Histopathological evaluation of aortic dissection: a comparison of congenital versus acquired aortic wall weakness.

OBJECTIVES: The aim of this study was to identify pathological changes of aortic dissection based on histopathological evaluation of aortic wall weakness by comparing patients with and without congenital abnormalities. METHODS: We reviewed records of patients who underwent repair for dissection-related aortic disease between 2008 and 2015. Fifty patients (20 men and 30 women; mean age 66.9 ± 14.0 years) who underwent surgery with subsequent histopathological examination of the aortic wall were divided into 2 groups. Group 1 had congenital abnormalities, including Marfan syndrome and bicuspid aortic valve (n = 5), and Group 2 had no congenital abnormalities (n = 45). We compared the histopathological characteristics of the aortic wall in these patients. RESULTS: There were significant differences in age and body surface area between the 2 groups. Although 80% of Group 1 patients developed dissection at the middle of the media, all Group 2 patients developed dissection at the outer one-third of the media, which is along the pathway of the vasa vasorum of the aortic wall. Both groups showed the same extent of degeneration of the vasa vasorum. Group 1 showed a severe score of mucoid extracellular matrix accumulation in the aortic media. CONCLUSIONS: Although it may be multifactorial, congenital maldevelopment of the media tends to result in dissection of the centre of the media, and acquired aortic wall weakness is concentrated in the outer third of the media. Degeneration of the vasa vasorum may be an important emerging substrate for developing aortic dissection.

Surgical resection of two independent primary intimal sarcomas in the left atrium.

Primary intimal sarcoma of the heart is an extremely rare tumor that is known to have a very poor prognosis. We present a case of a 65-year-old man who suffered from deteriorating congestive heart failure due to a severe mitral stenosis caused by a large mobile left atrial tumor. The patient underwent an emergency operation of the tumor in the left atrium. The tumor was attached to the inferior wall of the left atrium. After the resection of the tumor, a second tumor on the interatrial septum, which had not been detected in the preoperative investigation, was discovered and resected. The patient developed acute respiratory failure soon after the operation and succumbed to his illness. The appearance of the main tumor was cauliflower-like, which strongly suggested the possibility of malignancy. Immunohistochemistry revealed that the neoplastic cells were positive for vimentin, desmin, p16, and especially murine double minute 2 (MDM-2). The first tumor was CD34 positive and cdk4 negative, but the second tumor was more anaplastic and CD34 negative and cdk4 positive, which suggests a different origin of the two tumors. The two tumors were diagnosed as intimal sarcomas by MDM-2, which is currently considered a conclusive marker. This is an exceptionally rare case of two simultaneous and possibly independent primary intimal sarcomas in the left atrium.

Coronary artery bypass graft in a patient with Fabry's disease.

Fabry's disease is a lysosomal storage disease characterized by intracellular accumulation of ceramide trihexoside resulting from alpha-galactosidase A deficiency. While the heart is often involved, coronary artery disease and its management in Fabry's disease patients are extremely rare clinical entities. We report a case of a 72-year-old man with left main disease in Fabry's disease with special consideration of the arterial wall pathology.

Partial aortic root replacement for aneurysm of the right sinus of Valsalva.

An aneurysm of the sinus of Valsalva is clinically rare, and its operative indications and procedures are controversial. We herein report the rare case of a 68-year-old woman with severe right ventricular outflow tract obstruction caused by an aneurysm of the right sinus of Valsalva. We performed partial aortic root reconstruction using a bovine pericardial patch, and aortic valve replacement. Although this case provides evidence that these are suitable surgical techniques for treatment of aneurysm of the sinus of Valsalva, total aortic root replacement should have been chosen based on the pathological finding of aortic medial and valve degeneration.

Hematogenous umbilical metastasis from colon cancer treated by palliative single-incision laparoscopic surgery.

Sister Mary Joseph's nodule (SMJN) is a rare umbilical nodule that develops secondary to metastatic cancer. Primary malignancies are located in the abdomen or pelvis. Patients with SMJN have a poor prognosis. An 83-year-old woman presented to our hospital with a 1-month history of a rapidly enlarging umbilical mass. Endoscopic findings revealed advanced transverse colon cancer. computer tomography and fluorodeoxyglucose-positron emission tomography revealed tumors of the transverse colon, umbilicus, right inguinal lymph nodes, and left lung. The feeding arteries and drainage veins for the SMJN were the inferior epigastric vessels. Imaging findings of the left lung tumor allowed for identification of the primary lung cancer, and a diagnosis of advanced transverse colon cancer with SMJN and primary lung cancer was made. The patient underwent local resection of the SMNJ and subsequent single-site laparoscopic surgery involving right hemicolectomy and paracolic lymph node dissection. Intra-abdominal dissemination to the mesocolon was confirmed during surgery. Histopathologically, the transverse colon cancer was confirmed to be moderately differentiated tubular adenocarcinoma. We suspect that SMJN may occur via a hematogenous pathway. Although chemotherapy for colon cancer and thoracoscopic surgery for the primary lung cancer were scheduled, the patient and her family desired home hospice. Seven months after surgery, she died of rapidly growing lung cancer.

TNF inhibitors induce discoid fibrosis in the sublining layers of the synovium with degeneration of synoviocytes in rheumatoid arthritis.

We determined the characteristic features of synovial tissues of rheumatoid arthritis (RA) patients treated by TNF inhibitors in order to delineate their mechanism of action. Synovial tissues were obtained during the joint surgical operations from 12 RA patients who had been treated with TNF inhibitors in addition to disease modifying antirheumatic drugs (DMARDs) for at least 5 months (5-25 months) (RA-TNFinh), and from 12 RA patients who had been treated with DMARDs alone (RA-DMARD), and were evaluated under light microscopy. There were no significant differences in disease duration, serum CRP levels, DAS28, Steinbrocker's stages on X-ray and treatment regimen except for TNF inhibitors between RA-TNFinh and RA-DMARD. The most prominent changes in the synovium from RA-TNFinh were discoid fibrosis in the subliming layers of the synovium with degeneration and detachment of synoviocytes and marked decrease in vasculatures. There was no significant difference in these synovial features between RA patients with infliximab and those with etanercept. Interestingly, appearance of osteoclasts was observed in RA-TNFinh (3 out of 12 patients) and in RA-DMARD (1 out of 12 patients). These results indicate that not only infliximab, but etanercept might have direct actions on synovial cells in the deep lining layers of the synovium, leading to the discoid fibrosis thereof. Moreover, the data confirm that the deep lining or sublining layers of the synovium are the most important portions that steer the disease process of RA synovitis.

Aortic dissection in the outer third of the media: what is the role of the vasa vasorum in the triggering process?

OBJECTIVES: Extensive clinicopathological analyses of aortic dissection have implicated hypertension and genetic abnormalities as the major pathogenic mechanisms. However, previous findings from pathological examinations have often been inconsistent with these mechanisms. In this paper, we suggest a significant role for the vasa vasorum in the aetiology and pathogenesis of aortic dissection. METHODS: We reviewed records of patients who underwent thoracic aortic dissection repair at our institution between June 2008 and August 2011. Twenty-one patients (10 men, 11 women; mean age, 65.0 ± 12.0 years) underwent surgery with subsequent histopathological examination of the aortic wall. We evaluated the history and histopathological findings of these patients. RESULTS: Aortic medial changes were observed in all 21 patients. These changes included thinning and sometimes fragmentation of the elastic lamina, as well as atrophy of the smooth muscle cells, with surrounding accumulation of an Alcian blue-positive mucinous substance, mostly adjacent to the dissection. Importantly, the dissection was located in the outer third of the media in 20 of the 21 patients (95.2%). Of these 20, 18 showed histopathological evidence of sclerotic changes of the vasa vasorum, including muscular hyperplasia, elastosis, intimal fibrosis and/or luminal obstruction, and even rupture. These changes may have been secondary to hypertension or peri-aortic changes, leading to degenerative changes in the aortic media and even initiation of dissection. CONCLUSIONS: Most aortic dissections initially developed in the outer third of the media alongside the vasa vasorum. In this type of aortic dissection, dysfunction of the vasa vasorum may play a key role in long-standing ischaemia or malnutrition of the aortic media.

Histopathological analysis of cerebral hemorrhage in systemic lupus erythematosus complicated with antiphospholipid syndrome.

A 53-year-old woman was admitted due to akinetic mutism and fever. On admission, brain computed tomography (CT) scan revealed a large hemorrhagic lesion in the left anterior lobe that required surgical operation for total removal. Her neuropsychiatric manifestation was not attenuated after the operation, and was gradually ameliorated by high doses of corticosteroid thereafter, suggesting a diagnosis of lupus psychosis. Histopathological examination revealed a necrotizing vasculature, sometimes with a disrupted lamina elastica interna, completely filled with thrombi and infiltrated with inflammatory cells and their debris. It is therefore suggested that the large frontal lobe hematoma in our patient was induced by thrombosis and arteritis in the subarachnoid space.

[Distal arch aneurysm and acute aortic dissection with bicuspid aortic valve; report of a case].

A 49-year-old man was admitted to our institution with acute chest and back pain. Chest and abdominal computed tomography (CT) showed type A aortic dissection from the ascending aorta to the bilateral common iliac arteries and a distal arch aneurysm with a diameter of 65 mm. Echocardiogram showed a bicuspid aortic valve with severe regurgitation. We performed total arch replacement and aortic valve replacement. Pathological examination of the aortic wall revealed a typical cystic medial necrosis. After 3 months, we performed the 2nd stage operation for the distal arch aneurysm and descending aortic dissection. Here, we report a rare case of bileaflet aortic valve with aortic aneurysm and aortic dissection.

Analysis of CXCL9 and CXCR3 expression in a case of intravascular large B-cell lymphoma.

Intravascular large B-cell lymphoma is a rare disease with multiorgan involvement that also affects the skin. Skin manifestations include purpuric to red macules, plaques, or nodules with occasional edema and tenderness. We report a 68-year-old woman with bilateral leg edema and occasional high fever. A biopsy specimen from a subcutaneous nodule showed that the blood vessels in the dermis and subcutaneous tissue were filled with irregularly shaped chromatin-rich large atypical lymphocytes positive for CD20 and bcl-2, consistent with the diagnosis of intravascular large B-cell lymphoma. In addition, immunohistochemical analysis showed expression of CXCR3 in the atypical lymphocytes; its ligand, CXCL9, was detected in blood vessels. Although limited to a single case, our study could provide a possible new clue to the pathogenesis of intravascular large B-cell lymphoma by virtue of the characteristic expression of CXCL9-CXCR3.

Effect of G-1 column (Adacolumn) therapy in rats with adjuvant arthritis on the migration and immunoreactivity of peripheral and splenic leukocytes.

The G-1 column (Adacolumn), a novel extracorporeal adsorption device, is now available for the treatment of such chronic inflammatory diseases as ulcerative colitis and rheumatoid arthritis. G-1 column treatment sometimes results in a rapid decrease in clinical inflammatory parameters and/or has a delayed beneficial effect on disease activity. In order to identify the scientific basis for such clinical benefits, we studied rats with adjuvant arthritis induced by immunization with Mycobacterium butyricum antigen. The potential role of G-1 column treatment on the migratory properties and immunoreactivities of leukocytes was investigated. Treatment of arthritic rats for 60 min with an extracorporeal perfusion through the G-1 column led to the adsorption of a small proportion (20%) of circulating granulocytes and monocytes. However, after G-1 treatment, the migration of radiolabeled blood granulocytes and monocytes to sites of acute dermal inflammatory reactions decreased significantly, in the case of granulocytes, almost by half. The migration of granulocytes to the inflamed hindpaws of severely affected animals was diminished in the G-1 treated group. Granulocytes that have passed through the G-1 column may stay in the bloodstream because of their markedly diminished number of adhesion molecules. A slightly increased accumulation in the liver and a decreased localization in the lung was also observed. These results may be relevant to the rapid clinical anti-inflammatory effect observed in rheumatoid arthritis and possibly also in ulcerative colitis, without any pulmonary complications. In contrast, the adsorption rate by the G-1 column of T lymphocytes was very low, and their migration pattern to sites of dermal inflammatory reactions was not altered after treatment. However, the antigen (Mycobacterium purified protein derivative) reactivity of T lymphocytes in blood was almost completely abolished after G-1 column treatment of arthritic rats. This unexpected qualitative effect on T lymphocytes of G-1 treatment warrants further detailed study.