[Initial Recurrence around the Hilar of the Liver Which Was Difficult to Differentiate from Perihilar Cholangiocarcinoma with Portal Vein Invasion after Resection for Breast Cancer-A Case Report].

A 63-year-old woman underwent mastectomy and axillary dissection for right breast cancer(cT4bN1M0, Stage ⅢB, scirrhous carcinoma, moderately positive for ER, PgR negative, and HER2 negative)following neoadjuvant chemotherapy. She received no adjuvant therapy. A follow-up computed tomography 3 years later showed a soft tissue mass around the hilar bile ducts and mass in segment 6 of the liver. Based on these imaging findings, a diagnosis of perihilar cholangiocarcinoma with liver metastasis was made. She received chemotherapy with gemcitabine plus cisplatin, followed by S-1 monotherapy. Two years after the initiation of chemotherapy, an increase in the size of the liver mass and duodenal stenosis due to peritoneal dissemination were detected. Gastro-jejunal bypass was performed and a biopsy of the disseminated peritoneal mass supported a histologic diagnosis of breast cancer. The patient then received chemotherapy for breast cancer for 1 year. However, she eventually died due to the progression of the peritoneal dissemination. Although initial recurrence around the hilar of the liver is extremely rare after resection for breast cancer, when a new lesion is detected after breast cancer surgery the diagnosis and initial treatment should be made with the possibility of breast cancer recurrence in mind, based on the clinicopathological findings and the risk of recurrence.

Acute psychiatric symptoms in a young woman with anti-N-methyl D-aspartate receptor encephalitis: A case of successful early diagnosis and therapeutic intervention.

This clinical image presents a report on the diagnosis and treatment of anti-NMDAR encephalitis, a rare disease. This report emphasizes the importance of a differential diagnosis for acute psychiatric symptoms. Accurate and timely diagnosis is critical for the selection and implementation of treatment and for optimal patient outcomes.

Correction to: Multi-institutional re-evaluation of prognostic factors in chromophobe renal cell carcinoma: proposal of a novel two-tiered grading scheme.

The legends of Figs. 1 and 3 in the published original version of the above article are incorrect.

[Case of Ductal Carcinoma In Situ of the Nipple in a 93-Year-Old Man].

We report the case of an elderly male patient with ductal carcinoma in situ(DCIS) of the nipple. A 93-year-old man visited the hospital because of pain and bleeding in and swelling of the right nipple. A benign tumor was suspected, but a definite diagnosis could not be made before surgery based on echo and cytology findings; thus, a malignant tumor could not be ruled out. He underwent partial mastectomy combined with the areola and nipple for diagnosis and treatment. Histologic examination confirmed the diagnosis of DCIS of the nipple. The surgical margin was negative. At 6 months after the surgery, he was doing well with no evidence of disease in the absence of postoperative adjuvant therapy. Thus, clinicians should consider breast carcinoma of the nipple as a differential diagnosis when an elderly man presents with swelling of the nipple.

Multi-institutional re-evaluation of prognostic factors in chromophobe renal cell carcinoma: proposal of a novel two-tiered grading scheme.

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.

Classic Chromophobe Renal Cell Carcinoma Incur a Larger Number of Chromosomal Losses Than Seen in the Eosinophilic Subtype.

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.

Assessment of a simple method of heart weight estimation by postmortem computed tomography.

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.

Use of indocyanine green fluorescence imaging to determine the area of bowel resection in non-occlusive mesenteric ischemia: A case report.

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.

[Brain Metastasis of Triple Negative Breast Cancer after Pathological Complete Response to Neoadjuvant Chemotherapy - A Case Report].

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.

[A Case of Squamous Cell Carcinoma with Negative Conversion of ER Status after Endocrine Therapy in an Elderly Woman].

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.

Diversification of mitochondrial genome of Daphnia galeata (Cladocera, Crustacea): Comparison with phylogenetic consideration of the complete sequences of clones isolated from five lakes in Japan.

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.

Contrast-enhanced multidetector-row computed tomography can predict pathological findings of acute appendicitis in children.

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 < 0.001). Conclusions: 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.

Tumoral calcinosis involving the cervical spine.

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.

[Amyloid beta-related angiitis: brain lesions showing leptomeningeal gadolinium enhancement on MRI and characteristic surgical pathologic features].

Amyloid-ß-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 Aß(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.

Abdominal actinomycosis masquerading as an omental tumor in a 12-year-old female.

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.

[A case of malignant peritoneal mesothelioma successfully treated with systemic chemothrapy].

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.

The effectiveness of postmortem multidetector computed tomography in the detection of fatal findings related to cause of non-traumatic death in the emergency department.

OBJECTIVES: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

[Atypical myeloproliferative neoplasm with a small population of Philadelphia chromosome-positive clones in the bone marrow].

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.

Contribution of bone morphogenetic protein-2 to aortic valve calcification in aged rat.

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, ß-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.

An autopsy case of disseminated cryptococcosis manifesting as acute diarrhea in a patient with primary biliary cirrhosis.

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.