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Background Measurement of heart weight is important when investigating cause of death, but there is presently no satisfactory method of heart weight estimation by postmortem computed tomography (PMCT). Method We investigated 33 consecutive cases that underwent both PMCT and autopsy between February 2008 and June 2014. Heart and left ventricular (LV) weights were calculated by PMCT morphometry. We used a simple method to estimate LV weight: We assumed that LV was an ellipsoid and multiplied its volume on PMCT with myocardial specific gravity. We then compared the various heart and LV weights using linear regression. The calculated and estimated LV weights on PMCT were also compared. Results It was not possible to predict heart weight at autopsy from PMCT (R2 = 0.53). However, heart weight at autopsy could be accurately predicted from LV weight calculated by PMCT (R2 = 0.77). In addition, there was a strong correlation between the estimated and calculated LV weights by PMCT (R2 = 0.92). Heart weight at autopsy could also be accurately predicted using the PMCT-estimated LV weight (R2 = 0.72). Conclusion Heart weight at autopsy could be accurately predicted using a simple method in which LV volume was assumed to be an ellipsoid on PMCT.

A histological grading system of chromophobe renal cell carcinoma (chRCC) is highly desirable to identify approximately 5-10% of tumors at risk for progression. Validation studies failed to demonstrate a correlation between the four-tiered WHO/ISUP grade and outcome. Previous proposals with three-tiered chromophobe grading systems could not be validated. In this study, the presence of sarcomatoid differentiation, necrosis, and mitosis was analyzed in a Swiss cohort (n = 42), an Italian cohort (n = 103), a German cohort (n = 54), a Japanese cohort (n = 119), and The Cancer Genome Atlas cohort (n = 64). All 3 histological parameters were significantly associated with shorter time to tumor progression and overall survival in univariate analysis. Interobserver variability for identification of these parameters was measured by Krippendorff's alpha coefficient and showed high concordance for the identification of sarcomatoid differentiation and tumor necrosis, but only low to medium concordance for the identification of mitosis. Therefore, we tested a two-tiered tumor grading system (low versus high grade) based only on the presence of sarcomatoid differentiation and/or necrosis finding in the combined cohorts (n = 382). pT stage, patient's age (65 vs ≤ 65), lymph node and/or distant metastasis, and the two-tiered grading system (low versus high grade) were significantly associated with overall survival and were independent prognostic parameters in multivariate analysis (Cox proportional hazard). This multi-institutional evaluation of prognostic parameters suggests tumor necrosis and sarcomatoid differentiation as reproducible components of a two-tiered chromophobe tumor grading system.

We experienced a 58-year-old Japanese female complaining of bleedings at defecation for about one year. Endoscopy revealed an Ip submucosal tumor (SMT) at the rectum. Continuous bleedings at defecation and no evidence malignancy in a biopsy specimen led to a polypectomy. The specimen showed SMT with erosion, while the macroscopic cross-section implied a lipomatous mesenchymal tumor. Under the probable diagnosis of liposarcoma, especially myxoid liposarcoma, fusion-gene analyses were performed. The detection of type 1 FUS-DDIT3 fusion gene led to a definite diagnosis of rectal myxoid liposarcoma. For further treatment, a super low anterior resection was performed, revealing neither residual tumors nor lymph node metastases. The patient is now alive and free of disease for 5 years. To our knowledge of the literature, this is the first case of a rectal myxoid liposarcoma. Keywords: Rectum, Myxoid liposarcoma, Submucosal tumor

Introduction Non-occlusive mesenteric ischemia (NOMI) is a type of acute intestinal ischemia, and its associated mortality is very high. In laparotomy of NOMI, we often have difficulty determining the area of bowel resection. We herein describe a case in which we detected the area of bowel resection using indocyanine green (ICG) fluorescence imaging. Presentation of the case An 89-year-old man diagnosed as having advanced gastric cancer underwent distal gastrectomy. On the night of postoperative day 4, he strongly complained of distention of the abdomen. The laboratory data indicated severe metabolic acidosis and dehydration. The abdominal computed tomography scan showed a dilated small bowel, but there were no specific signs suggestive of bowel necrosis. We suspected NOMI and decided to perform emergency laparotomy because we could not exclude the possibility of bowel necrosis. During the operation, we could not detect the necrotic bowel macroscopically. After injecting 2.5 mg of ICG, the ischemic area of the bowel became visible as a region with poor fluorescence emission using the Photodynamic Eye™ (Hamamatsu Photonics K.K.). We resected the ischemic bowel and performed anastomosis. We confirmed that he was alive at 4 months after the operation of NOMI. Conclusion Intraoperative ICG fluorescence imaging makes it possible to detect necrotic intestine that cannot be found with the naked eye. By using this method, planned reoperation to find any newly developed necrotic intestine might be unnecessary. Intraoperative ICG fluorescence imaging is useful for defining the area of ischemic bowel in a patient with NOMI.

Chromophobe renal cell carcinoma (chRCC) is a renal tumor subtype with a good prognosis, characterized by multiple chromosomal copy number variations (CNV). The World Health Organization (WHO) chRCC classification guidelines define a classic and an eosinophilic variant. Large cells with reticular cytoplasm and prominent cell membranes (pale cells) are characteristic for classic chRCC. Classic and eosinophilic variants were defined in 42 Swiss chRCCs, 119 Japanese chRCCs and in whole-slide digital images of 66 chRCCs from the Cancer Genome Atlas (TCGA) kidney chromophobe (KICH) dataset. 32 of 42 (76.2%) Swiss chRCCs, 90 of 119 (75.6%) Japanese chRCCs and 53 of 66 (80.3%) TCGA-KICH were classic chRCCs. There was no survival difference between eosinophilic and classic chRCC in all three cohorts. To identify a genotype/phenotype correlation, we performed a genome-wide CNV analysis using Affymetrix OncoScan® CNV Assay (Affymetrix/Thermo Fisher Scientific, Waltham, MA, USA) in 33 Swiss chRCCs. TCGA-KICH subtypes were compared with TCGA CNV data. In the combined Swiss and TCGA-KICH cohorts, losses of chromosome 1, 2, 6, 10, 13, and 17 were significantly more frequent in classic chRCC (p < 0.05, each), suggesting that classic chRCC are characterized by higher chromosomal instability. This molecular difference justifies the definition of two chRCC variants. Absence of pale cells could be used as main histological criterion to define the eosinophilic variant of chRCC., Cancers, 11 (10), ISSN:2072-6694

We report the case ofan elderly patient with squamous cell carcinoma(SCC)ofthe breast, which showed negative conversion after endocrine therapy. An 82-year-old woman with a diagnosis ofER -positive SCC(cT1N0M0, StageⅠ)received primary endocrine therapy with 5 hormonal medicines. Following the endocrine therapy, she underwent mastectomy and axillary node resection. Histological examination confirmed a diagnosis of ER-negative SCC. At 10 months after the operation, she was doing well with no evidence ofdisease without postoperative adjuvant therapy. Thus, clinicians should be aware that the ER status may change after primary endocrine therapy. For elderly patients with breast cancer, it is important to be aware that primary endocrine therapy can become ineffective due to ER-negative conversion and aging due to prolonged treatment.

We report the case of a patient with triple negative breast cancer(TNBC)who showed isolated brain metastasis relatively soon after pathological complete response(pCR)to neoadjuvant chemotherapy. A 45-year-old woman with a diagnosis of TNBC(T2N1M0, Stage II B)received neoadjuvant chemotherapy with 5-FU/epirubicin/cyclophosphamide(FEC), followed by docetaxel. After the neoadjuvant chemotherapy, she underwent mastectomy and axillary lymph node dissection. Histological examination of the resected specimens revealed pCR. Brain metastasis, however, developed 7 months after the resection. She underwent total removal of the brain tumor and 50 Gy irradiation to the right frontal lobe. Histological examination confirmed a diagnosis of metastasis from TNBC. She is doing well with no evidence of disease 81 months after resection of the brain metastasis. This case and a review of the literature suggest that the clinician should be aware that brain metastasis from breast cancer may develop even after achieving pCR to neoadjuvant chemotherapy. Surgical resection followed by radiotherapy may provide a survival benefit for selected patients with isolated brain metastasis from breast cancer.

Aims To retrospectively evaluate the correlation between multidetector-row computed tomography findings of acute appendicitis and the pathological status of acute appendicitis, and evaluate the capability of multidetector-row computed tomography to predict the pathological status of acute appendicitis in children. Methods The presence of a distended appendix (>6 mm in transverse diameter) was used as a primary sign to indicate the presence of appendiceal inflammation. The presence of appendiceal wall thickening (>1 mm) and enhanced appendiceal wall continuity were also used as predictive findings to reflect the degree of progression of acute appendicitis on multidetector-row computed tomography findings. The findings of each individual case were classified into four grades. The final pathological diagnosis was classified into four groups: normal findings, only mucosal inflammation, inflammation with intramural spreading, and gangrenous. The relationship between the pathological grades and computed tomography grades was analyzed using Spearman's rank correlation test. Results Four of six cases in Grade 0 reflected normal appendiceal findings (66.7%) and 3 of 5 cases in Grade I reflected only mucosal inflammation status (60.0%). Forty-four of 51 cases in Grade II reflected intramural inflammation status (86.3%), and 40 of 57 cases in Grade III reflected gangrenous status (70.2%). The multidetector-row computed tomography grade was significantly correlated to the pathological grade with Spearman's rank correlation coefficient of 0.689 (P

We herein report a case of abdominal actinomycosis in a 12-year-old girl in whom an omental primary tumor was suspected before surgery. The patient began to experience intermittent lower left abdominal pain. Abdominal computed tomography (CT) scans were inconclusive at this time, but 6 months later, CT and magnetic resonance imaging (MRI) examinations showed a 7-cm, tumor-like lesion in the left abdominal cavity; malignancy could not be ruled out. The tumor, which originated in the omentum and adhered strongly to the left abdominal wall, was resected along with approximately 90 % of the omentum, the peritoneum in contact with the mass, and the posterior layer of the rectus abdominal sheath, under suspicion of a malignant tumor. However, omental actinomycosis was the final pathological diagnosis. The patient's antibiotic treatment was changed to a penicillin-series oral antibiotic to prevent recurrence of the actinomycosis. The patient was discharged from our hospital 16 days after the first surgery, but she developed three episodes of ileus; the first two required surgery. The patient has had no further recurrences of actinomycosis or postoperative ileus 20 months after discharge.

To characterize genetic diversity and gene flow among Daphnia galeata populations, the complete nucleotide (nt) sequences of the mitochondrial (mt) DNAs of D. galeata clones isolated from five lakes in Japan (Lakes Shirakaba, Suwa, Kizaki, Kasumigaura, and Biwa) were determined. Comparison of non-synonymous (amino acid altering) substitution rates with synonymous substitution rates of D. galeata mt protein-coding genes demonstrated that ATPase8 and COI genes were the most and least susceptible, respectively, to the evolutional forces selecting the aa substitutions. Several non-synonymous substitutions were found in ATPase8 and ATPase6 even in the comparison that no synonymous substitution was found. Comparison of the total number of nt variations among the mt DNAs suggested the phylogenetic relationship ((((Shirakaba/Suwa, Kizaki), Kasumigaura), Biwa), D. pulex). Maximum-likelihood analysis using the total nt sequences of mt protein-coding genes confirmed this relationship with bootstrap values higher than 98%. All the mtDNAs of the analyzed Japanese D. galeata clones contained a control region of essentially the same structure that is distinct from those of the previously reported European Daphnia species of the D. longispina complex. The two control regions of different structures spread among mtDNAs of the Japanese and European Daphnia species, respectively, probably after the divergence of the Japanese D. galeata under different selection pressures associated with their habitats.

To investigate the diagnostic performance of postmortem multidetector computed tomography (PMMDCT) for the detection of fatal findings related to causes of non-traumatic death in the emergency department (ED).494 consecutive cases of clinically diagnosed non-traumatic death in ED involving PMMDCT were enrolled. The fatal findings were detected on PMMDCT and classified as definite or possible findings. These findings were confirmed by autopsy in 20 cases.The fatal findings were detected in 188 subjects (38.1%) including 122 with definite (24.7%) and 66 with possible finding (13.4%). Definite findings included 21 cases of intracranial vascular lesions, 84 with intra-thoracic haemorrhage, 13 with retroperitoneal haemorrhage and one with oesophagogastric haemorrhage. In three patients who had initially been diagnosed with non-traumatic death, PMMDCT revealed fatal traumatic findings. Two definite findings (two haemopericardiums) and seven possible findings (two intestinal obstructions, one each of multiple liver tumours central pulmonary artery dilatation, pulmonary congestion, peritoneal haematoma, and brain oedema) were confirmed by autopsy. The causes of death were not determined in cases with possible findings without autopsy.PMMDCT is a feasible tool for detecting morphological fatal findings in non-traumatic death in ED. It is important to know the ability and limitation of PMMDCT.

Although aging is well established as an important risk factor for aortic stenosis, the mechanism of age-related aortic valve calcification is yet unknown. Here, we investigated this mechanism in tissue and cellular levels using middle-aged rats. Aortic valve specimens were obtained by dissecting from 9-week-old (young) and 30-week-old (aged) male Wistar rats. In the aged rats, the main risk factors for aortic stenosis in plasma were still in the normal range; however, their number of calcified specimens was significantly increased in comparison with the young rats. Aortic valve interstitial cells (AVICs) obtained from explants of aortic valve specimens were cultured for 14 days after reaching confluence. Spontaneous calcification, the expressions of calcigenic genes, that is, BMP-2, alkaline phosphatase (ALP), and osterix (osteogenic transcription factor) and ALP enzyme activity in AVICs from aged rats were enhanced in comparison with those from young rats. However, neither typical calcification inducing reagents (dexamethasone, β-glycerophosphate, and high concentration of phosphate) nor tumor necrosis factor-α (an inflammatory cytokine) accelerated the spontaneous calcification of AVICs from aged rats. These results suggest that aortic valve calcification progresses with age partly through an activation of the BMP-2 pathway. Keywords:: calcification, aortic valve, BMP-2, aging

Although primary carcinoid tumor of the ovary is an extremely rare neoplasm, survival is excellent if the disease is confined to one ovary. Herein, we present a case of primary strumal carcinoid tumor of the ovary, stage IA, borderline malignancy, in a 34-year-old woman. Histological findings of the right ovary indicated higher atypical nuclei, higher mitotic rate and focal necrosis of tumorous cells in some areas, findings that are compatible with atypical carcinoid of the lung. Immunohistochemical staining was positive for synaptophysin, neuron-specific enolase, chromogranin A, Ki-67, topoisomerase IIalpha, peptide YY, and thyroglobulin. Three and a half years postoperatively, multiple bone and breast metastases were found and anticancer chemotherapy was ineffective. The results in the present case indicate that an ovarian carcinoid tumor found to be 'atypical carcinoid' according to pulmonary carcinoid criteria or immunohistochemical staining (i.e. highly positive for topoisomerase IIalpha and Ki-67) may have a poor prognosis.

A 58-year-old woman with an 18-year history of primary biliary cirrhosis was admitted because of pneumococcal pneumonia. She was treated with antibiotics and mechanical ventilation. After the pneumonia improved, she developed severe watery diarrhea. Although vancomycin was administered enterally, the diarrhea persisted. She died of multiple organ failure within 16 days of the onset of diarrhea. An autopsy showed intracapillary cryptococci in the systemic organs, especially in the intestinal tract. The cause of diarrhea was considered to be extensive intestinal mucosal necrosis due to disseminated cryptococcosis. This is a rare case of cryptococcal infection manifesting as acute diarrhea.

Increased susceptibility to Rhizopus oryzae in diabetes was investigated using mice in which diabetic ketoacidosis was experimentally induced. All of the diabetic mice which had developed ketoacidosis died within four days after inoculation of R. oryzae and their serum UIBCs (Unbound iron binding capacity) were significantly lower than those of the normal control mice. This study suggested that the decreased serum UIBC produced by diabetic ketoacidosis enhances the growth of R. oryzae in vivo.

The authors report a case of a myxoid chondrosarcoma of the pineal region in a 37-year-old woman who presented with an intratumoral hemorrhage. Partial removal of the tumor in an initial surgery resulted in failure to establish a definitive diagnosis. The residual tumor enlarged after a second intratumoral hemorrhage 14 months after the onset of the first symptoms, and gross-total resection of the tumor was achieved in a second surgery. Histological and immunohistochemical findings after the second surgery were consistent with a diagnosis of myxoid chondrosarcoma. Radical excision of a tumor was considered to play an important role in the management of intracranial myxoid chondrosarcoma.

Background: Tumoral calcinosis (TC) is a disease of unknown etiology characterized by the presence of calcified masses in the juxta-articular regions of the extremities. Involvement of the cervical spine is very rare. In this report, the characteristics of TC of the cervical spine, including the clinical presentation, radiographic features, and surgical management are discussed. Case Description: A 90-year-old healthy female suffering from numbness of the upper extremities for 3 months presented with a 2-week history of progressive weakness of the lower extremities. A neurological examination revealed mild weakness and sensory impairment of the bilateral upper and lower extremities. Computed tomography (CT) scans demonstrated amorphous calcified masses posterior to the spinous process that extended into the interlaminar spaces of C3/4 and C4/5. The masses involved the posterior elements of C3-C4. Interestingly, CT scans performed 4 years earlier showed subtle calcification of a yellow ligament at C3/4 and C4/5. However, neither calcified masses nor bone erosion were observed. On magnetic resonance (MR) imaging, the mass showed hypointensity on T1- and T2-weighted images. The lesion was compressing the spinal cord and was resected surgically. The pathological findings were consistent with those of TC. The natural history of TC is not understood. However, this case suggests that calcified masses may progress within several years and that the bone around the mass may be involved. Postoperatively, residual masses may disappear spontaneously, while new bone is formed in the erosive lamina and facet. Conclusion: The treatment of choice for TC, if the lesion causes progressive symptoms, is surgical resection.

The pathogenesis of testicular yolk sac tumor (YST) of infants is still unclear. Infantile YSTs rarely show isochromosome 12p or aneuploidy, which are common in adult germ cell tumors. On the other hand, recent epigenetic studies suggest the involvement of some tumor suppressor genes, including the adenomatous polyposis coli (APC) gene. In the present study, we examined 10 infantile pure YSTs for mutation, allelic loss, promoter methylation, and protein expression status of the APC gene to evaluate whether the APC gene plays a significant role in the pathogenesis of infantile YSTs. Loss of heterozygosity at 5q21, where the APC gene is localized, was detected in at least 3 (30%) of the 9 YSTs examined. None of the 10 YSTs showed mutations. Promoter methylation was detected in 7 (70%) of the 10 YSTs; among 7 YSTs showing methylation, 3 YSTs also harbored loss of heterozygosity at 5q21. Immunohistochemically, 8 infantile YSTs did not express the APC protein, whereas 2 YSTs without showing APC methylation, as well as germ cells of normal infantile testes, expressed this protein in the cytoplasm. These data indicate that inactivation of the APC gene, by allelic loss and/or promoter methylation, is related to the occurrence of infantile YSTs.

An evaluating map of rubber roller for flexible media handling system, which can describe an operating condition of each rubber roller for well handling of flexible media, is introduced using two design parameters for the handling system such as speed of driven flexible media and applied load between rubber roller and drive media.Potential and durability of rubber roller for flexible media handling system can be evaluated by the range of operating condition for well handling of the paper in the map. The usefulness of the map is proven with the experimental results observed in handling system with rubber roller and paper.

To retrospectively evaluate the correlation between multidetector-row computed tomography findings of acute appendicitis and the pathological status of acute appendicitis, and evaluate the capability of multidetector-row computed tomography to predict the pathological status of acute appendicitis in children.The presence of a distended appendix (6 mm in transverse diameter) was used as a primary sign to indicate the presence of appendiceal inflammation. The presence of appendiceal wall thickening (1 mm) and enhanced appendiceal wall continuity were also used as predictive findings to reflect the degree of progression of acute appendicitis on multidetector-row computed tomography findings. The findings of each individual case were classified into four grades. The final pathological diagnosis was classified into four groups: normal findings, only mucosal inflammation, inflammation with intramural spreading, and gangrenous. The relationship between the pathological grades and computed tomography grades was analyzed using Spearman's rank correlation test.Four of six cases in Grade 0 reflected normal appendiceal findings (66.7%) and 3 of 5 cases in Grade I reflected only mucosal inflammation status (60.0%). Forty-four of 51 cases in Grade II reflected intramural inflammation status (86.3%), and 40 of 57 cases in Grade III reflected gangrenous status (70.2%). The multidetector-row computed tomography grade was significantly correlated to the pathological grade with Spearman's rank correlation coefficient of 0.689 (There was a close relationship between the multidetector-row computed tomography imaging findings and the pathological findings. This preoperative information is extremely useful for decision-making in the treatment strategy for acute appendicitis in children.

We report a case of myoclonus epilepsy associated with ragged-red fibers (MERRF)/mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episode (MELAS) overlap syndrome with hearing loss, external ophthalmoplegia, and myoclonus epilepsy in addition to stroke-like episode and diabetes mellitus. Pathologically, there was degeneration in the dentate nuclei, substantia nigra, red nucleus, and subthalamic nucleus which has been reported as characteristic of MERRF, as well as necrotic lesions of various stages in the cerebral cortex, characteristic of MELAS. The gene study disclosed 3243 mutation in the tRNALeu(UUR) gene of mitochondrial DNA. This case is the first neuropathological report of MERRF/MELAS overlap syndrome verified by gene analyses.

We report a case of hepatic infarction caused by the occlusion of both the hepatic artery and the superior mesenteric artery (SMA). In this case, we detected a thrombus at the left atrium as the embolic source by transesophageal echocardiography (TEE). A 53-year-old man was admitted to a local hospital with a diagnosis of renal infarction and atrial fibrillation. Seven days later he was transferred to our hospital because of complications from cerebral infarction. On the 2nd hospital day, a floating thrombus at the left atrial appendage was detected by TEE. On the 11th hospital day, he failed into shock and abdominal distension developed. Serum transaminase levels were markedly elevated. Computed tomography with a contrast agent showed a segmental non-enhancing lesion of the liver. At that time, TEE showed the disappearance of the left atrial thrombus. His condition worsened rapidly, and he died of multiple organ failure. Autopsy findings showed the thromboembolism of both the hepatic artery and the SMA, extensive liver infarction and massive intestinal necrosis. In this case, it is suggested that the obstruction of the hepatic artery and the SMA brought about a decrease in blood flow through the hepatic artery and the portal vein, and caused liver infarction.

A 81-year-old Japanese female was referred to our hospital with fever up, lumbago and marked splenomegaly (10cm below the left costal margin) without lymphadenopathy. Laboratory findings showed leukocytosis (60.5×109/l), mild thrombocytopenia (89×109/l) and M-proteinemia (total protein 8.1g/dl, IgG 5.469g/dl, IgG-λ positive). 92% of leukocytes were villous lymphocytes and the tartrate-resistant acid phosphate reaction was negative. The immunophenotypes of these cells were CD3-, CD5-, CD10-, CD11c+, CD19+, CD20+, CD22+, CD25-, FMC7+, MikB1+, sIgM+. The DNA analysis of the immunoglobulin gene of the peripheral blood showed rearrangement of JH gene. The first lumbar vertebra was fractured. We diagnosed splenic lymphoma with villous lymohocytes, started radiation to bone involvement and chemotherapy with VMCP regimen, but splenomegaly increased to the pelvis and leukocytosis persisted. Furthermore, serum electropheresis showed biclonal gammopathy (IgG-λ, IgM-λ), and the immunophenotypes of villous lymphocytes revealed IgG-κ and IgM-κ potitive.After two years she complained abdominal pain. Splenomegaly decreased rapidly and peripheral villous lymphocytes increased extremely to 150×109/l without blastic transformation. Abdominal computed tomography showed decreased splenomegaly with a few nodules of low density and slight ascites. Splenic infarction or rupture were not showed. Metabolic acidosis, hypoglycemia and paralytic ileus occured. She died next day.Autopsy showed systemic intravascular and severe extravascular infiltrations of the villous lymphocytes. The parenchyma of spleen was involved with severe lymphoid infiltration (μ+, γ+, α-, κ+, λ-) and white pulp was dissappeard. Bone marrow also showed the plasmacytoid cells (γ+ [>>μ+], λ+, κ-). Severe acute ischemic enterocolitis without thrombosis was seen in small intestine and colon. We suspect that rapidly increased villous lymphocytes came to peripheral circulatory insufficiency, but we couldn't know the relationship between decreased splenomegaly and rapidly increased villous lymphocytes.

Amyloid-β-related angiitis (ABRA) of the CNS occurs in association with vasculitis of small-and medium-sized leptomeningeal arteries. Here, we describe the clinicopathological features of a 76-year-old man with ABRA. The patient suffered progressive truncal oscillation, aphasia, and recent memory disturbance with a subacute disease onset. His cerebrospinal fluid showed a mild increase in protein levels (101 mg/dL) and pleocytosis (8/mm(3)). High-intensity brain lesion were detected on T(2)-weighted and FLAIR MRI scans, and prominent spread of gadolinium enhancement spreading was observed through the sulci of the left occipital and temporal lobes and left cerebellar hemisphere. A biopsy of the left temporal lesion showed a granulomatous and angiodestructive inflammation with infiltration of many CD4(+) T-lymphocytes and multinucleated giant cells and with fibrinoid necrosis of the arterial walls in the subarachnoid space. Immunolabeling for Aβ(1-40) revealed the abundant deposition of this protein in the affected arteries. On the basic of the diagnosis of ABRA, immunosuppressive therapy was conducted, and it ameliorated the clinical course.

A 79-year-old man with prostatic hypertrophy was performed trans-ureteral resection of prostate, and the transfusion of red cell concentrates stored in mannitol-adeninne-phosphate solution (RC-MAP) was required because of massive postoperative bleeding, despite of preparation for the autologous blood transfusion. On postoperative day 7 high fever developed, followed by widespread erythema, liver dysfunction, watery diarrhea and pancytopenia. Skin biopsy findings showed typical changes of transfusion associated graft-versus-host disease (TA-GVHD). Despite intensive treatment with methylpredonisolone, cyclosporine A, colony-stimulating factor and other supportive therapy, he died day 31 after surgery. The containing of foreign DNA fragments in his blood was detected by the analysis of microsatellite DNA polymorphism of his finger nail and peripheral blood.We emphasize that the transfusion of RC-MAP can cause TA-GVHD though the contaminated lymphocyte of RC-MAP is markedly reduced compared with the ordinary packed red cells.

A 64-year-old man with a 2-month history of abdominal distension was admitted for transient cerebral ischemic attack. A CT scan revealed massive ascites. Laparoscopy showed multiple whitish nodules on the visceral peritoneum and the omentum. Peritoneal biopsy revealed tumor cells consistent with malignant peritoneal mesothelioma (MPeM). Pemetrexed in combination with cisplatin was administered because it has been reported to be active in patients with MPeM. However his disease progressed. As second-line therapy paclitaxel was tried which yielded a complete response (CR). Eighteen months later he developed abdominal pain of the right upper region where a CT scan showed a mass with surrounding inflammation. As third-line therapy, gemcitabine was administered and again resulted in a CR. He is alive at 3 years from first presenting. Searches for case studies published in medical journals on MPeM were carried out, and 59 cases were analyzed in comparison with this case.

A 52-year-old woman presented with isolated thrombocytosis in 2003. After 5 years of observation under a tentative diagnosis of essential thrombocythemia (ET), she was referred to our hospital because of anemia and leukopenia. Bone marrow biopsy demonstrated increases of megakaryocytes and myelofibrosis, but splenomegaly was absent. A karyotype study of bone marrow detected t(9;22) (q34;q11.2) in 6 of the 20 metaphases studied. Peripheral blood neutrophil BCA-ABL fusion signals (FISH) were not detected. Because RT-PCR assay of bone marrow detected major-BCR-ABL mRNA (b3a2), treatment with imatinib (400 mg/day) was started. After transient thrombocytopenia, normalization of blood cell counts and improvement of myelofibrosis were achieved. JAK2 V617F mutation and M-BCR-ABL mRNA was negative in peripheral blood. Clinical and laboratory data suggest that this case represents a rare and atypical myeloproliferative neoplasm with BCR-ABL translocation restricted mainly to the megakaryocyte lineage.

Although primary carcinoid tumor of the ovary is an extremely rare neoplasm, survival is excellent if the disease is confined to one ovary. Herein, we present a case of primary strumal carcinoid tumor of the ovary, stage IA, borderline malignancy, in a 34-year-old woman. Histological findings of the right ovary indicated higher atypical nuclei, higher mitotic rate and focal necrosis of tumorous cells in some areas, findings that are compatible with atypical carcinoid of the lung. Immunohistochemical staining was positive for synaptophysin, neuron-specific enolase, chromogranin A, Ki-67, topoisomerase IIalpha, peptide YY, and thyroglobulin. Three and a half years postoperatively, multiple bone and breast metastases were found and anticancer chemotherapy was ineffective. The results in the present case indicate that an ovarian carcinoid tumor found to be 'atypical carcinoid' according to pulmonary carcinoid criteria or immunohistochemical staining (i.e. highly positive for topoisomerase IIalpha and Ki-67) may have a poor prognosis.

We report three patients who presented with high fever, anasarca, hepatosplenomegaly, lymphadenopathy and severe thrombocytopenia accompanied by reticulin fibrosis of the bone marrow. This constellation of symptoms is not compatible with any known disease entity, and we had difficulty in diagnosis and treatment. A 47-year-old woman was suspected of having splenic lymphoma and received one course of CHOP regimen followed by continued steroid therapy. Her condition was improved but repeatedly became exacerbated with tapering of steroid. A 56-year-old man was treated with steroid pulse therapy and splenectomy without improvement. Histology of the liver and spleen did not show any specific findings. Immunosuppressive therapy with cyclosporin A was successful. Another 49-year-old man showed histological findings of paracortical hyperplasia with vascular proliferation and atrophic germinal centers on inguinal lymph node biopsy. These findings were similar to those of the hyaline-vascular type of Castleman disease or POEMS syndrome, but non-specific. Although he received steroid pulse therapy, he died of multiple organ failure. Autopsy demonstrated cytomegalovirus infection and hemophagocytic histiocytosis without malignant lymphoma. We suggest that this constellation represents a new clinical entity belonging to systemic inflammatory disorders with a background of immunological abnormality, requiring prompt and vigorous immunosuppressive therapy.

Five patients with ovarian carcinoid who had severe constipation for a long period preoperatively showed marked reduction of this symptom postoperatively. Because this phenomenon was believed to be caused by some biologically active substance rather than a mechanical effect of the tumor, reactivity to 17 amine and peptide hormones was studied immunohistochemically in these patients. Numerous peptide YY (PYY)-positive cells were detected, with PYY-positive cells representing more than 50% of all carcinoid tumor cells in each patient. PYY, which has a pharmacologic inhibitory action on intestinal motility, was presumably the cause of the constipation in these patients.

A 37-year-old man with hemophilia A and an asymptomatic carrier of HIV infection developed convulsion in January 1989. Except for seborrhoic eczema on his face and head, the results of the physical examination were unremarkable. The white cell count was 2.3×103/μl with 14% lymphocytes. His CD4/CD8 ratio was 0.27 with 11.5% CD4 cells and 43.0% CD8 cells. Absolute numbers of CD4+- lymphocytes were 37/μl. A ring-shaped enhancing lesion with surrounding edema was disclosed in the right frontal region by computed tomographic scan. Cranial irradiation resulted in an increase of the mass and the development of left hemiparesis. Four pieces of cerebral tissue obtained by biopsy revealed necrosis with degeneration. Thereafter, he had a rapidly declining course, characterized by coma and pneumonia, and died in April 1989. Autopsy revealed cerebral toxoplasmosis, bronchopneumonia without pneumocystis carinii pneumonia, marked atrophy of systemic lymphnodes, and chronic hepatitis.

We report an autopsy case of angiocentric lymphoma initially manifested as lymph node infarction. The second biopsy obtained from the other lymph node showed a diffuse proliferation of various-sized lymphoid cells with cytologic atypia. Cellular infiltration to vascular walls accompanied by irregular necrotic areas was observed. Immunohistological study of the lymph node expressed the phenotype (CD3+CD8+CD4±), consistent with that of peripheral T-cell lymphoma, except that some large lymphoid cells were positive for B-cll specific antibody L26.The autopsy showed extensive lymphoma cell infiltration of the liver, spleen, kidneys, lungs, gastrointestinal tract, nasopharynx, and lymph nodes. Of interest, the angiocentric and angioinvasive nature of the lymphoma cell infiltration was evident, and necrosis of tissues was accompanied by the infiltration. These features are characteristic of angiocentric lymphoma, and retrospective studies suggest that the lymph node infarction was caused by angiocentric immunoproliferative lesions introduced by Jaffe in 1984.Southern blot analysis of DNA extracted from the lymph nodes of the second biopsy and of the autopsy showed germline configurations for T-cell receptor (TcR) β-chain, δ-chain and immunoglobulin heavy chain genes. Only TcR γ-chain exhibited multiple faint rearrangement bands, but this is compatible with polyclonal T-cell proliferation. The results obtained through our studies, in addition to the previously reported cases, suggest that certain types of peripheral T-cell lymphoma may lack clonal rearrangements of TcR genes.

The authors report a case of a myxoid chondrosarcoma of the pineal region in a 37-year-old woman who presented with an intratumoral hemorrhage. Partial removal of the tumor in an initial surgery resulted in failure to establish a definitive diagnosis. The residual tumor enlarged after a second intratumoral hemorrhage 14 months after the onset of the first symptoms, and gross-total resection of the tumor was achieved in a second surgery. Histological and immunohistochemical findings after the second surgery were consistent with a diagnosis of myxoid chondrosarcoma. Radical excision of a tumor was considered to play an important role in the management of intracranial myxoid chondrosarcoma.

For the speed sensor-less control in the ultra lower speed range, the mechanical simulator method is applied to cope with the problems due to the extremely lower induced voltage. In the method, however, the mechanical parameterspsila error, such as the error of the inertia, affects the starting performance. To reveal the conditions for the stable starting, we carry out the phase plane analysis under the condition that the actual inertia J changes from the set inertia Js. Consequently, we find out Js ges J is the condition for the stable acceleration. The derived condition is useful as a design index of the mechanical simulator. Additionally, to cope with the large variation of the inertia, we carry out the phase plane analysis of the IM simulator method which compensates the estimation error of the rotor frequency. The analysis reveals that the method enables the stable acceleration while the estimation error of speed is remained in the case of inertia variation. Furthermore, we propose the modified IM simulator method which eliminates the deviation. By the phase plane analysis and experimental tests, we confirm the effectiveness of modified method.

The authors study a basic and simple control strategy by managing the capacitor voltage for hybrid powered railway vehicle with a diesel engine EDLCs. In this paper, we first discuss the required capacity of EDLCs considering with the size and mass. Then, we propose a practical and simple method to manage the capacitor energy by changing the diesel engines output power according to EDLC voltage. The capacitance designing method and the proposed control method are verified by numerical simulations of train running, assuming a commercial line in Japan. We have concluded that the proposed method to decide the capacitance and the control method are available and this study contribute to promote environmental friendly transportation system.

Here we report a case of gastric cancer with diffuse abdominal wall invasion treated with weekly low-dose paclitaxel therapy. A 62-year-old male visited our hospital because of abdominal distention, prepubic tumor,and testicular hydrocele. Computed tomography revealed diffuse swelling of the abdominal wall and hydronephrosis of the right kidney. Upper gastrointestinal endoscopy demonstrated type 3' advanced gastric cancer. Pathological diagnosis of both gastric tumor and abdominal wall biopsy specimens was poorly-differentiated adenocarcinoma containing signet ring cell carcinoma. Low-dose paclitaxel (90 mg/body) was given once a week for 3 weeks. Abdominal wall swelling like cuirass disappeared after 2 courses of low-dose paclitaxel therapy. Nine repeated courses of this regimen have been given until now; the relapse of the abdominal wall invasion has not become apparent, and primary gastric lesion has been a stable disease. Diffuse abdominal wall invasion of gastric cancer like cuirass without ascites is a rare condition, and low-dose paclitaxel was very effective for this condition.

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of gamma-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.

A 72-year-old woman was admitted with left cervical lymphadenopathy, high fever, pancytopenia and liver dysfunction. Bone marrow aspiration showed infiltration of large atypical lymphoid cells and hemophagocytic histiocytes, thus suggesting a diagnosis of lymphoma associated hemophagocytic syndrome (LAHS). An abdominal CT scan revealed multiple low-density areas in the liver, and the patient's liver function rapidly deteriorated. Histologically, the cervical biopsy showed lymphoma cell infiltration with prominent necrosis and karyorrhectic debris. The lymphoma cells expressed CD3+, CD4-, CD8+, CD20-, CD56+/-, TIA-1+, granzyme B+, and EBER was positive using in situ hybridization. DNA analysis of the TCR beta and gamma chain gene with the Southern blot showed rearranged bands. These findings were compatible with those of EB-virus associated peripheral T-cell lymphoma. After chemotherapy with the THP-COP regimen, the patient's liver dysfunction improved rapidly, but she died from bacterial peritonitis due to perforation of a recurrent duodenal ulcer. Post-mortem examination of the liver showed multiple irregular massive necroses of the hepatocytes, where no lymphoma cell infiltration was present. Hemophagocytic histiocytosis was remarkable in the bone marrow, spleen, lymph nodes, and liver. Marked elevation of serum levels of cytokines such as TNF-alpha or IFN-gamma suggests that these cytokines played an important role in the pathogenesis of the hepatic cell necrosis.