[Comparison of radiological and clinicopathological features of craniofacial fibro-osseous lesions].

Objective: To investigate the clinicopathological and radiological features of benign fibro-osseous lesion (BFOL). Methods: Sixty-five cases of craniofacial BFOL, eight cases of peripheral ossifying fibroma (POF) and one case of low-grade central osteosarcoma diagnosed at Sichuan Provincial People's Hospital between January 2010 and March 2019 were collected. The clinicopathologic features, hematoxylin-eosin and immunohistochemical (IHC) staining and radiographic features were analyzed. MDM2 gene amplification was detected by FISH in difficult borderline cases. Results: This cohort of BFOLs included 50 cases of fibrous dysplasia (FD), 12 cases of ossifying fibroma (OF), and three cases of juvenile psammomatoid ossifying fibroma (JPOF). The average ages of patients with FD,OF and JPOF were 31.7, 39.2 and 26.0 years respectively. The male to female ratio was 1.0∶1.8.The average age of POF was 47.0 years, with male to female ratio of 1∶7. Patient of low-grade central osteosarcoma was a 48-year-old man. Twenty-seven cases of FD were located in the jaw, and 23 cases were in other craniofacial bones. Nine cases of OF were located in the jaw, and three cases were in the nasal cavity. Two cases of JPOF were in the nasal sinus, and one was in the jaw. All POF were located in the gingiva, and low-grade central osteosarcoma was located in the mandible. The imaging features of FD were luffa-like or ground-glass like signal shadows with poorly defined borders with expansion. OF had clear borders or sclerosing margins. Both JOF and low-grade central osteosarcoma were expansile intraosseously and with focally invasive nodular masses with ground-glass like signal shadows; and POF showed soft tissue mass with bone formation. Histological features of BFOLs showed mixed fibrous and irregular osteoid lesions. FD had no clear relationship with the host bone and no osteoblasts surrounded the bone trabeculae. Osteoblasts rimming was found in OF, and the boundaries of the host bone were clear. JPOF and low-grade central osteosarcoma infiltrated the host bone focally, and the latter showed mild cellular atypia. MDM2 amplification was detected in low-grade central osteosarcoma. Conclusions: BFOLs are a group of fibro-osseous lesions with similar morphology in the head and neck and face, but their clinical features and prognosis are different; and their imaging and histological characteristics are also slightly different. Attentions should be given to the combination of clinical, imaging and pathologic features of BFOLs, especially the differential diagnosis between BFOLs and low-grade central osteosarcoma. Molecular detection could be used to assist the diagnosis in difficult cases.

When to sweat: A history of chemotherapy in malignant sweat gland tumors. A unique case report and literature review.

Sarcomatoid sweat gland carcinomas are rare among cutaneous cancers, with less than 20 cases described. A 54-year-old woman with sarcomatoid sweat gland carcinoma of the right upper extremity suffered extensive recurrence at 15 months, unresponsive to chemotherapy. There is no standard treatment or chemotherapy regimens for metastatic sweat gland carcinoma.

Predicting adverse outcomes of cardiac surgery with the application of artificial neural networks.

Risk-stratification models based on pre-operative patient and disease characteristics are useful for providing individual patients with an insight into the potential risk of complications and mortality, for aiding the clinical decision for surgery vs non-surgical therapy, and for comparing the quality of care between different surgeons or hospitals. Our study aimed to apply artificial neural networks (ANN) models to predict mortality and morbidity after cardiac surgery, and also to compare the efficacy of this model to that of the logistic regression model and Parsonnet score. The accuracy of the ANN, logistic regression and Parsonnet score in predicting mortality was 83.8%, 87.9% and 78.4%. The accuracy of the ANN, logistic regression and Parsonnet score in predicting major morbidity was 79.0%, 74.3% and 68.6%. The area under the receiver operating characteristic curves (AUC) of the ANN, logistic regression and Parsonnet score in predicting in-hospital mortality were 0.873, 0.852 and 0.829. The AUCs of the ANN, logistic regression and Parsonnet score in predicting major morbidity were 0.852, 0.789 and 0.727. The results showed the ANN models have the best discriminating power in predicting in-hospital mortality and morbidity among these models.

Hydrophobic surfactant treatment prevents atherosclerosis in the rabbit.

The hypocholesterolemic effect of the hydrophobic surfactant, poloxalene 2930, was studied in the rabbit to determine whether this agent prevents experimentally produced atherosclerosis. Male rabbits were divided into four groups and fed a control diet (group A) or an atherogenic diet (groups B, C, and D) for 10 wk. Diets of groups C and D were supplemented with 0.5 and 1% poloxalene 2930, respectively. Animals in group B developed significantly greater levels of cholesterol in the serum and aorta compared with group A. Addition of poloxalene 2930 to the diets of groups C and D prevented significant elevations in cholesterol concentrations of both serum and aorta compared with group B with values for group D being essentially similar to those observed in group A. Groups C and D also had significant increases of fecal excretion of both neutral fat and neutral steroids as compared with either groups A or B. There were no atherosclerotic lesions of the aortas from group D. Aortas from rabbits in group B had numerous atheromatous plaques while one rabbit each from groups A and C had several very small atheromatous lesions. These results demonstrate that poloxalene 2930 reduces the rise of serum cholesterol in rabbits in response to an atherogenic diet and prevents the development of atherosclerosis. This hypocholesterolemic effect is likely mediated by the effect of this surfactant on the small intestine.

Relative specificities of inhibition of acid cholesteryl ester hydrolase and neutral cholesteryl ester hydrolase in cultured rabbit aortic smooth muscle cells by esterastin and cholesteryl oleyl ether.

Rabbit aortic smooth muscle cells in culture were incubated with 0.04-500 M esterastin. Acid cholesteryl ester hydrolase (ACEH) and neutral cholesteryl ester hydrolase (NCEH) activities were inhibited to a comparable degree, with 50% inhibition occurring in the range of 0.4 M esterastin. Cells incubated with cholesteryl oleyl ether showed 50% inhibition of NCEH at 5.0 M, but no inhibition of ACEH over a concentration range of 0.2-20 M. This relative specificity of cholesteryl oleyl ether for NCEH can be employed to study the relative roles of ACEH vs. NCEH in preventing cellular cholesteryl ester accumulation.

Cytotoxicity of oxidation derivatives of cholesterol on cultured aortic smooth muscle cells and their effect on cholesterol biosynthesis.

Aortic smooth muscle cell death is an important initial lesion of atherosclerosis. A number of autooxidation products of cholesterol which has been recognized recently has the capability of inducing rabbits' aortic smooth cell death in vitro. Twelve oxidation derivatives of cholesterol, available commercially, were dissolved in small amounts of ethanol, then added to the culture medium at levels not exceeding 0.8%. The medium contained 10% fetal calf's serum which served as an in situ vehicle for the sterols. The degrees of cytotoxicity were graded and measured as percentage of dying and dead cells in the cultures within 24 hr. 25-Hydroxycholesterol and cholesthan-3 beta, 5 alpha, 6 beta-triol, were the most toxic compounds among the sterols tested. When these oxidation derivatives of cholesterol were added to these cultured cells, they significantly depressed activity of 3-hydroxy-3-methylglutaryl coenzyme A reductase, a regulatory enzyme of cholesterol biosynthesis (up to 83% inhibition by 25 hydroxycholesterol at a 3 microgram/ml concentration in culture medium) but the sequence of degree of inhibition was not exactly correlated with that of cytotoxicity. Various mechanisms are speculated. Purified cholesterol showed no cytotoxic effect and minimal inhibition of cholesterol biosynthesis.

Poloxalene 2930, a hydrophobic surfactant that prevents atherosclerosis, alters composition of rabbit lipoproteins.

Poloxalene 2930, a hydrophobic surfactant, was incorporated into an atherogenic diet at dose levels, 0.5% and 1% of the diet, and fed to rabbits for 10 weeks. Another group received plain atherogenic diet alone and a control group was fed chow. After this period, serum lipoproteins were separated by centrifugation and analyzed. The composition of lipoproteins from rabbits on atherogenic diet was abnormal. The cholesterol: triglyceride ratio of every lipoprotein fraction was significantly increased as was the cholesterol: protein ratio of very low density lipoproteins. Supplementing the diet with Poloxalene 2930 prevented these alterations. In all groups on the atherogenic diet the percentage of apolipoprotein E in VLDL and HDL increased. In the case of Poloxalene-treated rabbits this developed even though serum cholesterol levels were normal or slightly increased. It is concluded that Poloxalene 2930 has a systemic effect on lipoproteins which may contribute to its antiatherogenic action.

Transport of cholesterol autoxidation products in rabbit lipoproteins.

Radiolabeled pure [4-14C]cholesterol was kept at 60 degrees C under air to autoxidize for 5 weeks, after which approximately 12% cholesterol oxidation products were formed. The mixture, suspended in gelatin, was given to rabbits by gastric gavage. Rabbits were killed 4, 24, and 48 h after treatment. Cholesterol and its autoxidation products were separated by thin-layer chromatography into 5 fractions and radioactivities of each fraction were measured. Percentages of each fraction of cholesterol oxidation products and cholesterol in the original mixture before administration and in the rabbit sera after administration were similar, suggesting that the rates of absorption of cholesterol oxidation products are not significantly different from that of cholesterol. Lipoproteins were fractionated by ultracentrifugation into VLDL, LDL and HDL. Radioactivities of each fraction in lipoproteins separated by thin layer chromatography showed that fractions containing cholestane-3 beta,5 alpha,6 beta-triol, 7 alpha- and 7 beta-hydroxycholesterol and 7-ketocholesterol were more selectively transported in VLDL, whereas most of the 25-hydroxycholesterol was present in LDL. HDL contained only minute amounts of cholesterol oxidation products.

Distribution of 25-hydroxycholesterol in plasma lipoproteins and its role in atherogenesis. A study in squirrel monkeys.

Oxidation products of cholesterol have been shown to be potent inhibitors of cholesterol biosynthesis and also highly toxic to cultured aortic smooth muscle cells. In rabbit experiments, these compounds produced arterial injury resulting in arteriosclerosis. Purified cholesterol only minimally inhibited cholesterol biosynthesis and had no effect on cultured aortic smooth muscle cells. This raises the possibility that plasma lipoproteins containing beta-apoprotein (i.e. LDL and VLDL), which are considered to be atherogenic, may carry more oxidation products than HDL which is not atherogenic [3H]25-hydroxycholesterol and [14C]cholesterol were given only orally to 10 squirrel monkeys (Saimiri sciureus) and blood samples were collected via femoral puncture 24 h after administration. Lipoproteins were separated by ultracentrifugation and the radioactivity in each fraction was counted. Results show that the distribution of labeled cholesterol in VLDL, LDL, and HDL was almost identical to that of unlabeled cholesterol. Most of the radio-activity of 25-hydroxycholesterol was located in LDL & VLDL (55.1% and 34.7%, respectively), only 10.2% was present in HDL. If the radioactivity of 25-hydroxycholesterol were calculated on the basis of the apoprotein content of the lipoprotein micelle, the relative capacity of VLDL and LDL to carry 25-hydroxycholesterol was even greater and more significant than that of HDL (90 X and 42 X, respectively).

Cholesterol oxidation derivatives and arterial endothelial damage.

Three groups of New Zealand male while rabbits were given either 2.5 mg/kg of 25-hydroxycholesterol, cholestane-3 beta, 5 alpha, 6 beta-triol or vehicle only, intravenously. 24 h after treatment, the luminal surfaces of aortae of rabbits receiving 25-hydroxycholesterol were examined by scanning electron microscopy (SEM) and showed numerous balloon-like protrusions and crater-like defects as well as circulating, formed elements adhering on the luminal surface. The luminal surface of aortae of rabbits given cholestane-3 beta, 5 alpha, 6 beta-triol had similar but more frequent lesions when compared with those of the 25-hydroxycholesterol group. Microthrombi were occasionally found. The aortae of the control group had significantly fewer lesions. Transmission electron-microscopic studies showed intracytoplasmic vacuoles and diffuse subendothelial edema in the aortae of the two groups receiving the oxidation derivatives of cholesterol. The balloon-like protrusions and crater-like defects observed by SEM appeared to represent the initial sterol-induced endothelial cell injury. Repeated episodes of arterial injury followed by thrombus formation could eventually lead to atherosclerosis.

Effects of cholesterol oxides on LDL receptors in cultured rabbit aortic smooth muscle cells.

The efficiency of receptor-mediated low-density lipoprotein (LDL) uptake and degradation has been implicated in the development of atherosclerosis. It has previously been shown that cholesterol oxides have more effect on the feedback control of cholesterol biosynthesis than does cholesterol per se on a molecule-for-molecule basis. Cholesterol oxides may also modify the expression or function of the LDL receptors, resulting in alteration in cholesterol homeostasis. Rabbit aortic smooth muscle cells were preincubated in 5% lipoprotein-deficient medium for 24 hours and then incubated with 1 or 5 µg/ml purified cholesterol or cholesterol oxides, including 25-hydroxycholesterol, 7-ketocholesterol, cholestane-3ß,5α,6ß-triol, and cholesterol 5α, 6α-epoxide for 12 to 24 hours. The uptake of (125)I-LDL was significantly suppressed in a dose-dependent fashion to 67% of the control value by 25-hydroxycholesterol at 1 µg/ml and to 53% at 5 µg/ml after the 24-hour incubation period. Other cholesterol oxides also had inhibitory effects on the LDL uptake at 5 µg/ml. At these concentrations cholesterol oxides additionally inhibited cellular degradation of LDL and the fractional turnover rate of LDL was prolonged.

Disseminated coccidioidomycosis with rapid progression to effusive-constrictive pericarditis.

This case reports coccidiomycosis presenting as pericarditis with tamponade rapidly progressing to effusive-constrictive pericarditis and death over 72 hours. Coccidiomycosis pericarditis is a rapidly progressing disease requiring early and complete pericardiectomy to avoid the hemodynamics of constriction. We illustrate the use of echocardiography in this case and demonstrate the histopathology. We review the literature and discuss therapy and management. Coccidiomycosis is often clinically unsuspected and unrecognized by the health care worker unfamiliar with the disease process.

Central nervous system vasculitis in cytomegalovirus infection.

A 51-year-old man received cyclophosphamide, vincristine, procarbazine and prednisone in the treatment of a small-cell undifferentiated lymphoma. Two years later, he developed a rapidly progressive neurological syndrome characterized by a decline in alertness, deafness, blindness and paraplegia. Examination of his eyes revealed severe hemorrhagic chorioretinitis. Leg weakness was thought to be due to transverse myelopathy at a thoracic level. He had a grand mal convulsion and died from terminal bronchopneumonia. Autopsy examination of the eyes revealed sweeping destruction of the retina due to inclusion body chorioretinitis. The brain and spinal cord showed multiple small infarcts accounting for the deafness and paraplegia. The lesions were due to occlusive arteritis in gray and white matter. Veins were also involved. Tissue surrounding the foci of necrosis contained cells with intranuclear an intracytoplasmic inclusion bodies. Some of the Cowdry type A inclusion bodies were large, measuring 30 micrometer in diameter and were located in enlarged cells. Electron microscopy of retina and brain tissue disclosed virus particles compatible with cytomegalovirus. The subject of cerebral and ocular angiitis due to herpes virus infections is reviewed.

Effects of cholesterol oxidation derivatives on cholesterol esterifying and cholesteryl ester hydrolytic enzyme activity of cultured rabbit aortic smooth muscle cells.

The effects of 5 micrograms/ml of 25-hydroxycholesterol; cholestane-3 beta,5 alpha,6 beta-triol; and cholesterol on acyl CoA cholesterol acyltransferase, acid cholesteryl ester hydrolase and neutral cholesteryl ester hydrolase was studied in cultured rabbit aortic smooth muscle cells. After 1 hour incubation, 25-hydroxycholesterol resulted in a fourfold stimulation of acyl CoA cholesterol acyltransferase activity. No stimulation by 25-hydroxycholesterol was noted before 15 minutes or after 5 hours of incubation. Neither cholestane-3 beta,5 alpha,6 beta-triol nor cholesterol influenced acyl CoA cholesterol acyltransferase activity at any time interval. No significant effects of any of the sterols were noted on acid cholesteryl ester hydrolase or neutral cholesteryl ester hydrolase activity. The imbalance between acyl CoA cholesterol acyl transferase and hydrolase activities induced by 25-hydroxycholesterol could result in cholesteryl ester accumulation by arterial smooth muscle cells, which may be associated with atherosclerosis.

Morphological changes in small vessels on endomyocardial biopsy.

Small vessel disease has been described in various cardiac conditions including diabetes mellitus, amyloidosis, and connective tissue disease. Less well understood is the incidence and morphological features of small vessel disease in patients with myocardial disease of unknown etiology. This study examines the incidence, clinical presentation, and pathological changes of small vessel disease in patients with normal epicardial coronary arteries undergoing endomyocardial biopsy. Biopsy specimens in 110 consecutive patients were analyzed by light and electron microscopy. Small vessel abnormalities were present in 16 patients (14.6 percent) of whom five patients had associated hypertension and 11 patients had idiopathic small vessel disease. There were six males and 10 females with a mean age of 53 (26 to 76) years. Clinical presentations were arrhythmias, heart failure, or chest pain. The left ventricular ejection fraction was reduced (less than 50 percent) in 12 of these 16 patients. The morphological features of small vessel disease included marked thickening of the arterial wall owing to subendothelial deposits of heterogeneous electron dense materials consisting of microfibrils, collagen and elastic fibers, cellular debris, and other amorphous substances. Subendothelial deposits comprised a mean 60 percent (40 to 76 percent) of the arterial wall thickness.

The role of cholesterol oxidation products in the pathogenesis of atherosclerosis.

Cholesterol undergoes spontaneous autoxidation, leading to the production of potentially atherogenic oxidation derivatives. When 25-hydroxycholesterol (25-OH) or cholestane-3 beta, 5 alpha, 6 beta-triol (triol) was injected intravenously into rabbits, the aortic surfaces showed numerous balloon-like protrusions and crater-like defects indicative of endothelial damage. Alterations in membrane function caused by these cholesterol oxides could be the mechanism for their cytotoxic effect. Carrier-mediated hexose transport by cultured rabbit aortic smooth muscle cells, measured using 2-deoxyglucose, was reversibly inhibited by triol within one hour. A membrane-bound enzyme, 5'-nucleotidase, was inhibited after 24 to 48 hrs incubation with either 25-OH or triol. Endocytosis was also significantly inhibited by both 25-OH and triol. Depletion of membrane cholesterol content by the cholesterol oxides could account for the membrane functional alterations. Cholesterol biosynthesis is markedly inhibited by 25-OH. Triol has a lesser effect on cholesterol biosynthesis, but it is more potent in blocking uptake of cholesterol by arterial cells in culture. Cholesterol oxides may also influence cholesteryl ester accumulation by arterial smooth muscle cells. Incubation of cells with 25-OH resulted in a four-fold increase in cholesterol esterifying activity but no effect on cholesteryl ester hydrolytic activity. The cholesterol oxides appear to be transported in the blood primarily by very low density lipoproteins (VLDL) and low density lipoproteins (LDL). Oxidized LDL has cytotoxic effects and enhances macrophage lipid accumulation. These be effects may be directly related to the cholesterol oxide content of these lipoproteins.

Association of primary intracranial meningioma and cutaneous meningioma of external auditory canal: a case report and review of the literature.

A cutaneous meningioma of the external auditory canal occurred in a 48-year-old Filipino woman who had undergone subtotal resection of a dural-based intracranial meningioma at the ipsilateral cerebellopontine angle 36 months previously. Radiologic findings demonstrated a recurrence of intracranial meningioma with surface erosion and heterogeneous densities of the mastoid bone, without extension to the area of the external auditory canal. Meningioma in the external ear canal is extremely rare. To our knowledge, there have been only two previously reported cases, both without intracranial lesion. In this case, the auditory canal lesion may represent either an ectopic meningioma arising from an arachnoid cell rest or an occult direct extension from intracranial menigioma.

Direct cocaine cardiotoxicity demonstrated by endomyocardial biopsy.

The morbidity and mortality associated with cocaine abuse has markedly increased in recent years. Although several articles indicate a possible connection of cocaine with coronary spasm and acute myocardial infarction, this study in seven patients with a history of cocaine abuse, who underwent endomyocardial biopsy, suggests that cocaine may cause direct toxicity to the myocardium. Myocardial specimens from five of seven patients showed multifocal myocyte necrosis, of which two specimens revealed focal myocarditis, while three specimens had changes consistent with dilated cardiomyopathy. Ultrastructurally, extensive loss of myofibrils and sarcoplasmic vacuolization were observed. It is postulated that the pathogenesis of acute cocaine-induced toxicity is direct destruction of myofibrils resulting in myocyte necrosis and that these changes may or may not be associated with interstitial inflammatory cell infiltrates. Long-term abuse of cocaine may lead to interstitial fibrosis and eventually congestive heart failure.

Light chain cardiomyopathy associated with small-vessel disease.

Intramyocardial small-vessel disease associated with systemic light chain deposition is a rare condition that may occur in patients with monoclonal plasma cell proliferation. This article describes a 39-year-old woman who had experienced several episodes of subendocardial myocardial infarction and was found to have plasma cell dyscrasia. Endomyocardial biopsy revealed kappa light chain deposits along the sarcolemmal and vascular basement membranes, the latter of which resulted in vascular occlusion and myocardial infarction. Postmortem examination showed polyvisceral deposition of kappa light chains. This rare complication of plasma cell proliferative process has a poor prognosis.

Coinfection of visceral leishmaniasis and Mycobacterium in a patient with acquired immunodeficiency syndrome.

We report a case of coinfection of visceral leishmaniasis and Mycobacterium avium-intracellulare in the same lesions in the small bowel and bone marrow of a 33-year-old man with acquired immunodeficiency syndrome who complained of abdominal pain and chronic diarrhea. The duodenal mucosa and bone marrow biopsy specimens showed numerous foamy macrophages packed with two forms of microorganisms that were identified histologically and ultrastructurally as Leishmania and Mycobacterium species. Visceral leishmaniasis is rarely suspected in patients residing in nonendemic countries including the United States. It should be included in the differential diagnosis for opportunistic infection in patients with acquired immunodeficiency syndrome. An appropriate travel history is important. To our knowledge, this is the first reported case showing coinfection of visceral leishmaniasis and Mycobacterium avium-intracelluulare in the same lesion in a patient with acquired immunodeficiency syndrome.