Primary fibroxanthosarcoma of the thoracic aorta

Summary A case is presented of a 58-year-old female with a primary malignant tumor of the aorta. The lesion was diagnosed histologically as a fibroxanthosarcoma and represents the first primary neoplasm of the aorta so classified. The gross and microscopic features of the lesion are described and the morphologic characteristics of previously reported aortic sarcomas are briefly reviewed and examined in light of the current histologic criteria for soft tissue tumors.     

附子水煎剂对家兔离体主动脉血管张力的影响

背景：附子为毛莨科乌头子根的加工品,具有温肾回阳,回阳救逆和温里散寒之功效。研究发现其具有明显的强心、降压、扩张血管、镇痛、抗炎及毒性等作用。目的：观察附子水煎剂对主动脉环的舒张作用及机制。方法：采用家兔离体主动脉平滑肌标本,以去甲肾上腺素(10^-6 mol/L)预收缩主动脉后给予0.5,1.0,2.0,4.0,8.0 g/L附子水煎剂观察其张力变化。然后将不同浓度的附子水煎剂干预的家兔离体主动脉平滑肌加入去除血管内皮、给予一氧化氮合酶抑制剂左旋硝基精氨酸      (1×10^-4 mol/L)、甲烯蓝(1×10^-5 mol/L)、环氧酶抑制剂吲哚美辛(1×10^-5 mol/L)和β肾上腺素能受体阻断剂普萘洛尔(1×10^-5 mol/L),探讨它们对血管张力变化的影响。结果与结论：附子水煎剂对血管环静息张力无明显影响,但不同剂量的附子水煎剂可使10^-6 mol/L 去甲肾上腺素预收缩血管产生明显舒张。去除血管内皮、给予左旋硝基精氨酸或给予甲烯蓝可减弱附子水煎剂对血管的舒张作用,但吲哚美辛和普萘洛尔对此无影响。另外,4 g/L附子水煎剂对无内皮细胞血管环去甲肾上腺素和KCl的量效收缩反应无明显影响。提示附子对主动脉的舒张作用是内皮依赖性,与内皮细胞释放的一氧化氮有关,而与平滑肌细胞膜上的受体依赖性Ca²⁺通道和电压依赖性Ca²⁺无关。中国组织工程研究杂志出版内容重点：组织构建;骨细胞;软骨细胞;细胞培养;成纤维细胞;血管内皮细胞;骨质疏松;组织工程     

Malignant fibrous histiocytoma

Summary The ultrastructural findings in 17 cases of malignant fibrous histiocytoma (MFH) are described. The tumors consisted of fibroblast-like cells and histiocyte-like cells in different proportions in different cases. Intermediate, undifferentiated, xanthomatous and multinucleated giant cells were also identified. In 12 of 17 cases myofibroblasts were evident. Acid phosphatase activity was detected cytochemically in the Golgi zone, endoplasmic reticulum and lysosomes (GERL) mainly within histiocyte-like cells, in three cases. These observations indicate that the GERL of the tumor cells are engaged in the formation of lysosomes. The polymorphic cellular composition, including undifferentiated cells, lends support to the concept that the MFH originates from a primitive multipotent undifferentiated mesenchymal cell.Supported by Grants-in-aid for Cancer Research from the Ministry of Health and Welfare (52-1), and from the Ministry of Education, Science and Culture (401057) of Japan and by a Grant from the Fukuoka-ken Anti-Cancer Association. 1976Presented at the 69th Annual Meeting of the Japanese Pathological Society on June 26, 1980, Sapporo     

Malignant hemangiopericytoma and other sarcomas with hemangiopericytoma-like pattern

This clinicopathologic study concerns 19 cases of malignant hemangiopericytoma among 755 cases of soft tissue sarcomas. The age of the patients ranged from 18 to 76 years, with a median of 43 years. Tumors occurred on the trunk in 8, lower extremities in 5, the head in 3, and the retroperitoneum in 3. According to follow-up information, nine of the 19 patients had died. Histologically the tumor was characterized by its homogeneous vascular pattern, its uniform cell population and a wide range of cellular anaplasia. After extensive sampling of the tumors, a comparative light microscopy revealed differences in diagnostic histology between malignant hemangiopericytoma and other soft tissue sarcomas with a hemangiopericytoma-like vascular pattern. The frequency of appearance of such pericytoma pattern in different soft tissue sarcomas was as follows: 4/4 cases (100%) in extraskeletal mesenchymal chondrosarcoma, 11/14 (79%) in infantile fibrosarcoma, 27/45 (60%) in synovial sarcoma, 62/201 (30%) in malignant fibrous histiocytoma, 9/37 (25%) in malignant schwannoma and 6/72 (8%) in liposarcoma.     

原发性十二指肠恶性肿瘤临床影像病理学研究

目的　探讨原发性十二指肠恶性肿瘤的临床表现、病理学特征影像学诊断及治疗方法 .方法　对 82例原发性十二指肠恶性肿瘤患者的临床资料进行回顾性分析 .结果　本组患者发病部位乳头区 5 3例、降段 18例 .临床表现依次为上腹疼痛 5 7例、黄疸 5 3例、消化道出血 4 1例 .乳头区肿瘤多表现为腹痛、黄疸 ,乳头区以外肿瘤常则多表现为腹痛、黑便及腹部包块 .病理学类型以腺癌多见 (72例 ) ,平滑肌肉瘤 (5例 )、恶性淋巴瘤 (3例 )次之 .结论　原发性十二指肠恶性肿瘤以上腹痛、黄疸、消化道出血为主要表现 ,但缺乏特征性 .病理类型以腺癌为主 ,好发于十二指肠乳头区及降段 .CT、B超和内镜检查是主要诊断手段 .手术切除是原发性十二指肠恶性肿瘤的主要治疗方法 ,早期诊断是改善患者预后的关键     

Epithelioid Malignant Schwannoma

A case of epithelioid malignant schwannoma (EMS) is reported. The tumor arose in the left radial nerve at the axillary fossa of a 65-year old male. A few months after resection of the primary axillary tumor, several intrapulmonary metastases appeared. Microscopically, the primary tumor showed highly cellular areas of polygonal or rounded cells, resembling lymphoma or melanoma, while the metastatic tumors revealed cord formation or rows, resembling carcinoma. Immunohistochemical studies showed that some of these tumor cells contained S-100 protein. Ultrastructurally, these tumor cells revealed delicate cytoplasmic projections, which contained bundles of microfilaments. However, the tumor cells did not have melanosomes. Varying amounts of basal lamina material surrounded the tumor cells. From the above features, we obtained a correct diagnosis of EMS. Acta Pathol. Jpn. 32: 195∼202, 1989.     

Malignant myoepithelioma of the breast metastasizing to the jaw

A breast tumor in a 52-year-old female was interpreted as a malignant myoepithelioma based on morphological and immunohistochemical studies. The tumor consisted of elongated cells with clear cytoplasm and lacked glandular components. The tumor cells were stained positively for keratin, S-100 protein, glial fibrillary acidic protein (GFAP) and muscle-specific actin. Distant metastasis in the right jaw developed 8 years after the initial surgery and the metastatic deposit showed a similar morphology and immunoreactivity. Myoepithelial tumors are generally considered as benign or low-grade lesions and distant metastasis has been rarely documented. The present case presents the possibility of delayed occurrence of distant metastasis in myoepithelial tumor of the breast. Received: 22 January 1999 / Accepted: 18 May 1999     

Malignant coarctation of the aorta

A case of primary malignant fibrous histiocytoma of the aorta is described.     

C-Kit-positive metastatic malignant pigmented clear-cell epithelioid tumor arising from the kidney in a child without tuberous sclerosis

We report the first pediatric case of malignant pigmented epithelioid clear-cell tumor arising from kidney; the lesion occurred in a 12-year-old girl without tuberous sclerosis. The tumor was widely metastatic to the retroperitoneum and chest, and the patient died of the disease 9 months after diagnosis, despite active chemotherapy. Pigmented epithelioid clear-cell tumor of the kidney is a rare variant of epithelioid angiomyolipoma and a member of the family of perivascular epithelioid cell tumors (PEComas). The tumor demonstrated overlapping features between clear-cell sugar tumor and epithelioid variant of angiomyolipoma. Tumor cells were positive for HMB-45 expression, negative for any evidence of muscular differentiation, and contained melanin pigment and premelanosomes in the cytoplasm. Diffuse C-Kit (CD117) positivity was identified throughout the tumor. This is the first report of C-Kit–positive malignant PEComas.     

Concomitant changes in endothelial cell junctions and extracellular matrix components in the chick embryo aorta

Summary According to previous studies, a process of endothelial activation seems to be occurring in the chick embryo between days 7 and 18. Also, endothelial cells respond to collagen as a substratum between 12 and 18 days, and this response diminishes until it almost disappears after birth. In the present study, aortas from chick embryos (days 7 to 21), and from chicks (14 days posthatching) were used. The results obtained by the freeze-fracturing technique, showed that between days 12 and 14 the intramembranous particles were aggregated into linear or clustered arrays in the fracture P-face of endothelial cells. This could signify that some kind of gap junction-like coupling may occur between adjacent endothelial cells. Our results also indicate that in advanced stages (21-day-old chick embryos and 14-day-old chicks) the growth of small aggregates into larger aggregates or plaques could occur. In addition to gap junctions, the presence of macular and linear tight junctions, reported as focal tight junctions (day 14 of development) macular and linear tight junctions with free-ending strands orientated parallel to one another (21 days) and smooth contoured ridges (14 days posthatching) were observed. This sequence of changes may represent a development from linear to macular, to a more occluding arrangement, and may also reflect an endothelial cell polarization. Histochemical study of proteoglycans was done by using cuprolinic blue according to the critical electrolyte concentration method. Cuprolinic blue-positive granular, elongated and microfibrillar materials were found in the subendothelial region, forming a meshwork that occupies the extracellular space. Qualitative and quantitative changes were observed both in proteoglycans and in other extracellular matrix components throughout development, suggesting an increase in extracellular matrix complexity. These results lead us to suggest that the assembly of a more complex extracellular matrix, concomitantly with the formation of intercellular junctions during development, might influence the polarization of endothelium in the aorta of the chick embryo.     

主动脉处的血流动力学特性及其生理意义

主动脉结构复杂成三维螺旋状,在心脏收缩期该处流动成旋动流态。这种旋动流态也许有着积极的生理意义,能够通过抑制湍流、促进物质传输,从而抑制心血管疾病的发生。引起主动脉处的旋动流主要是左心室收缩的扭曲运动、主动脉的三维空间几何构型、主动脉处的脉动流特征以及主动脉的运动。基于以上研究结果,近年来,研究者们以期将旋动流原理应用于心血管介入治疗和心血管介入器械的设计中。本文就主动脉处的旋动流态观察、其生理意义、引起该旋动流态的原因、及旋动流在临床器械设计中的应用等进行综述。     

Malignant pleuropulmonary epithelioid hemangioendothelioma – Unusual presentation of an aggressive angiogenic neoplasm

Epithelioid hemangioendothelioma (EHE) of the lung and pleura are rare tumors. Primary pleural EHE are associated with aggressive behavior and poor clinical outcome. Recent advances in EHE include the development of immunohistochemical markers of vascular differentiation, namely Fli-1, and the identification of a specific chromosomal translocation (t(1;3)(p36;q25)). We present a 19 year old male patient with a rapidly progressive malignant neoplasm that presented as a multinodular lung parenchymal process, with associated pleural effusion and thickening. Pathologic examination revealed a pleuropulmonary neoplasm with cytologic features including round to oval epithelioid cells with frequent cytoplasmic vacuoles. Immunohistochemistry confirmed vascular differentiation of tumor cells (CD34, CD31 and Fli-1 positive). While the cytologic features were suggestive of EHE, the aggressive nature of the neoplasm, with disseminated pleuropulmonary involvement, raised the question of whether the neoplasm should be classified as EHE or epithelioid angiosarcoma. Here, we review the clinicopathologic characteristics of pleuropulmonary EHE and the overlap between malignant EHE and angiosarcoma.     

Malignant angioendotheliomatosis (Angiotropic lymphoma) of the gallbladder

Summary We present a case of malignant angioendotheliomatosis of the gallbladder, the first reported. Diagnostic problems connected with this rare malignancy are underlined. Immunohistochemical studies were useful in providing further evidence of the lymphoid nature of the neoplasm and avoiding possible misdiagnosis. We suggest that the term angiotropic lymphoma might be more appropriate to define this malignancy.     

Unexpected sclerosing mediastinitis involving the ascending aorta in the setting of a multifocal fibrosclerotic disorder

Sclerosing mediastinitis is an uncommon condition associated with infections, inflammatory diseases, autoimmune disorders, and malignancies. It predominantly affects the trachea, superior vena cava, pulmonary veins, and pulmonary arteries. We herein present the first case of sclerosing mediastinitis involving the ascending aorta as part of a multifocal fibrosclerotic disorder.     

铁抑制血管舒张活动及其作用机制

目的和方法:循环系统内铁的增加可能是导致冠脉粥样硬化病人血管功能损伤的原因之一。然而,目前关于铁对内皮依赖性血管舒张功能的影响尚不明确。本文采用血管环灌流装置,观察铁对离体SD大鼠胸主动脉环舒张功能的影响。结果:(1)主动脉环用100μmol/L枸橼酸铁(FAC,含Fe³⁺)孵育后,可显著降低乙酰胆碱(ACh)产生的内皮细胞依赖性主动脉环的舒张作用。主动脉环用FAC孵育之前,补充一氧化氮(NO)的前体物质L-精氨酸(L-Arg),ACh(>10^-9mol/L)所致的血管舒张幅度与单纯铁孵育组相比无明显差异。(2)FAC孵育后,L-Arg引起的内皮完整主动脉环舒张幅度显著减小(P<0.05),而无内皮的主动脉环用FAC孵育后,对NO的供体—硝普钠产生的血管舒张作用无明显影响。(3)二甲基亚砜对FAC抑制ACh血管舒张的作用无明显影响;在ACh浓度为(10^-9、10^-6、3×10^-6)mol/L时,过氧化氢酶可显著增加FAC孵育后血管的舒张幅度(P<0.01);还原型谷胱甘肽(GSH)可明显对抗FAC对ACh血管舒张作用的抑制。(4)正常大鼠主动脉环中NOS活性为(56.49±2.49)×10³U/g蛋白,FAC处理组大鼠主动脉环中NOS活性低至(25.15±5.75)×10³U/g蛋白,两者有显著差异(P<0.05)。结论:铁可能通过降低胸     

Malignant histiocytosis

Summary Diagnosis of malignant histiocytosis (MH) was confirmed in 16 patients. Stage at diagnosis was I–II in nine, and III–IV in seven patients. Poor prognosis and B-symptoms were correlated to advanced stages. Bone marrow biopsy proved most useful to verify organ involvement. Scintigraphy and computerized tomography, too, detected organ involvement in some patients and were helpful for judging response to therapy. Relapses after radiotherapy were frequent. Polychemotherapy using CHOP-combination is recommended for most patients and may in stages I–II be supplemented by primary or secondary involved or extended field irradiation and in more advanced stages by mainbulk-irradiation. The value of prophylactic CNS-therapy remains controversial. Pathophysiological aspects and differential diagnosis are discussed.