A case of primary pleural leiomyosarcoma]

A 70-year-old man was admitted to our hospital with complaints of chest pain and exertional dyspnea. Chest radiography and computed tomography (CT) revealed right pleural effusion and pleural thickening on admission. The pleural fluid was bloody. Microbiological and cytologic examinations of the fluid were negative. The chest CT revealed progress of pleural thickening after hospitalization. A thoracoscopic pleural biopsy was performed, and the histological finding of the excised specimen was leiomyosarcoma. Because no organ of origin of the leiomyosarcoma, other than the pleura, was detected, this case was diagnosed as a primary pleural leiomyosarcoma. It is thought that leiomyosarcoma originating from the pleura is rare.     

Cytochrome P450 oxidoreductase gene mutations and Antley-Bixler syndrome with abnormal genitalia and/or impaired steroidogenesis: molecular and clinical studies in 10 patients

We report on molecular and clinical findings in 10 Japanese patients (four males and six females) from eight families (two pairs of siblings and six isolated cases) with Antley-Bixler syndrome accompanied by abnormal genitalia and/or impaired steroidogenesis. Direct sequencing was performed for all the 15 exons of cytochrome P450 oxidoreductase gene (POR), showing two missense mutations (R457H and Y578C), a 24-bp deletion mutation resulting in loss of nine amino acids and creation of one amino acid (L612_W620delinsR), a single bp insertion mutation leading to frameshift (I444fsX449), and a silent mutation (G5G). R457H has previously been shown to be a pathologic mutation, and computerized modeling analyses indicated that the 15A>G for G5G could disturb an exonic splicing enhancer motif, and the remaining three mutations should affect protein conformations. Six patients were compound heterozygotes, and three patients were R457H homozygotes; no mutation was identified on one allele of the remaining one patient. Clinical findings included various degrees of skeletal features, such as brachycephaly, radiohumeral synostosis, and digital joint contractures in patients of both sexes, normal-to-poor masculinization during fetal and pubertal periods in male patients, virilization during fetal life and poor pubertal development without worsening of virilization in female patients, and relatively large height gain and delayed bone age from the pubertal period in patients of both sexes, together with maternal virilization during pregnancy. Blood cholesterol was grossly normal, and endocrine studies revealed defective CYP17A1 and CYP21A2 activities. The results suggest that Antley-Bixler syndrome with abnormal genitalia and/or impaired steroidogenesis is caused by POR mutations, and that clinical features are variable and primarily explained by impaired activities of POR-dependent CYP51A1, CYP17A1, CYP21A2, and CYP19A1.     

Association of early annual peak influenza activity with El Niño southern oscillation in Japan

Background Seasonality characterizing influenza epidemics suggests susceptibility to climate variation. El Niño southern oscillation (ENSO), which involves two extreme events, El Niño and La Niña, is well‐known for its large effects on inter‐annual climate variability. The influence of ENSO on several diseases has been described. Objectives In this study, we attempt to analyze the possible influence of ENSO on the timing of the annual influenza activity peak using influenza‐like illness report data in Japan during 1983–2007. Materials Influenza surveillance data for 25 influenza epidemics, available under the National Epidemiological Surveillance of the Infectious Diseases, was used in this study. ENSO data were obtained from the Japan Meteorological Agency. Results Influenza‐like illness peak week varied largely during the study period, ranging between 4th and 11th weeks (middle of winter to early spring). The average of peak week during ENSO cycles (n = 11, average = 4·5 ± 0·9) was significantly earlier than in non‐ENSO years (n = 14, average = 7·6 ± 2·9; P = 0·01), but there was no significant difference in the peak timing between hot (El Niño) and cold (La Niña) phases. Earlier peaks of influenza activity were observed in 16, out of 25, epidemics. These coincided with 10 (90·9%) out of 11 ENSO and 6 (85·7%) out of seven large‐scale epidemics. Conclusion Influenza activity peak occurred earlier in years associated with ENSO and/or large scale epidemics.     

Comparison of myocardial perfusion by distal protection before and after primary stenting for acute myocardial infarction: angiographic and clinical results of a randomized controlled trial.

OBJECTIVES: To assess the myocardium-reperfusing effect of a distal protection device, GuardWire Plus (GuardWire Plus), in patients with acute myocardial infarction (AMI). BACKGROUND: Distal embolization may result in reduced myocardial perfusion, increasing the risk of non-Q-wave myocardial infarction and death. Distal protection devices may protect the microcirculation from embolic debris, improving short- and long-term clinical outcomes. METHODS: From February 2002 to July 2003, a total of 341 AMI patients at 22 institutions in Japan were enrolled in the present, multicenter, prospective, randomized trial. Patients experiencing AMI within 12 hr of symptom onset, who were considered treatable by stenting and who met the inclusion criteria, were eligible for randomization. Stenting with and without GuardWire Plus was conducted to examine whether the device provides faster and more complete ST-segment resolution, smaller infarct size, and improved myocardial blush score. RESULTS: The rates of slow flow and no-reflow immediately after PCI were 5.3 and 11.4% in the GuardWire Plus and control groups, respectively (P = 0.05). Blush score 3 acquisition rates immediately after PCI were 25.2 and 20.3% in the GuardWire Plus and control groups, respectively (P = 0.26), and the rates at 30 days after PCI were 42.9 and 30.4%, respectively (P = 0.035). CONCLUSIONS: A significant difference was found between the GuardWire Plus and control groups with respect to the total incidence of distal embolization, indicating that GuardWire Plus angiographically improved myocardial perfusion without demonstrating the preventive effect of myocardial damage.     

Treatment of elderly patients with early gastric cancer by endoscopic submucosal dissection using an insulated-tip diathermic knife

OBJECTIVE: In recent years, the number of elderly patients with early gastric cancer (EGC) has steadily been increasing. In our institute, endoscopic submucosal dissection (ESD) involving the use of an insulated-tip diathermic knife (IT-ESD) was introduced for the treatment of mucosal gastric carcinoma in 1996. The purpose of this study was to evaluate the effectiveness of IT-ESD for the treatment of elderly patients with EGC. MATERIALS AND METHODS: A total of 144 patients with EGC were treated at Shikoku Cancer Center in the 5-year period from January 2000 to December 2004, including 53 patients over 75 years old. The performance status (PS) for all patients was less than 2. We compared patient's backgrounds, the one-piece resection rate, complete resection (CR) rate, operation time, bleeding rate, perforation rate, blood pressure, and peripheral oxygen saturation (SpO(2)) between patients over 75 years of age (elderly group) and the remaining 91 younger patients (non-elderly group). RESULTS: The rate of having underlying disease was significantly higher for the elderly group (p<0.05) and we found no significant difference for the one-piece resection rate, CR rate, operation time, bleeding rate, and perforation rate between the 2 groups. There were also no significant differences for the frequency of the use of oxygen, pressor and depressor between the 2 groups. CONCLUSION: There was no significant difference in the outcome resulting from ESD between the 2 groups. Our study proves that ESD is a feasible treatment for elderly patients with EGC PS of less than 2.     

A rare tumor in the adrenal region: neuron-specific enolase (NSE)-producing leiomyosarcoma in an elderly hypertensive patient

A 73-year-old Japanese woman was referred for examination of right flank pain and progressive hypertension. Abdominal CT incidentally detected a right adrenal mass 8 cm in size. The tumor exhibited isodensity by CT and contained high-intense lesion by T2-weighted MRI. Scintigraphy with (131) I-metaiodobenzylguanidine and (131) I-adosterol showed no abnormal uptake by whole body scan. Positron emission tomography scan with (18) F-2-fluoro-D-deoxyglucose demonstrated an exclusive uptake in the right adrenal mass. Adrenocortical hormone levels and catecholamine secretion were within normal range; however, the level of serum neuron-specific enolase (NSE) was found to be markedly high. After controlling systemic blood pressure with an alpha1-blocker, the right adrenal tumor was surgically removed, along with the right kidney and inferior vena cava which adhered to it. The tumor was pathologically proven to be leiomyosarcoma, which was immunohistochemically positive with alpha-smooth muscle actin and negative with CD57, S-100 and c-kit proteins. Notably, NSE protein was massively expressed in the resected tumor. After surgery blood pressure was controlled with regular medication and serum NSE levels have since normalized. The possibility of leiomyosarcoma should be kept in mind in adrenal incidentalomas with rapid growth and atypical radiological images. Our findings suggest that circulating NSE levels may be clinically useful for early detection of recurrence.