Pancreatic metastasis from lung cancer: report of an autopsy case]

A 69 year-old man with abnormal lung shadows in the right lung field was admitted to our hospital. A chest computed tomography (CT) scan showed a lung tumor with hilar and mediastinal lymph node swelling. A CT scan of the abdomen demonstrated a solitary pancreatic head tumor with a diameter of 3 cm. Pathological examination of a transbronchial biopsy specimen revealed squamous cell carcinoma (SCC) of the lung. Since obstructive jaundice had progressed rapidly, the patient received endoscopic nasobiliary drainage (ENBD) and stent-drainage therapy prior to chemotherapy using gemcitabine. However, he died 4 months later of respiratory failure and systemic candidiasis associated with progression of the cancer. An autopsy was performed, and microscopic and immunohistochemical examination revealed that the pancreatic tumor was a metastasis from lung cancer. To our knowledge, obstructive jaundice due to pancreatic metastasis from lung SCC, especially that preceding the advent of a clinical manifestation of primary lung cancer, has rarely been reported.     

Familial gastrointestinal stromal tumor with hyperpigmentation: association with a germline mutation of the c-kit gene

We describe 2 siblings with multiple gastrointestinal stromal tumors (GISTs) and cutaneous hyperpigmentation. Both had a point mutation of the c-kit gene. The patients were sisters who had exhibited cutaneous hyperpigmentation since their late teens, but the diagnosis of multiple gastrointestinal submucosal tumors was not made until they were 41 and 45 years old. Histologic examination showed that these tumors were GISTs expressing CD34 and Kit protein. Both patients died of GISTs. Single-strand conformation polymorphism analysis showed a mutation of c-kit in tumor DNA extracted from paraffin-embedded specimens. Direct sequencing analysis showed that the point mutation occurred at codon 559 of exon 11 (Val-->Ala). The same single-point mutation was detected in DNA extracted from peripheral leukocytes obtained from the younger sister and her 2 children (who had similar general hyperpigmentation) as well as in DNA from a skin biopsy specimen taken from the older sister. The germline mutation at codon 559 of the c-kit gene found in the present familial GISTs differed from that in a previously reported case of familial GISTs. We propose that GISTs caused by a germline mutation of the c-kit gene should be referred to as GIST-cutaneous hyperpigmentation disease.     

High efficacy of pleurodesis using OK-432 for controlling intractable pneumothorax associated with pulmonary lymphangioleiomyomatosis]

We evaluated the efficacy of pleurodesis using OK-432 (Picibanil) for the treatment of pneumothorax associated with pulmonary lymphangioleiomyomatosis (LAM). Seven episodes of pneumothorax in five patients with LAM were treated with pleurodesis using OK-432. First, all patients underwent tube thoracostomy drainage. After drainage, 5 or 10 KE of OK-432 was administered via the tube. If incomplete lung expansion or a continuous air leak occurred, an additional 5 KE of OK-432 was administered. Of the five patients, two developed pneumothorax for the first time and three had a history of previous pneumothorax. The mean total dose of OK-432 administered was 13.6 KE, and the mean period of tube drainage was 11 days. The only recurrence of ipsilateral pneumothorax after OK-432 pleurodesis was observed seven years and eight months later in association with chronic respiratory failure in one patient. This patient was successfully treated with repeated pleurodesis using OK-432 during mechanical ventilation, and no recurrence has developed in the eight years since then. The main side effects of the procedure with OK-432 were fever and chest pain, which were well controlled by non-steroidal anti-inflammatory drugs. The study concluded that pleurodesis with OK-432 was an effective and safe treatment for intractable and recurrent pneumothorax associated with LAM.     

Localization of increased hepatic vascular resistance in liver cirrhosis

To determine the localization of increased vascular resistance in cirrhotic liver, blood pressures were measured by a direct cannulation method at several key points in the hepatic vascular pathway in normal and cirrhotic rats. Cirrhosis was produced by feeding a choline-deficient diet. Blood pressures in normal rats were 110 mm H2O in the portal vein, 68 mm H2O in the terminal portal venule, 28 mm H2O in the terminal hepatic venule and 20 mm H2O in the inferior vena cava. In cirrhotic rats, blood pressures in the portal vein and the terminal portal venule were 173 and 100 mm H2O, respectively, while those in the terminal hepatic venule and the inferior vena cava were elevated only slightly above normal. These hemodynamic data suggest that an increase in vascular resistance in cirrhotic liver is present in the intrahepatic portal vein and sinusoids, but not in intrahepatic hepatic vein. In cirrhotic liver, stenosis and distortion were found in peripheral branches of the portal vein, and sinusoidal stenoses and a decrease in sinusoidal space were recognized. Accordingly, it is suggested that the increase in vascular resistance in the intrahepatic portal vein and sinusoids correlate with these structural changes. Although severe stenoses and distortion were found in hepatic vein branches, it was thought that they do not contribute to portal hypertension because of lack of increase in vascular resistance in the intrahepatic hepatic vein.     

Impaired exercise-related myocardial uptake of technetium-99m-tetrofosmin in relation to coronary narrowing and diabetic state: assessment with quantitative single photon emission computed tomography

Despite the diagnostic efficacy of stress myocardial perfusion imaging, the correlation between the actual perfusion tracer activity and diseased state of a coronary artery has not been studied in detail. We estimated exercise-related perfusion augmentation in relation to disease states of a coronary artery in diabetic and non-diabetic patients by a newly developed quantitative technetium (Tc)-99m-tetrofosmin myocardial imaging technique. Tc-99m-tetrofosmin tomographic imaging with an exercise-rest protocol was performed in 26 stable coronary patients and in 8 age-matched controls. Percent increase (%IR) in myocardial count during symptom-limited submaximal exercise-stress was calculated in 16 non-infarcted polar map segments and in each coronary territory by a subtraction technique with corrections for physical decay and injected tracer doses, and the results were compared with those of angiographically quantified coronary diameter stenosis (%DS). Percent IR and peak heart rate during exercise showed a positive linear correlation both in coronary territories with significant stenosis (%DS > or = 75%) and in control or nonstenotic (%DS < 75%) territories. The regression line in stenotic regions was, however. significantly (p < 0.01) shifted downward compared to that in non-stenotic regions. Percent IR in stenotic regions showed a significant inverse correlation with %DS. Coronary stenosis of 75% or more was identified by a %IR cutoff value of 40% with 77% sensitivity, 70% specificity, and an accuracy of 72%. In coronary territories with a %DS of less than 75%, %IR in diabetic patients was significantly lower (46+/-15%) than that in nondiabetic patients (61+/-25%). Thus, blunted exercise-related augmentation of myocardial uptake of Tc-99m-tetrofosmin correlates with the severity of coronary narrowing and diabetic state.     

Long-term follow-up study of three patients with the long QT syndrome.

We studied three women with the long QT syndrome. They were aged 42, 52 and 25 years and had experienced recurrent syncopal attacks. We followed case 1 for 17, case 2 for 18, and case 3 for over 6 y. The attacks tended to occur during the premenstrual stage in case 1 and case 2; case 3 often experienced attacks after exercise. The QT(U)c intervals on admission were 0.68, 0.62, and 0.50 in case 1, 2, and 3, respectively. Torsade de pointes followed by ventricular fibrillation was documented in case 1 and case 2. Although each was treated with a beta-blocker, none was fully compliant with the regimen. In case 1, estrogen therapy administered to maintain the hormonal balance premenstrually effectively prevented attacks. Despite the inconsistent use of beta-blockers, the attacks in case 1 and case 2 tended to decrease with age. Case 2 experienced no attacks after menopause. Cause 3 took medication consistently and remained free of attacks for over 6 y. Although she discontinued beta-blocker therapy because of pregnancy, she has experienced no attacks to date. These case studies suggest that hormonal status may be important in the development of syncopal attacks in female patients with the long QT syndrome.     

A case of pulmonary coccidioidomycosis presented with bilateral infiltrative opacities and eosinophilia

A 53-year-old male was admitted to our hospital complaining of high fever with chillness, cough and dyspnea after traveling to Arizona in the United States. The chest X-ray films taken on admission showed consolidation in the right middle lung field and bilateral nodular shadows. The laboratory data revealed an increase in white blood cell counts with eosinophilia, and a rise in erythrocyte sediment rate and serum C-reactive protein. The biopsied lung specimen by video-assisted thoracoscopic surgery showed granulomatous inflammation consisting of eosinophils and giant cells. In addition, typical spherules filled with endopores were detected in the specimen. The diagnosis of primary pulmonary coccidioidomycosis was made. After the treatment of a three months' regimen with itraconazole at the daily dosage of 200 mg, the patient's symptoms, laboratory data and radiological findings markedly improved.     

Modulation of corticospinal excitability dependent upon imagined force level

Motor imagery is defined as the mental execution of a movement without any muscle activity. In the present study, corticospinal excitability was assessed by motor evoked potentials (MEPs) when the subjects imagined isometric elbow flexion at various force levels. Electromyography was recorded from the right brachioradialis, the biceps brachii and the triceps brachii muscles. First, the maximum voluntary contraction (MVC) of elbow flexion was recorded in each subject. Subjects practiced performing 10, 30 and 60 % MVC using visual feedback. After the practice, MEPs were recorded during the imagery of elbow flexion with the forces of 10, 30 and 60 % MVC without any feedback. After the MEPs recording, we assigned subjects to reproduce the actual elbow flexion force at 10, 30 and 60 % MVC. The MEPs amplitudes in the brachioradialis and biceps brachii in the 60 % MVC condition were significantly greater than those in the 10 % MVC condition (p < 0.05). These findings suggest that the enhancement of corticospinal excitability during motor imagery is associated with an increase in imagined force level.