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Gastrointestinal stromal tumors (GISTs) associated with von Recklinghausen's disease (neurofibromatosis type 1 [NF1]) have different pathogenesis and characteristics from common GISTs. Furthermore, no treatment strategy for this type of GIST has been established. This study presents the case of a 76-year-old man previously diagnosed with NF1 who was later diagnosed with GISTs. A resection of the horizontal leg of the duodenum was performed, and no recurrence was observed 18 months after the surgery.

An 82-year-old male with jaundice was referred to our hospital for a detailed examination. The computed tomography (CT) examination detected an enhanced mass lesion of the distal bile duct. Endoscopic retrograde cholangiography showed a filling defect corresponding to the CT findings. Simultaneously, a forceps biopsy and an endoscopic retrograde biliary drainage were performed. We performed pancreatoduodenectomy, and adenocarcinoma was pathologically proven. The histopathological finding of the resected specimen was a mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) composed of large cell neuroendocrine carcinoma and well-differentiated adenocarcinoma. Although pathological R0 resection was achieved, liver metastasis was observed 6 months after the operation. Although neuroendocrine carcinoma (NEC) rarely develops in the bile duct, it manifests a higher degree of malignancy than other ordinary bile duct adenocarcinomas. Further investigation is needed to choose an appropriate treatment.

This paper reports the 5-year operational status of the third phase of the"All Japan E-Learning Cloud of the Training Program for Oncology Professionals"by tabulating the viewing trends of available lecture contents. In this phase, the goal was to train cancer genome medical professionals in this new, advanced medical technology field as well as train personnel to treat rarely encountered pediatric, adolescent/young adult, and other life stage cancers. Additionally, new lecture items have been added to the e-learning cloud in collaboration with 7 oncology specialist centers, contributing to the development of human capital in cancer care(including graduate student education)and faculty development for local medical professionals.

A 68-year-old woman with an 11-day history of sudden abdominal pain and severe watery diarrhea was transferred to our hospital due to an exacerbation of renal function despite hydration. After treatment for dehydration and acidemia was provided in our intensive care unit, patient's renal function improved. Contrast-enhanced abdominal computed tomography was finally performed, revealing a hypervascular pancreatic mass with multiple hepatic masses. This imaging finding along with her clinical symptoms indicated watery diarrhea hypokalemia achlorhydria (WDHA) syndrome caused by a pancreatic VIPoma. Somatostatin analog was administered immediately leading to the improvement of her diarrhea and her general condition. As a result, endoscopic ultrasonography-guided fine-needle aspiration could be performed. Consequently, she was diagnosed with a pancreatic neuroendocrine tumor. She then underwent surgical resection of the pancreatic tumor and liver metastasis. As revealed in the immunohistochemical analysis of the excised tumor tissue, VIP was highly expressed, resulting in the final diagnosis of pancreatic VIPoma. Therefore, the immediate use of a somatostatin analog is crucial for improving the patient's general condition and achieving a definitive diagnosis pathologically when a patient is suspected of having a pancreatic VIPoma.

Estracyt○R (estramustine phosphate) is a medical drug for prostate cancer with cytotoxic activity causing disruption of microtubule organization and indirect androgen production suppressing activity by its metabolite, estradiol. Based on the data obtained from the Estracyt○R Special Drug Use Investigation which surveyed the clinical efficacy and safety of Estracyt○R in patients with prostate cancer whose relapse of prostate cancer after combined androgen blockade (CAB) therapy was confirmed, we evaluated the progression-free survival, prognostic factor, decrease in prostate specific antigen (PSA) level and safety. This surveillance was conducted at 147 institutions nationwide between October, 2010 and September, 2013 and clinical efficacy was evaluated in 239 cases and safety in 329 cases. The median duration of progression-free survival, PSA progression-free survival and PSA response were 169 days (95%CI, 142-190), 197 days (95%CI, 169-267) and 385 days, respectively. The decrease in PSA level was observed in 125 cases (52.3%). Rate of PSA decline ＞50 and ＞25% were 18.4 and 43.1, respectively, and rate of PSA best response (PSA decline ＞ 50%) was 32.6%. Multivariate analysis demonstrated that long duration of prior CAB therapy, Estracyt○R - pretreatment PSA value and bone metastasis influenced progression-free survival significantly. Adverse events were observed in 127 cases (38.6%). The major adverse events were anorexia which was observed in 35 cases (10.9%), gastrointestinal disorders observed in 32 cases (9.7%), abnormal laboratory test values observed in 31 cases (9.4%) and gynecomastia observed in 16 cases (4.9%). These results suggest the clinical efficacy and safety of Estracyt○R for chemotherapy-naïve castration-resistant prostate cancer (CRPC), and Estracyt○R is regarded as one of the treatment options for patients with CRPC, especially for patients who had long duration of prior CAB therapy.

A 64-year-old male came to our hospital emergency department with fever and consciousness disturbance. Culture tests of blood and spinal fluid samples revealed meningococci (Neisseria meningitidis), and we made a diagnosis of meningococcal meningitis. Brain magnetic resonance imaging (MRI) findings revealed ventriculitis. Ceftriaxone was administered for 17 days, however, relapse was noted after that was discontinued, with neutropenia and renal impairment thought to be adverse reactions to the beta-lactam antibiotic. Hence, treatment was switched to oral administration of moxifloxacin for a total of 12 weeks, including in an outpatient setting. After moxifloxacin was discontinued, no side effects or relapse were seen, and treatment was ended. Although antibacterial agents generally show favorable effects for meningococcal meningitis, we consider that sufficient antimicrobial therapy is difficult in cases complicated with ventriculitis.

The blood culture contamination rate is often used to validate specimen-collection procedures. CUMITECH has set its optimal target to be 2% to 3%. However, the term "contamination rate" has been defined in many ways, limiting its generalizability. The definitions used in earlier studies can be divided into two categories; definitions based on clinical judgements, and those based on preset rules. According to each principle, the equation must be composed of a defined numerator and denominator. The problem with clinical definitions is that the decision is inevitably subjective, and the process is too cumbersome. Also, if the number of positive cultures is used as the denominator, the value would be equivalent to the positive predictive value, given that contamination is regarded as a "positive case." Thus, the value would not be useful for validating a procedure. On the other hand, when the preset algorithm was adopted, true infection would, to some degree, inevitably be classified as contamination. Also, if the algorithm adopted the number of blood culture sets as the denominator and contamination was defined as the identification of 1 or more specified organisms in only 1 of multiple sets of blood cultures, its theoretical maximum value would not be 100%. This is a problem because the value is a mixture of several numbers with different scales. In other words, whether the blood cultures are collected once, twice, or thrice or more a day would affect the result. The study cited by CUMITECH aimed to evaluate the equivalence between the clinical definition and the laboratory definition with preset rules, rather than to establish a benchmark for the contamination rate. It is undesirable for the number to be perceived as a benchmark. "A Guide to Blood Culture" (2013) by the Japanese Society for Clinical Microbiology introduced a calculation for the contamination rate, but the definition of the term "number of specimens" in the formula is ambiguous. In addition, the references cited in the guide do not concern contamination and do not even mention the definition of contamination rate. Thus, it is impossible to confirm the definition. In view of the weaknesses of these previous works, we defined the contamination rate as a benchmark for the validation of blood culture procedures as follows. [number of series in which 1 or more specified organisms (*) were identified in only 1 of multiple sets of blood cultures]/[total number of multiple sets of blood cultures in the series] *coagulase-negative staphylococci, Propionibacterium acnes, Micrococcus spp., Viridans-group streptococci, Corynebacterium spp., and Bacillus spp., but not B. anthracis.

Objective: To compare the surgical outcomes of total arch replacement with the outcomes of ascending aorta replacement in patients with acute Stanford type A aortic dissection., Methods: From April 2002 to May 2014, 90 patients with acute Stanford type A aortic dissection underwent emergency surgical treatment at our institution. Among them, 16 underwent total arch replacement and 74 underwent ascending aorta replacement. The decision to perform total arch versus ascending aorta replacement was dependent on an intimal tearing site., Results: There were no statistically significant differences in characteristics between the groups with regard to the 2 surgical procedures. Twenty early deaths were reported, but there was no statistically significant difference in mortality rate between the groups. The aortic operation had to be redone in 12 patients. The reoperations were not associated with an increased risk of death or neurological complications. Overall 5-year survival rates were 68% in the ascending aorta replacement group and 59% in the total arch replacement group. There was no statistically significant difference between the groups., Conclusions: The surgical outcome and prognosis of ascending aorta replacement is satisfactory among patients undergoing treatment of acute type A aortic dissection.

79-year-old woman, who had undergone resection of a leiomyosarcoma of the inferior vena cava(IVC)[at 68 years old], was admitted to our hospital due to local recurrence of the leiomyosarcoma with extention into the right atrium (RA). She had progressive dyspnea and bilateral edema of the lower extremities. A computed tomography (CT) scan revealed a tumor that obstructed the IVC. A transthoracic echocardiography showed a large mobile right atrial mass. To prevent sudden death, a reoperation was performed. The tumor in the IVC and the RA was resected under deep hypothermic circulatory arrest. A postoperative CT scan showed stenosis of the IVC by residual tumor, and a Z-stent was therefore implanted in the IVC. Postoperative recovery was uneventful and the patient no longer showed any symptoms.

For lung cancer surgery which merged ischemic heart disease to need coronary artery treatments, the strategy is demanded on the timing of each treatment. Our department conforms to American College of Chest Physicians( ACCP) guideline and treatment strategies are decided as follows. 1) If right heart load has already occurred, we choose limited surgery for lung cancer. 2) Two-stage surgery is performed with principle. Coronary artery treatment is given priority to against left main trunk disease and unstable angina. 3) Simultaneous surgery is chosen for lung cancer more than stage II or lung cancer pressing neighboring organ and vessel not to be able to wait coronary artery treatments. Since 2007, we performed 4 simultaneous surgeries and experienced 3 pneumonia cases, 1 patient died in 5 months. We must decide a strategy in consideration of progress of the lung cancer and cardiac urgency.

An 82-year-old bedridden man with sequelae from a cerebral infarction was admitted to a welfare institution for the elderly. He developed a high fever and hematuria and was prescribed levofloxacin for the treatment of a suspected urinary tract infection. Although his condition improved, the symptoms subsequently recurred; therefore, levofloxacin was again administered. He remained febrile and was admitted to a hospital due to recalcitrant urinary tract infection. Immediately after admission, he developed ischuria and pyuria. Urine and blood cultures at admission indicated the presence of levofloxacin-resistant Oligella urethralis (O. urethralis). He recovered with ceftriaxone medication. To our knowledge, this is the first report of bacteremia associated with a urinary tract infection caused by O. urethralis in Japan.

We present a rare case of intraorbital abscess caused by Candida allociferrii, which was described as a new species of Stephanoascus ciferrii complex in 2002, in a patient after enucleation of choroidal melanoma. This strain showed resistance to itraconazole, and fluconazole. After debridement and topical use of amphotericin B, his wound abscess was disappeared. To our knowledge, this is the first C. allociferrii infection case in human.

Objective: Recently, some technical improvements in aortic arch repair have brought better outcomes. In this article, we reviewed our surgical results of open arch repair using selective cerebral perfusion(SCP)., Methods: Between 2001 and 2012, a total of 813 patients (median age of 70 years) underwent total arch replacement with SCP. We preferably use the right axillary artery cannulation for SCP and stepwise technique for distal anastomosis. The temperature at hypothermic circulatory arrest was moder ate in 46%, and deep in 54%.Of them, 22% had emergency surgery., Results: Early mortality was 5.2% in all. The incidences of permanent( PND) and temporary neurological deficits were 3.4% and 7.3%. No spinal cord injury occurred, even with moderate hypothermia. The cumulative survival rates were 78.4% and 49.9% at 5 and 10 years, respectively. The freedom from arch reoperation was 98.0% and 96.2% at 5 and 10 years, respectively., Conclusions: Open total arch replacement using selective cerebral perfusion with some technical improvement provides favorable outcome, even in long-term period.

In September 2008, a 60-year-old woman presented to our hospital with a complaint of bloody sputum; she was healthy until this event. Chest computed tomography scan revealed a cavity, nodular shadows, and bronchiectasis in the left upper lobe and in the left and right middle lobes. Acid-fast bacilli were detected 2 times on gastric juice culture and Mycobacterium gordonae was identified on biochemical study. No active chemotherapy was administered because the discharge of this strain was considered casual and clinically nonsignificant. However, her radiological findings worsened in the following 1 year and 3 months, and M. gordonae was detected 2 more times on gastric juice culture. Subsequently, she was diagnosed with pulmonary mycobacteriosis caused by M. gordonae and was treated with clarithromycin, rifampicin, and levofloxacin. After 1 month, her gastric juice culture yielded negative results for M. gordonae, and after a year and a half, her radiological findings improved. In this case, gastric juice culture was as useful as sputum examination for diagnosis and evaluation of the disease. Although M. gordonae is usually considered nonpathogenic, our study shows that it can be pathogenic, and M. gordonae infection may require treatment with chemotherapy.

This chapter describes anesthetic consideration in patients undergoing catheter ablation for atrial fibrillation (AF) based on electrophysiologic or pharmacological aspects. In the 2011 guidelines of the Japanese Circulation Society for non-pharmacotherapy of cardiac arrhythmias, catheter ablation is recommended as Class I therapeutic modality for the patients with drug-refractory paroxysmal AF. Catheter ablation of AF is an invasive and long-lasting procedure necessitating sedation during treatment. However, in the most of the patients, sedation or anesthesia is possibly performed by cardiologists using propofol, midazolam or dexmedetomidine. Deep sedation accompanies a high risk of ventilatory or circulatory derangement. Furthermore, life-threatening complications, such as cerebral infarction or cardiac tamponade, can occur during ablation. Patients with AF are increasing in number as a trend in the aging society, resulting in an increase in catheter ablation in high risk patients. To accomplish safe anesthetic management of the patients for catheter ablations, anesthesiologists are required to have fundamental knowledge and skill in the performance of the catheter ablation.

Background: The anesthetic management of Jehovah's Witnesses (JW) patients undergoing open heart surgery with cardiopulmonary bypass (CPB) is associated with a major risk of anemic hypoxia., Methods: Fifteen patients from the community of JW underwent open heart surgery. The surgical procedures undertaken were aortic valve replacement in 7 patients, mitral valve plasty or replacement in 5 patients, repair of congenital heart diseases in 2 patients, and removal of left atrial myxoma in one patient. Hemoglobin values, cardiac output, and oxygen delivery (Do2) as well as consumption (Vo2) were evaluated during and after surgery., Results: After CPB, the mean hemoglobin (Hb) value decreased to 8.2 g x dl(-1) from the baseline level of 11.6 g x dl(-1) (P = 0.0007); however, it increased to 10.5 g x dl(-1) at the end of surgery Cardiac index remained unchanged. Oxygen delivery (Do2) decreased after CPB from its baseline level, whereas oxygen consumption remained unchanged. Twenty-four hours after surgery, Do2 recovered to its baseline level because of an increase in cardiac output with the Hb value of 10.7 g x dl(-1) ., Conclusions: The decrease in Hb level is unavoidable in cardiac surgery with CPB in these JW patients. This survey showed that the decrease in Hb level may be compensated by conserved cardiac output avoiding the decrease of Do2 to its critical level when hypoxia occurs.

Objective: This research was conducted to determine the prevalence of recognition of child maltreatment among mothers with children aged 12 or under, and to identify associated factors in order to prevent child maltreatment., Methods: The subjects of this study were 3,000 women extracted by systematic random sampling of mothers of 6,790 children who had had four-month health check-ups in Nishinomiya city. The response rate was 57.5%. After excluding mothers with children aged 13 years and over, the study sample included 1,471 mothers with children aged 12 or under. A questionnaire survey was conducted by mail. Recognition of child maltreatment by mothers was assessed with a question that asked the mother if she was sometimes aware that she had potentially abused her child, and, if yes, what kind of acts had she performed., Results: There were 333 mothers (22.6%) who answered "yes" to the question "Are you sometimes aware that you have potentially abused your child?" These mothers reported emotional or physical aggression toward their children. Results of logistic regression showed that recognition of child maltreatment was associated with existence of a child whom the mother felt difficulty in cherishing, number of children, maternal poor health, higher scores of STAI trait anxiety and disabled children. Mothers reported difficulties in child-rearing for more than one child as the main reason behind existence of a child whom the mother felt difficulty in cherishing., Conclusion: These findings suggested that mothers with more than one child need more support in order to prevent child maltreatment.

Median sternotomy is the most widely used incision in cardiac surgery. The skin incision should extend from just below the sternal notch to a few centimeters below the xiphoid process. Careful dissection behind sternal notch and xiphoid process should be required to prevent accidental adjacent vessels injuries. The sternotomy should be made on the midline of the sternum after detecting the lateral margin of the sternum by dipping the thumb and the index finger into the intercostal space. Off-midline sternotomy may cause the closure wires to cut through the thinner segment of the bone, which may cause wound infection. There has been an increase in the number of patients who undergo a 2nd or even a 3rd time cardiac surgery. Redo sternotomy is becoming a major technique in cardiac surgery. The sternum could be divided with an oscillating saw safely by lifting previous wires untwisted, which helps prevent possible right ventricular injury. Blunt digital manipulation or dissection can often result in tearing of the right ventricular wall which can be fatal.

As longer lives have become possible due to advances in medical technology and diagnostic technology in recent years, weakly toxic fungus have been emerging as causative agents of opportunistic infections, primarily in high-risk groups. We report a case in which the yeast-like fungus Stephanoascus ciferii, which has morphological characteristics that differ from those of the more common Candida species, was isolated by culturing the aural discharge of a patient with intractable otitis media. Drug susceptibility testing showed that it was resistant to fluconazole, flucytosine, and itraconazole, suggesting that it is a species that has a strong tendency to become resistant. The principal sites of infection by Stephanoascus ciferii are thought to be in the ENT area, but in other countries it has been isolated from patients with acute myeloid leukemia and immunodeficiency as well as superficial mycoses, and it may also be a causative agent of deep mycoses. We therefore think that it is necessary to bear in mind detection in other areas outside the ENT field and to adequately monitor the circumstances under which this fungus is isolated.

Factors that affect the response of multiple myeloma patients to thalidomide were evaluated in 40 patients who were not eligible for chemotherapy (untreated: 14, relapse/refractory: 26). The complete response (CR) rate was 2.5%; partial response (PR) 50.0%; minimal response (MR) 25.0%; no change (NC) 12.5%; and progressive disease 10.0%. The response to thalidomide could be evaluated after four weeks of treatment. Significantly higher responses were associated with untreated patients, patients with combined use of thalidomide plus dexamethasone, and patients with kappa light chain. Patients who responded well to thalidomide showed a significantly higher progression-free survival (PFS) rate. In patients with kappa light chain, PFS and overall survival rates were significantly higher than those with lambda light chain. Frequent adverse reactions were numbness (47.5%), constipation (32.5%), and eruption (30.0%). In patients previously treated with vincristine, numbness occurred in a significantly higher percentage of patients.

Case 1: A 41-year-old man developed right chest pain. Computed tomography (CT) revealed right tension hemopneumothorax. Chest tube was placed in the pleral cavity immediately. Because of continuous bleeding and inadequate lung expansion, video-assisted thoracic surgery (VATS) was performed on day 11 afer admission. Thoracoscopic view clearly showed bleeding point at the thoracic apex. Hemostasis was performed and peel was removed for expansion of lung. Case 2: A 38-year-old man developed right chest pain and dyspnea. CT revealed right hemopneumothorax. Because of massive continuous bleeding, emergent VATS was performed. Thoracoscopic view clearly showed bleeding point at the thoracic apex and the hemostasis and the resection of bullae was performed. Postoperative course of both cases was uneventful. It is thought to be important to consider early surgical approach for hemopneumothorax to identify the cause of airleakage before forming thick peel on the visceral pleura.