Marfan syndrome with aortic dissection and triple-barrel aorta.

A case of Marfan syndrome with spontaneously and subsequently developed dissections of the aorta, one in the form of triple-barrel aorta, three times corrected by grafts is described. The autopsy revealed "healed" and acute dissections in almost the entire aorta outside the grafts. "Healed" thoracoabdominal dissection had true lumen (with entry and re-entry intimal tears), and old false lumen and in addition in its distal portion of a triple-barrel aorta was formed (dissection of healed aortic dissection). Lethal adventitial rupture occurred in the portion with an old false lumen. Dissection of the left subclavian artery, the right common carotid artery, resulting in saccular aneurysm, and avulsion of the right renal artery were also found.     

The clear cell variant of epithelioid intravenous leiomyomatosis of the uterus: Report of a case

A case of very rare uterine clear cell epithelioid leiomyomatosls Is reported. The patient presented with a recurrent pelvlc mass after hysterectomy 2 years earlier. The recurrent tumor was located mainly In the vessels of the broad llgament as worm-like plugs. The revlewed hysterectomy speclinen showed a typical multi-lobuiated intramural mass together with worm-like plugs wlthin the myometrlal vessels. Computer tomography exhlbited metastatic nodules In both lungs. According to an histologlcal examlnatlon, all the tumor cells were of epltheliold type and contalned abundant clear cytoplasm. Characteristic large, thick-walled blood vessels were observed. Immunohisto-chemical staining and ultrastructural examinatlon sup ported smooth-muscle origin In this case. Electron mlcrosmplc study revealed that the clear cytoplasm was attributed to the presence of numerous dllated mitochondria that had lost their crlstae. The patient is still alive wlth the disease 28 months after surgery.     

Coarctation of the abdominal aorta--case report with autopsy

An autopsy case of coarctation of the abdominal aorta incidentally found in an 80-year-old male was presented. The stenotic lesion extended below the level of renal artery to the bifurcation and showed elongated hypoplastic type. Histological examination revealed adventitial fibrosis, vasa vasorum with occlusive change, degeneration and disappearance of the medial elastic fibers, and initial fibrosis. These features of lesser degree could also be found in the rest portion of the aorta and its branching arteries. It seemed reasonable to speculate that the inflammatory process worked on as the etiologic factor in the present case. The microscopic features had some resemblance to Takayasu's disease, but the macroscopic figures were quite unusual for it. Etiological possibility of aortitis in the present case was discussed.     

冠状动脉外膜炎症在动脉粥样硬化病灶形成中的作用

目的：探讨动脉外膜炎症诱发载脂蛋白E基因剔除（apoE-/-）小鼠冠状动脉粥样硬化病灶发生的机制。 方法： 取apoE-/-小鼠心脏做连续切片，选择冠状动脉外膜有炎性细胞浸润的3类部位代表粥样硬化病灶形成过程中的3个阶段：①未发现粥样硬化病灶；②有直接从主动脉延伸的病灶顶端和③成熟粥样硬化病灶的冠状动脉。分别采用HE染色、Movat染色、免疫组化和透射电镜方法鉴定3个病变阶段的冠状动脉外膜中炎细胞类型。 结果： ①未发现粥样硬化病灶；②有直接从主动脉延伸的病灶顶端和③成熟粥样硬化病灶的冠状动脉外膜分别以巨噬细胞、中性粒细胞和淋巴细胞浸润为主，其构成比分别为60.00%、57.65%和66.67%，各组构成比分别与其它两组比较均有显著差异（P<0.01）。 结论： 动脉外膜炎症可能是诱发动脉粥样硬化发生的早期事件之一，ApoE-/-小鼠冠状动脉粥样硬化病灶形成过程中，动脉外膜经历了一个从急性炎症到慢性炎症的过程。     

高血压大鼠大小动脉血管重构的差异比较

目的:探讨自发性高血压大鼠(SHR)大、小动脉血管重构的差异。方法:以20周龄雄性SHR为实验组,同龄Wistar-Kyoto(WKY)大鼠为对照组。每周测量体重和血压,43周龄大鼠麻醉取血,留取胸主动脉和肠系膜小动脉组织。HE染色观察比较大、小动脉形态学变化,天狼星红-维多利亚蓝染色检测胶原纤维和弹力纤维变化,激光扫描共聚焦显微镜和Western blot分析Ⅰ、Ⅲ型胶原蛋白的表达,透射电子显微镜观察动脉超微结构变化,原位末端标记法(TUNEL)和增殖细胞核抗原(PCNA)检测评估血管壁细胞凋亡与增殖情况。结果:小动脉血管内径(ID)和血管腔横截面积(LCSA)减小,壁厚内径比(WT/ID)和壁腔横截面积比(WCSA/LCSA)增大,外膜成纤维细胞增殖并部分迁移,胶原增多,以III型胶原增多为主,血管壁细胞的增殖指数和凋亡指数增加;主动脉ID、LCSA、WT/ID和WCSA/LCSA均增大,中层血管平滑肌细胞(VSMCs)肥大增生明显,胶原增多,血管壁细胞的增殖指数和凋亡指数增加。结论:高血压大鼠大、小动脉血管重构差异明显,小动脉以基质增多,特别是外膜III型胶原蛋白增多为主,血管壁细胞凋亡增加;大动脉以血管壁细胞增生,特别是VSMCs增生、肥大为主。     

Adventitial infiltrates associated with advanced atherosclerotic plaques: structural organization suggests generation of local humoral immune responses

Advanced atherosclerotic lesions often contain adventitial lymphoid infiltrates, which occasionally contain nodular aggregates resembling lymphoid follicles. The structural organization suggests that local maturation of B cells may take place at these sites, as described for the mucosa-associated lymphoid tissue (MALT). This concept was evaluated by studying the micro-anatomy and cellular composition of adventitial infiltrates associated with advanced atherosclerosis of the aorta. Sections of 22 atherosclerotic aortas were stained immunohistochemically for cellular markers characteristic for lymphoid follicles, such as HECA-452-positive endothelial cells, CD20-positive B cells, CD21-positive follicular dendritic cells, and CD68-positive macrophages. Ki-67 was used as a proliferation marker. The TUNEL technique was used to study the presence of apoptotic cells. Specimens containing MALT served as comparison and positive controls. Seven of the 22 atherosclerotic aortas contained adventitial infiltrates resembling lymphoid follicles. The organized nodular centres were composed of CD45RA+ B cells, follicular dendritic cells (CD21+), a few T lymphocytes (CD3+) and 'tingible body' macrophages (CD68+). A large number of cells were Ki-67-positive; apoptotic bodies were numerous and phagocytosed by macrophages. The parafollicular area contained CD45RO-positive T cells and HECA-452-positive vessels. Vessels elsewhere were always HECA-452-negative. Specimens with MALT showed similar features. This study reveals a close resemblance between adventitial lymphoid infiltrates in advanced atherosclerotic aortic disease and MALT, suggesting local generation of a humoral immune response, likely to be initiated by antigens released during a process of long-standing tissue injury and inflammation as part of advanced atherosclerosis.     

Use of histomorphometry in the assessment of fatal vertebral artery dissection.

AIM--To assess morphometrically the structural changes, which occur with ageing, along the length of the vertebral artery. METHODS--A series of 36 vessels were removed at necropsy from subjects aged between 9 months and 86 years. Image analysis was used to measure the medial width, the circumference, the intimal: medial area ratio and the adventitial: medial area ratio along each artery. The artery from a case of fatal vertebral artery dissection, which occurred after a game of cricket and then chiropractic neck manipulation, was also examined in the same manner. The proteoglycan accumulation in the media was quantified using an eyepiece graticule. RESULTS--The vertebral arteries were, on average, larger around the origin of the vessel from the subclavian artery, and the adventitia were relatively thicker at this point, and also after piercing the dura mater. The media were much thinner within the intracranial segment and pronounced intimal thickening occurred with increasing age. The dissected artery showed undoubtable pre-existent structural abnormalities, in the form of massive proteoglycan accumulation, which predisposes an artery to dissection. CONCLUSIONS--These data should help pathologists faced with the task of assessing the underlying structural integrity of the vessel wall in cases of vertebral artery injury.     

Sudden death from coronary artery dissection.

Spontaneous dissection of the coronary arteries is an uncommon condition that may lead to sudden, fatal coronary artery occlusion. Three cases of sudden death attributable to coronary artery occlusion are presented. Dissection was associated with Marfan's syndrome in the first case, and occurred three weeks postpartum in the second case. In case 1, dissection occurred within the intima, and was not associated with an inflammatory cell infiltrate. In cases 2 and 3, dissection occurred between the tunica media and the external elastic lamina, and was associated with a mixed inflammatory infiltrate, rich in eosinophils, T lymphocytes, and histiocytes. The spatial limitation of the inflammatory infiltrate to the adventitial compartment, together with the absence of inflammation in case 1, suggests a reactive origin rather than a causative role for the inflammatory cells. Detailed examination of serial blocks of any coronary artery occlusion is essential in young patients.     

Perivascular injury leads to a reduction in vascular reactivity of the collared and to an enhancement on contralateral carotid artery of rats.

BACKGROUND: The first response to perivascular injury is observed in the adventitial layer. The purpose of this study was to determine the time course of vascular reactivity alterations after collar injury in rats. We also analyzed the relation between adventitial layer injury and vascular responsiveness to vasoconstrictor agents. METHODS: Wistar rats had a silicone collar positioned around the carotid artery. The ipsilateral and contralateral arteries were morphologically analyzed 4, 7, 14 and 28 days after injury, and cumulative concentration-response curves to phenylephrine (Phe), angiotensin II (Ang II) and KCl were obtained for arteries isolated from collared and sham-operated groups. RESULTS: Inflammatory cells and fibroblasts were observed in the adventitial layer of collared arteries 4, 7, 14 and 28 days after injury. Intimal thickening was observed in collared arteries only 14 and 28 days after perivascular injury. A decrease in maximum effect values (Emax) for Phe, Ang II and KCl was observed in the collared artery when compared with the contralateral artery at all times after injury, whereas an increase in vascular responsiveness was observed in the contralateral artery 4 days after injury. CONCLUSIONS: The impairment of the contractile response preceded the intimal thickening. The compromise of vascular reactivity coincided with the presence of inflammatory cells and angiogenesis in the adventitial layer. The enhancement of the efficacy and potency of Ang II and Phe in collared-contralateral arteries 4 days after collar placement may be related to a receptor-mediated compensatory mechanism stimulated by the collar injury.     

Adventitial inflammation: a possible pathogenic link to the instability of atherosclerotic plaque

A variety of cells, including fibroblasts, mast cells, macrophages, and ganglionic cells, are present in coronary artery adventitia. In the infarct-related coronary arteries of myocardial infarction patients, the majority of mast cells are found in the outer layer of the adventitia. Neurogenic stimulation of mast cells in the adventitia of coronary arteries may release vasoactive compounds, such as histamine and leukotrienes, which can contribute to the complex neurohormonal response that leads to abnormal coronary vasoconstriction. Lymphocytes and bacteria are also present mainly in the adventitial layer. Chlamydia pneumoniae is directly involved in the development of adventitial and plaque inflammation (pan-arteritis), leading to plaque rupture. Adventitial O(2)(-) may also play an extensive role in the control of vascular tone. Therefore, adventitial inflammation may play a pivotal role for atherosclerotic lesion development and atheroma instability.     

Synchronous and metachronous multicentric squamous cell carcinomas in the upper aerodigestive tract

A rare case Is presented of a 57-year-old Japanese male wlth synchronous and metachronous multicentric squamous cell carcinomas (SCC) In the upper aerodlgesthre tract. Durlng a 9-year-perlod from the appearance of first primary SCC to autopfky, 14 focl of prlmary SCC and one severe dysplasla developed In wccesalon In the thoraclc esophagus, oral floor, soft palnte, uvula, lingual radix, pirlform recess, hypo-phsrynx, cervlcal esophagus, trachea and lingual body. The patlent dkd of severe bronchopneumonla due to Gram-nsgatlve bacterlal lnfectlon that developed as a result of recurrent nerve paralysls. Human papllloma vlrus and Epsteln-Barr virus, which are risk factors, were not detected by lmmunohlstochemlalry or by the polymerase chaln reaction method. Genetlc analysis revealed the absence of polnt mutations In K-ras codon 12. Heavy consumptlon of alcohol and excesstve smoklng may have been responsible for the mulcentrk carclnogenesls. Thls Is the first case report In the llterature of the development of so many prlmary SCC leslons In the upper aerodlgeuwe tract during such a short period.     

p53 protein overexpression and K-rascodon 12 mutation in pancreatic ductal carcinoma: Correlation with histologic factors

The correlation of p53 protein overexpression and the K-ras codon 12 mutatlon wlth histologlc type, grade of cytologic atypla, depth of lnvasion and other histologlc prognostic factors was studied In paraffin sectlons from 43 ductectatic-and 70 solid-type pancreatic ductal carcinomas. Overexpression of p53 was found in 23.3% (10143) of ductectatic carcinomas (17.2% of intraductal and 35.7% of lnvaslve carclnomas) and in 61.4% (43/70) of solid carcinomas. In ductectatic cancers, p53 overexpression was detected In 14.8% (4/27) of carcinomas wlth lowgrade atypla (CAL), 50.0% (5110) of carcinomas wlth high-grade atypla (CAH) and in 16.7% (In) of mixed low- and hlgh-grade cancers. In the last group, expression was restricted to an area of CAH. In solld cancers, p53 overexpression did not dlffer by histologic type or grade. Overexpresslon of p53 and K-ras mutatlons did not correlate with histologlc prognostic factors (lymphatic, venous and perineural Invasion, and lymph node metastasls) in ductectatlc and solld cancers or depth of invasion of solld carclnomas. Our data suggest that p53 alteratlon occurs at an early intraductal stage of solld carcinoma, irrespectlve of cellular atypla, but Is low in ductectatic CAL and becomes hlgher In ductectatlc CAH. K-ras mutatlon, present In a high percentage of tumors of all groups and not correlating with the factors above, showed no changes In frequency with tumor progression.     

Focal endothelial cell injury in rabbit aorta, aggravation of injury by 2 days of cholesterol feeding.

A randomized morphometric study of "spontaneous" endothelial cell injury in rabbit aorta was performed blindly. Four different areas of the aorta were sampled in a standardized fashion and examined by scanning electron microscopy. The finding of protruding endothelial cells was taken as sign of injury. The endothelial cell injury was focal and, in the sampled areas, particularly prevalent in the distal part of the lesser curvature of the aortic arch and the distal lip of intercostal artery orifices. In the area between two intercostal artery orifices on the same side of the midline injured cells were only exceptionally found. Rabbits fed a 2% cholesterol diet for 2 days showed an increase in protruding cells, particularly at the intercostal artery orifices. It is concluded that the endothelial cell injury in rabbit aorta may be caused by local factors, determining the focal nature, and by systemic factors, such as acute hypercholesterolemia.     

Collateral circulation in experimental coarctation of the aorta in minipigs: a possible association with hypertrophied vasa vasorum

Experimental coarctation of the thoracic aorta was produced in 13 adult Yucatan minipigs by placing a gradually expanding "C-shaped" occluder around the aorta. The aortic constriction was standardized by measuring the blood pressure above the coarctation, with a mean arterial pressure of 165-170mmHg as the target. The pigs were humanely killed after 8 weeks of hypertension. No deaths were associated with constriction of the aorta, and neither rear limb weakness nor neurological dysfunction was noted. The lack of clinical signs in these animals suggested the development of an extensive collateral circulation. Collateral vessels, which were verified angiographically, included intercostal arteries, muscular intercostal arteries, and the internal thoracic artery. In two of the animals in which the thoracic aorta was completely occluded, revascularization also occurred via small arterial channels. Histologically, there was a marked increase in the number of vessels as well as significant hyperplasia and hypertrophy of smooth muscle of adventitial vasa vasorum. These findings support the hypothesis that collateralization of occluded thoracic aortas may be aided by hypertrophy of the vasa vasorum. To our knowledge, these vessels have not previously been shown convincingly by histopathological methods to revascularize occluded large arteries such as the aorta.     

Mucinous adenocarcinoma arising in a giant urachal cyst associated with pseudomyxoma peritonei and stromal osseous metaplasia

An unusual urachal lesion, which Is a muclnous adenocarcinoma arising in a giant urachal cyst and is associated with pseudomyxoma peritonei and stromal osseous metaplasia of the cyst wall, was examlned in a 45-year-old male. m cyst was encapsulated, measured 22×20 ×20 cm and weighed 3800 g. The unilocular cavlty was filled with much. Most of the cystic cavity was lined with tall, slmple or stratifled columnar eplthellum wlth a focus of papillary provon into the cavity. These findings suggest that this cystic lesion represents cystadenoma rather than a simple cyst. Foci of lnvasive moderately differentiated mucinous adenocarclnoma were detected in the area of macroscopic papillary fronds. Carcinoembryonic antigen and CA19–9 were immunohistochemically positive for tumor cells and their serum levels were also elevated. Stromal dystrophic calcification was extensively observed In the cyst wall with focl of osseous metaplasia. Muclnous Implants, whlch histologically demonstrated adherent muclnous masses without epithellal components on the surface, were observed in the pelvic and abdominal cavity, lndicating pseudomyxoma peritonei.     

Primary aorto-esophageal fistula due to Takayasu's aortitis.

BACKGROUND: Aneurysmal dilatation in Takayasu's arteritis is a recognized complication; however, fistula formation, especially to the esophagus, is very rare. METHODS: A 22-year-old male presented with severe hematemesis. Investigation by means of esophagogastroscopy and CT scan revealed a saccular aneurysm in the proximal descending aorta with communication to the esophagus. The patient was taken to theater, the aneurysm excised and replaced by a graft. RESULTS: Gross examination of the aneurysm showed multiple points of outpouching from the aneurysm. Histopathological examination of the showed marked intimal fibromyxoid thickening, loss of outer medial muscle and elastic fibers and marked fibrosis of the adventitial layer. The histological features were in keeping with Takayasu's arteritis. No evidence of tuberculosis was noted. CONCLUSIONS: This case illustrates an unusual complication of Takayasu's arteritis, in the form of a fistula between the aorta and the esophagus, which resulted in massive hematemesis and the ultimate demise of the patient.     

Spontaneous disruption of mycotic aneurysm involving innominate artery

We report a case of ruptured mycotic aneurysm involving innominate artery requiring an urgent surgical treatment. A 62-yr-old woman presented with fever and dyspnea. Previously, she was diagnosed with colon cancer and received right hemicolectomy and one cycle of adjuvant chemotherapy. On echocardiogram, pericardial effusion was noted and emergency pericardiocentesis was performed. CT scan revealed aortic aneurysm involving ascending aorta and innominate artery, and thrombi surrounding those structures. Patch repair of the defect in the ascending aorta and ringed Goretex graft to bypass the innominate and ascending aorta were performed. We believe that this is the first case of ruptured mycotic aneurysm involving innominate artery.     

Retroesophageal right subclavian artery: a case report and review of the literature

BackgroundVariations of vessels arising from the aortic arch are numerous. One of the common anatomical variations is the right subclavian artery originating as the last branch of the aortic arch. This is a report of a case of an adult male cadaver with a retroesophageal right subclavian artery.ObjectiveTo highlight the significance of a retroesophageal right subclavian artery, especially its clinical and surgical implications.MethodIs a report of a case of an anomalous vessel found during routine student dissection of the chest region in a male cadaver.ResultThe retroesophageal subclavian artery was seen originating as the last branch from the postero-lateral aspect of the thoracic aorta at the vertebral level T4. The heart was normal with no other vascular variations seen in this region.ConclusionAnatomists and pathologists mainly encounter a retroesophageal right subclavian artery by chance and is usually described as asymptomatic, but several clinical conditions have been associated with its occurrence. This is a clear example of when knowledge of an anatomical variation is helpful in clinical practice.     

损伤家兔动脉后外膜成纤维细胞增殖、表型变化与TGF-β1表达的关系

目的:观察损伤家兔动脉后外膜成纤维细胞增殖、表型变化与TGF-β1表达的关系。方法:采用免疫组化、透射电镜和原位杂交技术,观察兔腹主动脉损伤后外膜细胞增殖、细胞表型、超微结构和TGF-β1 mRNA表达水平的变化。结果: 损伤后3 d和7 d血管外膜PCNA 阳性细胞显著增多, 14 d接近正常。外膜细胞在损伤后逐渐获得α-actin表达 ,3 d呈弱阳性,7 d和14 d呈强阳性改变。损伤后7 d和14 d外膜细胞发生了显著的超微结构改变:大量的微丝出现和粗面内质网明显扩张,呈现出肌成纤维细胞的特征。损伤后3 d,外膜开始出现TGF-β1 mRNA表达,7 d和14 d表达持续上调,至28 d表达开始下调。 结论: 提示动脉损伤后外膜成纤维细胞增殖水平和表型变化与TGF-β1表达水平具有相关性。     

血管紧张素Ⅱ对血管外膜成纤维细胞亚群转化的影响

目的:探讨血管紧张素Ⅱ(Ang Ⅱ)对大鼠胸主动脉血管外膜成纤维细胞亚群转化的影响。方法:采用克隆环法进行血管外膜成纤维细胞的单克隆培养,逆转录聚合酶链反应(RT-PCR)和免疫荧光染色方法鉴定细胞纯度;随机分为对照组和Ang Ⅱ(10~1 000 nmol/L)组,采用荧光定量聚合酶链式反应(FQ-PCR)、免疫荧光染色和Western blot法检测α-平滑肌肌动蛋白(α-SMA)的表达情况。结果:克隆环法获得血管外膜成纤维细胞2个亚群:圆形细胞亚群和纺锤形细胞亚群。自分型后,从第3代至第8代,纺锤形细胞亚群的α-SMA表达量有减少趋势,而圆形细胞亚群的α-SMA表达量显著增多。在Ang Ⅱ诱导下,纺锤形细胞亚群和圆形细胞亚群的α-SMA表达量均增多,且圆形细胞亚群随Ang Ⅱ浓度(10~1 000 nmol/L)的增加,α-SMA的表达量显著增多(P0.01)。Western blot实验结果显示,Ang Ⅱ能刺激圆形细胞亚群更多地转化为肌成纤维细胞。结论:Ang Ⅱ能不同程度地影响外膜成纤维细胞亚群的分化能力,进一步揭示出2个细胞亚群在血管重构过程中扮演不同角色。