%) showed similar mild regenerative changes. In only one case of NRH was PCNA staining increased over controls. A band of PCNA-positive hepatocytes was seen adjacent to the tumor in 21 cases, suggesting that the presence of tumor may cause a local increase in PCNA expression. Mitoses in hepatocytes and assessment of the thickness of liver cell plates were more sensitive indicators of regeneration than PCNA. Vascular changes, such as sinusoidal fibrosis (11 of 72 cases), thickened hepatic arterioles (13 of 72 cases), and mild thickening of central veins (10 of 72 cases), did not correlate with NRH, hyperplastic foci, or chemotherapy. No cases of hepatoportal sclerosis were identified.

A 41-year-old white man with facial pain and diplopia was found to have an invasive lesion of the clivus. The final pathological diagnosis was eosinophilic granuloma. The patient's symptoms resolved completely after transsphenoidal resection of the lesion. The pathological and radiological diagnosis and the treatment of solitary eosinophilic granulomas are discussed.

We report a case of an 18-month-old male, born to a woman with third trimester febrile illness, who had a history of congestive heart failure and respiratory distress, cardiomegaly, and electrocardiographic (ECG) findings suggestive of cardiomyopathy and myocarditis. After gradual improvement in heart size and function with pharmacologic therapy, he developed a terminal episode of respiratory distress and cardiogenic shock, with ECG findings of an anterolateral infarct. At autopsy it was found that endocardial fibroelastosis with mural thrombi in the left ventricle had been complicated by thromboembolism to the left anterior descending coronary artery, resulting in transmural infarction of the anteroseptal region of the left ventricle. Myocardial infarction is a potential but unusual thromboembolic complication of endocardial fibroelastosis. A high index of suspicion for coronary artery thromboemboli should be maintained in pediatric patients with cardiomyopathy and suspected myocardial infarction.