[Calcified Amorphous Tumor Diagnosed After Stroke:Report of a Case].

Calcified amorphous tumor (CAT), a non-neoplastic tumor, is rare. Histopathologic features are the presence of calcified nodules in an amorphous background of fibrin. CAT is reported to be associated with renal dysfunction or hemodialysis, and possibly causes cerebral embolism. We report a case of CAT diagnosed after stroke. A 58-year-old male with a 2-year history of hemodialysis was diagnosed with an acute stroke, and was treated medically. Paralysis promptly improved, but transthoracic echocardiography revealed a tumor attached to the posterior mitral leaflet and dense mitral annular calcification. To prevent embolism due to the large tumor, we performed resection of the tumor. Pathological findings showed calcifications surrounded by amorphous fibrous tissue, indicating CAT. Postoperative course was uneventful.

[Pulmonary Adenocarcinoma in Situ with Positive Findings in Pleural Lavage Cytology].

We report a rare case of positive findings in pleural lavage cytology(PLC) in the patient with pulmonary adenocarcinoma in situ (AIS). A 78-year-old woman was presented with a 30 mm pure groundglass nodule (GGN) in the left upper lobe on chest computed tomography (CT). After 2 years follow- up, thoracoscopic surgery was performed to resect the nodule. PLC was performed before pulmonary resection. Histopathological diagnosis was 25 mm AIS. However, PLC showed positive findings of malignant cells. CT examination at 1 year and 6 months postoperatively showed pleural dissemination findings and the patient died of lung cancer at 3 years and 2 months postoperatively. PLC's contribution to TNM staging has not yet been clarified. The positive findings in PLC and large size of pure GGN were considered likely to be poor prognostic indicators.

[Coronary Aneurysm with Fistula].

We successfully treated two rare cases of coronary aneurysm with fistula. Case 1;A 65-year-old female referred to our hospital with the coronary aneurysm and fistula. Right coronary aneurysm with fistula leading to coronary sinus was observed. Coronary bypass surgery using a saphenous vein to #4PD was performed, and two right ventricle branches were reconstructed. Coronary aneurysm was resected. Case 2;A 46-year-old male was admitted with chest discomfort. Coronary aneurysm with fistula from the left main trunk to left ventricle was demonstrated. Ligation of the coronary artery aneurysm and suture closure of the entry site to the left ventricle was performed. Both patients had uneventful recovery.

[Third Tricuspid Valve Replacement:Report of a Case].

A 79-year-old man underwent tricuspid valve replacement (TVR) with a bovine pericardial bioprosthesis, Carpentier-Edwards Perimount (CEP), for tricuspid regurgitation 27 years earlier. Twenty-one years after the first operation, he underwent re-TVR with a bovine pericardial bioprosthesis, Magna Mitral Ease, due to prosthetic valve dysfunction. Since the prosthetic valve dysfunction progressed again, the third TVR was performed six years after the second operation. The findings of the resected bioprosthesis showed sclerosis of the septal cusp, and pannus formation between the septal and posterior cusps. For the third TVR, the porcine bioprosthesis Epic was used. Neither transvalvular nor perivalvular leakage was observed postoperatively, and the patient was discharged without any complications.

[Papillary Fibroelastoma Arising from the Left Atrial Wall:Report of a Case].

Papillary fibroelastoma (PFE) is a rare benign cardiac tumor generally arising from the valvular endocardium. We report an extremely rare case of PFE arising from the left atrial wall. A 70-year-old male patient was admitted to our hospital with a diagnosis of left atrial tumor. Echocardiography and enhanced computed tomography showed an approximately 14 mm mass on the left atrial wall. Moreover, the magnetic resonance imaging showed hyperintenseness on a T2-weighted image. We diagnosed the tumor as a myxoma. Intraoperatively, we found a mobile tumor on the left atrial wall. It had a sea anemone-like appearance and was suspected to be PFE. We performed the tumor resection including the left atrial wall. Histological examination confirmed PFE. His postoperative course was uneventful.

[Pulmonary Non-tuberculous Mycobacteriosis Complicated with Pulmonary Large Cell Neuroendocrine Carcinoma;Report of a Case].

A 64-year-old man with pulmonary non-tuberculous mycobacteriosis(pulmonary NTM) who had been treated by antituberculous chemotherapy, developed a new nodule of 8 mm in size in the segment 3 of the right upper lobe. The cavity of 4.0 cm in size in the segment 1+2 of the left upper lobe due to Mycobacterium avium infection was preexisted. Radiologically, new nodule of the right lung was suspected to be lung cancer. Left upper lobe apical trisegmentectomy was performed at first. Three months later, enlarging of the right lung nodule with increased fluoro-2-deoxy-D-glucose(FDG) activity was noted, and the diagnosis of lung cancer was made by transbronchial lung biopsy(TBLB). Then, right upper lobectomy with systematic nodal dissection were performed.

[Anterior Mediastinal Myxoid Liposarcoma Detected by Chest Computed Tomogram in the Patient with Pneumothorax].

A 57-year-old woman visited our hospital with left chest pain. Chest computed tomography (CT) scanning showed left pneumothorax with apical bullae and a nodular shadow in the left anterior mediastinum accidentally. However, a week later, we could not detect a mediastinal shadow on chest CT image after healing of left pneumothorax. Video assisted thoracoscopic surgery was scheduled in order to remove bullae and evaluate an anterior mediastinal lesion. The mediastinal lesion was tumorous and resected with around pericardial fat tissue. Pathological diagnosis was a myxoid liposarcoma of 15×10mm in size without infiltration into the surrounding tissue. The postoperative course was uneventful without recurrence 6 months later.

Endothelin B receptor expression in malignant gliomas: the perivascular immune escape mechanism of gliomas.

In order to clarify the role of endothelin B receptors (ETBRs) in gliomas, we analyzed cell cultures and surgical specimens of gliomas using RT-PCR and immunohistochemistry. RT-PCR measured the absolute expression of ETBR mRNA in twelve samples, which included gliomas that were classified using the World Health Organization (WHO) classification system Grade I-IV, as well as two glioblastoma cell lines (CCF-STTG1 and U87-MG). Using immunohistochemistry, 77 glioma specimens were evaluated for their expression of ETBR and infiltrating T lymphocytes, including an analysis of cytotoxic T cells (CTLs) and regulatory T lymphocytes (Tregs). The number of ETBR-positive vessels in the glioblastomas (Grade IV) was significantly higher than in other grades of gliomas (comparisons to Grade IV, Grade I: p = 0.0323, Grade II: p = 0.0009, Grade III: p = 0.0273). The ETBR expression rate (defined as the number of ETBR-positive blood vessels divided by the total number of blood vessels) in the glioblastomas was higher than the ETBR expression rate in the low-grade gliomas (compared to Grade IV, Grade I: p = 0.0132, Grade II: p = 0.0018, Grade III: p = 0.0745). In addition, the cases which had an ETBR expression rate of 50 % or higher exhibited fewer infiltrating CTLs and more infiltrating Tregs compared to the cases with an ETBR expression rate <50 % (CTLs: p = 0.0342; Tregs: p = 0.0175). Isocitrate dehydrogenase 1 (IDH-1) mutations were identified in 21 cases, but there was no correlation between ETBR expression and IDH-1 mutations for any WHO grade. These results suggest that ETBR expression during neo-angiogenesis may interfere with the homing of CTLs around the tumor and be involved in the immune escape mechanism of gliomas.

Alternatively Activated Macrophages Play an Important Role in Vascular Remodeling and Hemorrhaging in Patients with Brain Arteriovenous Malformation.

BACKGROUND: Angiogenic and immunoactive lesions in brain arteriovenous malformation (BAVM) contribute to hemorrhagic events and the growth of BAVMs. However, the detailed mechanism is unclear. Our objective is to clarify the relationship between hemorrhagic events of BAVM and alternatively activated macrophages in the perinidal dilated capillary network (PDCN). METHODS: We examined microsurgical specimens of BVMs (n = 29) and focused on the PDCN area. Ten autopsied brains without intracranial disease were the controls. We performed immunostaining of the inflammatory and endothelial cell markers, macrophage markers (CD163 and CD68), and vascular endothelial growth factor A (VEGF-A). We evaluated each cell's density and the vessel density in the PDCN and analyzed the relationship to hemorrhagic events of BAVM. RESULTS: The PDCN was involved in all the resected arteriovenous malformations, and these vessels showed a high rate of CD105 expression (72.0 ± 10.64%), indicating newly proliferating vessels. Alternatively activated macrophages were found, with a high rate (85.6%) for all macrophages (controls, 56.6%). In the hemorrhagic cases, the cell density was significantly higher than that in the nonhemorrhagic cases and controls (hemorrhagic group, 290 ± 44 cells/mm(2); nonhemorrhagic group, 180 ± 59 cells/mm(2); and control, 19 ± 8 cells/mm(2)). The cell density of alternatively activated macrophages showed a positive correlation with the vessel density of the PDCN. Double immunostaining showed that VEGF-A was secreted by alternatively activated macrophages. CONCLUSION: Our data suggest that alternatively activated macrophages may have some relationships with angiogenesis of PDCN and hemorrhagic event of BAVM.

[Stanford Type A Aortic Dissection after Off-pump Coronary Artery Bypass Grafting Using Automated Proximal Anastomotic Device;Report of a Case].

Operative mortality of Stanford type A aortic dissection( AAD) repair with previous cardiac surgery is high, especially with previous coronary artery bypass grafting. We report an extremely rare case of AAD related to the PAS-Port system. A 68-year-old male patient on dialysis was admitted to our hospital with a diagnosis of AAD. Half a year before he had undergone off-pump coronary artery bypass grafting using PAS-Port system for a proximal anastomosis of a vein graft. Urgent graft replacement of the ascending aorta was performed, and the proximal anastomotic site of the vein graft was attached to the prosthetic graft. Intraoperative investigation revealed that an intimal tear was located on the anastomotic site made by the PAS-Port system. The postoperative course was uneventful and he has been visiting our hospital regularly for dialysis.

Primary meningeal melanocytoma of the sellar region: review of the literature and differential diagnosis with special reference to angiographical features.

PURPOSE: Primary intracranial melanocytomas are rare neoplasms, especially in the sellar region. Intracranial melanocytoma is usually a dural-based tumor, fed by dural arterial branches in a manner similar to meningioma. Primary sellar melanocytoma may be misdiagnosed as hemorrhagic pituitary macroadenoma, spindle cell oncocytoma, and intrasellar meningioma. These tumors differ in some radiological respects, but are difficult to differentiate preoperatively. METHODS: Only five cases of primary sellar/suprasellar melanocytic tumors, excluding melanomas have been reported thus far. In this paper, we report an instructive new case of a 31-year-old woman presenting with a 2-year history of amenorrhea and an intrasellar mass with suprasellar extension, suggestive of hemorrhagic pituitary adenoma. RESULTS: Transsphenoidal surgical excision was difficult due to extensive bleeding from the lesion, and at the time, the tumor could not be diagnosed histopathologically. Six years later, we operated again because of tumor regrowth. Angiography revealed a hypervascular tumor, which was fed from the dorsal sellar floor. We had difficulty resecting the tumor, but achieved total removal. Our case had typical radiographic characteristics of melanocytoma, revealed by both magnetic resonance imaging and angiography. However, it was difficult to reach a final diagnosis. Further histopathological examination, including immunohistochemical and ultrastructural studies, was helpful for diagnosis of melanocytoma. CONCLUSIONS: Primary sellar melanocytic tumors are derived from melanocytes in the meningeal lining of the sellar floor or in the diaphragm sellae, based on both embryological assumptions and the clinical findings of our case. We discuss the problems of differential diagnosis and management of primary sellar melanocytic tumors.

Neurogenic pulmonary edema following Cryptococcal meningoencephalitis associated with HIV infection.

Neurogenic pulmonary edema (NPE) is a clinical syndrome characterized by the acute onset of pulmonary edema following a significant central nervous system insult. Only a few cases of NPE after Cryptococcal meningitis have been reported. We report a case of NPE following Cryptococcal meningoencephalitis. A 40-year-old man with no medical history was hospitalized for disturbance of consciousness. Blood glucose level was 124 mg/dL. Non-contrast head computed tomography showed no abnormalities. Lumbar puncture revealed a pressure of over 300 mm H2 O and cerebrospinal fluid (CSF) confirmed a white blood cell count of 65/mm(3) . The CSF glucose level was 0 mg/dL. The patient was empirically started on treatment for presumptive bacterial and viral meningitis. Four days after, the patient died in a sudden severe pulmonary edema. Autopsy was performed. We found at autopsy a brain edema with small hemorrhage of the right basal ganglia, severe pulmonary edema and mild cardiomegaly. Histologically, dilated Virchow-Robin spaces, crowded with Cryptococci were observed. In the right basal ganglia, Virchow-Robin spaces were destroyed with hemorrhage and Cryptococci spread to parenchyma of the brain. No inflammatory reaction of the lung was seen. Finally, acute pulmonary edema in this case was diagnosed as NPE following Cryptococcal meningoencephalitis. After autopsy, we found that he was positive for serum antibodies to human immunodeficiency virus.

[Surgical Treatment for Mycotic Aortic Aneurysms].

Mycotic aortic aneurysms are relatively rare. Diagnosis and treatment may sometimes be difficult. We have treated 5 cases of mycotic aortic aneurysms in our institution. The patients were 4 males and 1 female with a mean age of 65.6±16.7 years old. All patients had vascular diseases and complained of fever. Preoperative blood cultures were positive in 4 cases. The strategy for treatment of mycotic aortic aneurysms consisted of removal of infective focuses as much as possible and revascularization using an artificial graft combined with omentopexy. Administration of antibiotics was continued for 6 months. All patients have been alive without recurrence of infections for a mean period of 22.6 months, which was considered to be an acceptable result. Artificial graft replacement combined with omentopexy and long-term administration of antibiotics is suggested to be useful for the treatment of mycotic aortic aneurysms.

CD5-positive follicular lymphoma characterized by CD25, MUM1, low frequency of t(14;18) and poor prognosis.

CD5-positive follicular lymphoma (FL), although rare, has been described in a number of case reports. However, a statistically valid, clinicopathological comparison between CD5-positive FL and CD5-negative FL has never been performed because of its rarity. We statistically compared clinicopathological characteristics of 22 cases of CD5-positive FL, diagnosed by immunohistochemistry, flow cytometry and morphological findings, with those of 62 cases of FL without CD5 expression (control cases). CD5-positive FL patients showed a higher tendency of peripheral blood involvement (P = 0.076) and a higher frequency of CD25 expression (P = 0.0004) and MUM1 protein expression (P = 0.0008), and a lower frequency of t(14;18)(q32;q21) (P = 0.017). The overall survival (OS) curve of CD5-positive FL was significantly worse than that of control cases (P = 0.0266), although progression-free survival curves did not show a significant difference (P = 0.7899). Moreover, CD5 expression was shown to be an independent poor prognostic factor for OS in both univariate analysis [Hazard Ratio (HR), 3.63; P = 0.0464] and multivariate analysis (HR, 57.16; P = 0.0001). CD5-positive FL showed different clinicopathological characteristics from FL lacking CD5 expression. These results suggest that CD5-positive FL should be considered a different type of FL, and its clinicopathological management should be conducted differently.

Intraoperative rapid diagnosis of primary central nervous system lymphomas: advantages and pitfalls.

To study the advantages and pitfalls of intraoperative rapid diagnosis (IRD) of primary central nervous system lymphomas (PCNSL), pathology reports and frozen sections in our institution were reviewed. We examined 27 cases of PCNSL, one case of anaplastic glioma, and one case of metastatic brain tumor that were diagnosed on neuroimaging. Fifteen cases of intraoperative cytological preparations were also reviewed in a correlative manner. Among the 27 cases initially diagnosed as PCNSL, 18 were also diagnosed as PCNSL by IRD. However, IRD identified four of the 27 cases as gliosis, two as demyelination, one as atypical epithelial cells, one as malignant glioma and anaplastic astrocytoma. In addition, the case identified as metastatic brain tumor on neuroimaging was corrected to a diagnosis of PCNSL based on IRD. The final accuracy of IRD in the present study was 89.6% (26/29). After postoperative definitive diagnosis, two cases of anaplastic astrocytoma and one case of PCNSL by IRD were corrected to PCNSL, anaplastic oligodendroglioma and demyelination, respectively. PCNSL were sometimes histologically indistinguishable from malignant gliomas or demyelinating diseases in the present study, particularly in frozen sections. Notably, all cases for which both intraoperative cytology and frozen section were performed concomitantly were correctly diagnosed in the present study. In particular, lymphoglandular bodies were highly characteristic cytological findings of PCNSL. Both intraoperative cytology and frozen sections should therefore be performed concomitantly when PCNSL are suspected.

Ependymosarcoma with eosinophilic granular cells.

Ependymosarcoma is a new entity of malignant gliomas composed of ependymal and sarcomatous components. Were port a rare case of ependymosarcoma with eosinophlic cells which occurred to the right trigon of the lateral ventricle.A 62-year-old man complained of headaches over a 2-month period. A hard, gray mass was found in the right trigon of the lateral ventricle during the operation.Although he received radiation and chemotherapy, the patient died due to tumor disseminating through the whole brain within 7 months after the operation. The histological examination revealed that the anaplastic glial components intermingled with the sarcomatous components. Immunohistochemically, sarcomatous cells were positive for α smooth muscle actin and desmin. However, anaplasticglial cells were not positive for these markers. In addition, Masson trichrome stain showed a plethora of collagen fibers between sarcomatous cells, but no collagen fibers were produced by the glial tumor cells. Solid focal papillary lesions of the glial tumor showed dot-like epithelial membrane antigen and diffuse cytoplasmic D2-40 immunoreactivity. Based on the above findings, these anaplastic glial tumor cells should show focal ependymal differentiation, and sarcomatous cells show myofibroblastic differentiation. In addition, almost 10%of the tumor cells in the neoplasm showed bright eosinophilic granules in the cytoplasm. These cytoplasmic eosinophilic granules and bundles were negative on PAS staining. Intracytoplasmic eosinophilic granules of tumor cells were strongly positive for αB-crystallin, HSP 27 and GFAP, respectively. These findings suggest that the clinicopathological characteristics of the present case should be consistent with the criterion of ependymosarcoma by Rodriguez et al.

[Therapeutic use of amiodarone against postoperative fibrillation after cardiac surgery].

We investigated the effect of amiodarone (AMD) administered intravenously for the treatment of post-operative atrial fibrillation( POAF) after cardiac surgery. After rapid administration of 150 mg for 10 minutes, AMD was administered in 1.0 mg per minute for 6 hours followed by 0.5 mg per minute for 18 hours. AMD was then administered orally 200 mg per day for 1 or 2 weeks. Twenty-five cases were enrolled from January 2010 to June 2013. In 18 cases(72%), the patients were successfully defibrillated by this protocol. In the other 7 cases, the patients required electrical defibrillation, but in 1 patient developed chronic atrial fibrillation. It was thought that AMD could be a choice for POAF.

[Treatment strategy for patients with simultaneous cardiac and malignant diseases].

The choice of treatment strategy for patients with simultaneous cardiac and malignant diseases is often debatable. We report 11 patients with simultaneous cardiac and malignant diseases who were hospitalized in our institution over the past 6 years. The patients were 9 males and 2 females with a mean age of 71.9±9.0 years old. There were 7 cases of ischemic heart disease( IHD), and 4 cases of valvular disease. Malignant diseases consisted of 4 cases of colon cancer, 2 cases of pulmonary cancer, 2 cases of hepatic cancer, and 1 case each of sigmoid colon cancer with liver metastasis, gallbladder cancer, and pancreatic cancer. Eight of the 11 cases(73%)were hospitalized because of a symptom associated with cardiac disease, and malignant disease was diagnosed incidentally during examination or treatment. Four of the IHD cases preceded coronary revascularization by off-pump coronary artery bypass grafting (OPCAB) or percutaneous coronary intervention (PCI). Two of the valvular cases and 2 of the IHD cases underwent cardiac surgery after treatment of malignant diseases. In 3 cases, only 1 of the diseases was treated. Seven cases died of malignant diseases at the present. The choice of treatment strategy should be established for patients with simultaneous cardiac and malignant diseases in consideration of the severity of the cardiac disease and the expected prognosis of the malignant disease because there is no standardized method of treatment.

Methotrexate/iatrogenic lymphoproliferative disorders in rheumatoid arthritis: histology, Epstein-Barr virus, and clonality are important predictors of disease progression and regression.

OBJECTIVES: Patients with rheumatoid arthritis (RA) may develop lymphoproliferative disorders (RA-LPD). Immunosuppressive states due to methotrexate (MTX) and Epstein-Barr virus (EBV) reactivation have been regarded as causes. Sometimes spontaneous regression occurs after withdrawal of MTX. The objective of this study was to identify factors predictive of relapse and survival in patients with RA-LPD, and spontaneous regression in patients with RA-LPD treated with MTX (MTX-LPD). METHODS: We evaluated the clinicopathological features, clinical characteristics, and treatment outcomes in 102 cases of RA-LPD. In addition, EBV infection and clonality of immunoglobulin heavy chain gene (IGH) were analyzed by in situ hybridization and polymerase chain reaction, respectively. RESULTS: The 102 cases included patients with diffuse large B-cell lymphoma (DLBCL; n = 53), Hodgkin lymphoma (n = 9), polymorphic B-cell LPD (n = 20), reactive lymphadenitis (n = 11), peripheral T-cell lymphoma (PTCL; n = 4), composite lymphoma (n = 2), and follicular lymphoma (n = 3). EBV was detected in 60% (56/93) of patients. Spontaneous regression occurred in 59% (28/47) of patients in whom MTX was withdrawn. Regression was associated with EBV positivity (P = 0.007) and non-DLBCL (P = 0.006), but not with MTX amount and other clinical features. Monoclonal bands of IGH were observed in 31 of 74 cases. In patients with DLBCL, poor disease-free survival (P = 0.05) was associated with clonality of IGH. In all patients, factors predictive of shorter survival were age (>70 yr) and histological type of DLBCL. CONCLUSIONS: Histology, EBV positivity, and monoclonality of IGH are useful for predicting clinical outcomes in patients with RA-LPD.

Anaplastic astrocytomas with abundant Rosenthal fibers in elderly patients: a diagnostic pitfall of high-grade gliomas.

To investigate the clinicopathological features of anaplastic astrocytoma (AA) with abundant Rosenthal fibers (RFs), this study assessed four cases of AA (elderly patients; age ≥ 70 years). Histologically, these tumors were composed of diffusely infiltrating astrocytomas with brightly eosinophilic cytoplasmic granules or cork-screw or beaded bundles. Tumor cells showed pleomorphism, bizarre giant cells, and mitotic activity, but no necrosis. The cytoplasmic granules showed negativity on PAS staining. Immunohistochemically, the tumor cells with cytoplasmic granular cells showed a positive reaction for GFAP. The cytoplasmic eosinophilic granules or bundles were positive for αB-crystallin, ubiquitin and HSP27. In addition, tumor cells showed strong cytoplasmic positivity for isocitrate dehydrogenase 1 (IDH1)-R132H protein in all cases. The MIB-l labeling index of these cases ranged from 7% to 10%. In cases 1 and 2, ultrastructurally, the tumor cells had electron-dense, amorphous structures in the cytoplasm and in the processes. These structures were bound to glial intermediate filaments. Based on these microscopic, immunohistochemical and ultrastructural findings, case 1 was diagnosed as AA with abundant, mixed, common type of RFs and miniature (m) RFs, and cases 2,3, and 4 were diagnosed as AA with abundant mRFs. These results indicate that the presence of RFs in astrocytic tumors does not necessarily exclude a diagnosis of high-grade astrocytoma. In addition, AAs with abundant mRFs in elderly patients should be classified as a peculiar variant of AA.