血管淋巴样增生伴嗜酸性粒细胞增多症临床病理观察

目的：探讨血管淋巴样增生伴嗜酸性粒细胞增多症(angiolymphoid hyperplasia with eosinopilia, ALHE)的临床病理学特征、免疫组织化学染色(immunohistochemical stain,IHC)及鉴别诊断。方法：对3例ALHE进行临床病理分析及免疫组化研究,并进行文献复习。结果：2例ALHE为女性患者,1例为男性患者,病变位于头面部真皮内,组织形态以小到中等大小血管增生为主,血管内衬胞浆丰富的上皮样内皮细胞,血管周围见大量炎细胞浸润。免疫组化染色显示CD34、CD31、CD3阳性,CD20及CK阴性。结论：ALHE是一种少见的、良性的血管瘤,诊断主要依靠特征性组织学特点。     

胸腺原发黏膜相关淋巴组织淋巴瘤及淋巴上皮性涎腺炎样胸腺增生的临床病理分析

目的探讨胸腺原发黏膜相关淋巴组织(mucosa associated lymphoid tissue,MALT)淋巴瘤和淋巴上皮性涎腺炎(lymphoepithelial sialadenitis,LESA)样胸腺增生的临床病理学特征、两者相关性及鉴别诊断。方法分析3例胸腺MALT淋巴瘤和1例LESA样胸腺增生的临床病理学和免疫表型特征,并复习相关文献。结果 3例胸腺MALT淋巴瘤,其中2例伴Sj9gren综合征;镜下胸腺正常结构损毁,增生的淋巴滤泡间可见肿瘤性淋巴样细胞浸润伴明显的淋巴上皮病变,以中心细胞样和单核样B细胞形态为主。瘤细胞表达CD20、PAX-5和BCL-2,其中1例伴显著浆细胞分化者Lambda轻链限制性表达。3例胸腺MALT淋巴瘤免疫球蛋白(immunoglobulin,Ig)基因检测均示单克隆性重排。LESA样胸腺增生镜下胸腺分叶状结构大体尚存,可见包含增生滤泡的丰富淋巴细胞浸润,胸腺上皮增生伴显著淋巴上皮病变,未见有单核样B细胞形态。免疫组化染色示增生淋巴组织由B和T细胞混合;Ig基因重排检测示多克隆性增生。结论 LESA样胸腺增生和胸腺MALT淋巴瘤均是胸腺少见的淋巴增生性病变,两者具有相似的组织学和免疫表型特征;结合基因重排技术详细分析两者的鉴别要点,有助于鉴别。     

肺黏膜相关淋巴组织边缘区B细胞淋巴瘤及良性淋巴组织增生性疾病的临床病理分析

目的 研究肺原发性黏膜相关淋巴组织边缘区B细胞(MALT)淋巴瘤及良性淋巴组织增生性疾病的临床病理形态、免疫组织化学表型和B细胞重链基因重排,比较肺MALT淋巴瘤和良性淋巴组织增生性疾病的差异.方法 回顾性的分析原发性肺MALT淋巴瘤13例,7例肺良性淋巴组织增生性疾病资料.对标本行常规HE染色,EnVision免疫组织化学染色(抗体包括AE1/AE3、CD20、CD79α、CD3、CD5、CD10、CD21、bel-2、bcl-6、cyclinD-1)及免疫球蛋白重链IgH基因重排检测.结果 13例肺MALT淋巴瘤,细胞成分多样,分别由不同比例的小淋巴细胞样细胞、中心细胞样细胞、单核样B细胞组成,常伴有浆细胞分化.肿瘤细胞以弥漫性和滤泡边缘区排列为主,常见反应性淋巴滤泡和滤泡中心的植入.肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.MALT淋巴瘤中,均未见坏死.9例可见肿瘤细胞侵犯血管壁,6例可见胸膜累及,2例肺门淋巴结侵犯.9例肺MALT淋巴瘤可见淋巴上皮样病变,免疫组织化学显示上皮细胞内的淋巴细胞CD20阳性,CD3阴性.7例肺良性淋巴组织增生性疾病,2例可见淋巴上皮样病变,免疫组织化学显示,其淋巴上皮样病变内的淋巴细胞,部分CD20阳性,部分CD3阳性.9例肺MALT淋巴瘤进行了免疫球蛋白重链IgH基因重排,8例阳性;7例良性淋巴组织增生性疾病均为阴性.结论 肺MALT淋巴瘤在细胞组成和排列上与其他部位结外MALT淋巴瘤相同,肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.在肺内淋巴上皮样病变常见于MALT淋巴瘤,并有助于诊断,但并非其特异性病变,一些肺的反应性淋巴组织增生也可出现,用免疫组织化学有助于区别两种病变.免疫球蛋白重链IgH基因重排可以帮助鉴别肺MALT淋巴瘤和良性淋巴组织增生性疾病.     

头颈部Kimura 病的临床病理特点与诊断

目的探讨头颈部Kimura病（Kimura’s disease,KD）的临床病理特点。方法对10例发生在头颈部的KD病临床资料、发病年龄、病理表现、实验室检查等进行回顾性分析。结果10例KD者临床上主要表现为头颈部无明显压痛的软组织包块。组织学上以小血管增生、大量嗜酸性粒细胞浸润、淋巴细胞增生及滤泡形成、生发中心扩大、纤维组织增生为基本病变。结论KD是一种具有特殊临床病理表现的炎性增生性病变,应与部分富含淋巴组织的肿瘤相鉴别,组织病理学对于诊断有着十分重要的意义。免疫组化结果显示KD是一种多克隆淋巴结炎性病变,提示细胞异常增殖与凋亡的抑制共同发挥作用。     

肺上皮样血管内皮细胞瘤2例临床病理分析

目的 探讨肺上皮样血管内皮细胞瘤(pulmonary epithlioid haemangioendothelioma,PEH)的临床病理特征并文献复习.方法 对2例在我院诊治的肺上皮样血管内皮细胞瘤的病例进行临床症状、影像学、病理形态及免疫组化进行详细的观察.结果 肺上皮样血管内皮细胞瘤无特征性的临床表现,影像学上常常表现为双肺多发小结节,病理学改变有特征性的中心硬化、细胞稀疏区和外周的细胞丰富区,免疫组化均表达vimentin,表达CD31、CD34、FⅧRAg等血管内皮标记物中的一项或多项.结论 肺上皮样血管内皮细胞瘤是一种罕见的肺肿瘤,无明确临床症状,影像学常常误诊,需要做活检才能诊断.     

嗜酸性淋巴肉芽肿的病理诊断及鉴别诊断

嗜酸性淋巴肉芽肿首先由中国的金显宅于１９３７年以“嗜酸性细胞增多性淋巴肉芽肿”之名报道，１９４８年日本的Ｋｉｍｕｒａ（木村）作了较为详细的描述，故本病又叫金氏病或木村病。此外，本病还有“嗜酸性粒细胞性淋巴结病”、“嗜酸性粒细胞性滤泡增生症候群”、“软组织嗜酸性肉芽肿”等名称。嗜酸性淋巴肉芽肿曾报告为“软组织肿瘤”，但由于常伴有淋巴结肿大，且二者组织象相同，有些病例只侵犯淋巴结而无皮下软组织的表现，所以，也可见到“淋巴结病变”的报告。在欧美许多文献中，把血管淋巴组织增生伴嗜酸性细胞增多（ａｎｇｉｏ…     

血管淋巴组织增生伴嗜酸细胞浸润与Kimura病

报道７例血管淋巴组织增生伴嗜酸细胞浸润和Ｋｉｍｕｒａ病，结果显示：两者为同一种疾病的不同阶段，前者以小淋巴和小扭核细胞浸润，为多克隆性；后者出现中扭核细胞，为单克隆性，复发病例则随病情的发展从小扭核细胞向中扭核过渡，亦为单克隆性，结果提示，本病有从良性向恶性发展的趋势，上皮样和组织细胞样血管增生是本病特征之一。     

丛状血管瘤13例临床病理分析

目的探讨丛状血管瘤的临床表现、病理学特征、免疫表型、诊断及鉴别诊断。方法回顾性分析13例婴幼儿丛状血管瘤的临床特征、病理学特征、免疫表型,并复习相关文献。结果 13例婴幼儿丛状血管瘤中,男童7例、女童6例,年龄2个月～2岁10个月,其中位于头颈部2例、躯干7例、四肢4例,临床主要表现为体表呈暗红色或紫红色浸润性斑片、斑块或多发丘疹、结节,随患儿生长皮损逐渐增大,其中1例伴卡梅现象(Kasabach-Merritt phenomenon, KMP)。镜下均为真皮及皮下组织中可见散在或广泛分布的多个小簇状结节或小叶,小叶由短梭形血管内皮细胞、血管周细胞及毛细血管腔构成,低倍镜下呈同心漩涡状或"炮弹"样生长,增生的细胞短梭形、较肥胖,核无异型性,可见少量核分裂象,增生细胞挤压或突入邻近管腔,使管腔呈裂隙状、半月形。免疫表型:CD31、CD34、SMA、CD61和vimentin均阳性,D2-40灶阳性,VEGF弱阳性(7/13),FⅧRAg部分血管内皮细胞阳性,GLUT1阴性,Ki-67增殖指数5%。结论丛状血管瘤是一种少见的血管良性增生性病变,诊断主要依据临床特征、病理检查及免疫组化标记。     

上皮样血管瘤七例临床病理观察

目的 探讨上皮样血管瘤的临床病理学特征、诊断及鉴别诊断.方法 对7例分别发生于皮肤、骨及静脉的上皮样血管瘤进行临床病理学分析.结果 男性4例,女性3例,年龄14～54岁(平均34岁).其中发生于皮肤和皮下者3例,骨2例,静脉血管2例.临床表现:发生于皮肤者为暗红色丘疹或结节,质软或韧,部分伴瘙痒,病程长者可相互融合;发生于骨者表现为溶骨性破坏,境界清楚;发生于静脉内者,表现为软组织内境界清楚的灰白灰红质韧结节.组织学上,病变以增生的小血管伴内皮细胞上皮样改变为特征,多数病例血管有明确的腔隙形成,部分病例局部可见内皮细胞实性片状生长,并见胞质空泡化,提示原始管腔形成.间质可伴有多少不等的炎性细胞浸润,以嗜酸性粒细胞、淋巴细胞和浆细胞为主.免疫组织化学上均表达血管内皮标志物CD31、CD34,上皮标志物细胞角蛋白阴性.6例患者获得随访结果,随访时间为5个月至3年,均未见发生复发及转移.结论上皮样血管瘤是一种罕见的可以发生于皮肤、软组织及骨的肿瘤/病变,可单发或多发,是良性的、可成功治愈的.其形态学谱系广泛,易与木村病、上皮样血管内皮瘤等混淆.     

肺上皮样血管内皮瘤的临床病理观察

目的探讨肺上皮样血管内皮瘤(pulmonaryepithelioidhemangioendothelioma,PEH)的临床病理特征、诊断及鉴别诊断。方法对3例PEH进行光镜观察和免疫组化标记,并结合文献进行分析。结果镜下见肿瘤细胞具有上皮样或组织细胞样形态,瘤细胞为圆形或多角形,呈小巢状、索状甚至腺样结构或不规则状,分布于黏液间质中;间质可见少量黏液样变或玻璃样变;瘤细胞内含有原始血管腔,核分裂象、多形性及坏死少见。肿瘤细胞表达CD34、FⅧRAg、CD31等血管内皮细胞标记,部分病例同时表达CK和(或)Vim。结论PEH是一种低度恶性肿瘤,其病理形态具有一定的特征性,诊断时需要与肺转移癌、肺上皮样血管肉瘤、肺淋巴管肌瘤病鉴别。     

A clinicopathological study of Kimura's disease and epithelioid hemangioma

The clinicopathological features of 9 cases of Kimura's disease and 5 cases of epithelioid hemangioma(EH) were reviewed. Patients with Kimura's disease presented with multiple or solitary large tumors occurring in the thigh, arms as well as parotid gland. Histologically, the lesions were characterized by numerous lymphoid follicles with proliferation of post-capillary venules lined by plump endothelium and a marked eosinophilic infiltration. Patients with EH were older than those with Kimura's disease and presented papular nodular lesions, less than 1 cm in size. Microscopically, there was proliferation of blood vessels lined by epithelioid endothelium with infiltration by lymphocytes and eosinophils. Although there may occasionally be clinicopathological overlap between Kimura's disease and EH, we consider that these two disorders can be recognized as separate entities.     

Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia) and Kimura''s disease in Chinese

Although Kimura's disease has often been considered to be identical to angiolymphoid hyperplasia with eosinophilia (epithelioid haemangioma), recent studies suggest that they are different clinicopathological entities. In this study, we have made a detailed morphological comparison of 10 cases of epithelioid haemangioma and 40 cases of Kimura's disease occurring in the Chinese population. The epithelioid haemangiomas occurred in the subcutaneous tissue, skin and maxillary antrum, whereas Kimura's disease affected the subcutaneous tissue, major salivary glands and lymph nodes. Distinctive features of epithelioid haemangiomas were exuberant proliferation of vessels lined by cuboidal to hobnail endothelial cells with irregular nuclei and cytoplasmic vacuoles, fibromyxoid matrix, involvement of muscular coat of blood vessels and zonation of inflammatory infiltrate towards the peripheral portion of the lesion. Distinctive features of Kimura's disease were florid lymphoid infiltrate with prominent lymphoid follicles, vascularization of germinal centres, germinal centre necrosis, marked eosinophilia with or without eosinophil abscess formation, proliferation of high endothelial venules, and sclerosis. The histological features suggest that epithelioid haemangioma is a proliferation of atypical endothelial cells, possibly neoplastic, that is associated with a variable inflammatory infiltrate, whereas Kimura's disease is primarily an inflammatory condition in which high endothelial venules are usually found.     

Observations concerning the pathogenesis of epithelioid hemangioma (angiolymphoid hyperplasia)

Epithelioid hemangioma (EH) is a benign vascular lesion that is characterized by well-formed, capillary-sized vessels lined by histiocytoid or epithelioid endothelial cells and often accompanied by a secondary inflammatory infiltrate. Whether or not these lesions are reactive or neoplastic has been debated and is reflected in the various designations, such as angiolymphoid hyperplasia with eosinophilia (ALHE), pseudopyogenic granuloma, and inflammatory angiomatous nodules. In order to study the pathogenesis of this lesion, 96 examples of EH of superficial and deep soft tissue origin were reviewed, noting the frequency with which these lesions arose adjacent to a damaged vessel and the incidence of antecedent trauma. In 60 cases (63%), an artery or vein was associated with the mass and, in the majority (52 cases), the vessel was damaged, as evidenced by the presence of fibrointimal proliferation, discontinuity of the internal elastic lamina, and/or mural disruption. No evidence of active arteritis was seen in any case. Antecedent trauma was documented in 12 cases and, in one of these, the subsequent formation of an arteriovenous malformation and development of the EH were noted. EH associated with damaged vessels or those in which a preceding history of trauma was available did not differ histologically from cases in which these variables were absent. Our findings suggest that a significant percentage of EH of soft tissue are not neoplastic, but arise on a reactive basis probably secondary to damage and repair of an artery or vein.     

A possible hypoxia-induced endothelial proliferation in the pathogenesis of epithelioid hemangioma

Epithelioid hemangioma is an uncommon benign vascular lesion with distinct clinicopathological characteristics. Histologically, this entity mainly composed of well-formed but often immature vessels lined by epithelioid endothelial cells with prominent chronic inflammatory component. There is considerable controversy whether epithelioid hemangioma is a reactive lesion or a true neoplasm. We postulated that the local hypoxia may play a role in the pathogenesis of this vascular tumor. This local hypoxic condition may caused by many incidences such as congenital vascular malformation or trauma. The hypoxia will lead to the proliferation of endothelial cells and the formation of this vascular tumor, which may lead by putative stimulators VEGF and HIF-1. Hypoxia may also promote endothelial cell proliferation through the renin-angiotensin-aldosterone system. Additionally, the inflammatory cells including eosinophils and mast cells may contribute to the endothelial cell proliferation in EH. Further study investigating the associated factors of hypoxia may lead to new, potentially important insights into epithelioid hemangioma, and might also contribute to novel strategies for the management of this entity.     

Angiolymphoid hyperplasia with eosinophilia associated with pregnancy: a case report and review of the literature

A case of angiolymphoid hyperplasia with eosinophilia (ALH) is reported in a 33-year-old woman who developed an auricular nodule during the second trimester of her pregnancy. Angiolymphoid hyperplasia with eosinophilia usually occurs on the head and neck of young adults and is more common in women than in men. Characteristic histologic features of ALH present in this case included proliferation of thick-walled blood vessels lined by prominent endothelial cells, infiltration of the interstitium by chronic inflammatory cells (mainly eosinophils), and presence of lymphoid follicles with germinal centers. The auricular tumor was completely excised. Thirteen months after excision, the patient remains tumor free. Although there are not many case reports on ALH during pregnancy or involving use of oral contraceptive pills, sex hormones may play a role in the pathogenesis of ALH. This hypothesis, in the context of cases previously described in the literature, and the differential diagnosis of ALH are discussed.     

Angiolymphoid hyperplasia with eosinophilia: report of a lesion mimicking soft tissue tumor of extremely long duration

We present a large sized lesion of the right upper arm in which characteristics of the angiolymphoid hyperplasia with eosinophilia (ALHE) intermingled with those of Kimura's disease (KD). The laboratory findings, the prominent vascular proliferation and the features of endothelial cells were suggestive of ALHE. However, the long duration of the disease, the site of involvement, the abundant lymphoid component forming lymph follicles with germinal centers and the fibrosis are features of KD. In agreement with other reports, our case shows that clinicopathologically there is an overlap between ALHE and KD.     

Epithelioid haemangioma--angiolymphoid hyperplasia with eosinophilia. Case report and review of literature

Epithelioid haemangioma (angiolymphoid hyperplasia with eosinophilia: ALH) is a rare tumour most often affecting the skin and subcutaneous tissue in young adults. Unusual findings in the present case included deep location oft the lesion with involvement of striated muscle and periosteum which complicated clinical diagnosis. These tumours are generally composed of proliferating small vessels with prominent endothelial cells and heavy inflammatory infiltrate made up of lymphocytes, lymphoid follicles, plasma cells, mast cells, and especially eosinophils. The lesion is benign, and local excision is curative. Clinical manifestation and morphological characteristics of this rare lesion are described in this paper and are compared with other cases reported in the literature.     

Angiofollicular lymph node hyperplasia (Castleman's disease): an immunohistochemical and enzyme-histochemical study of the hyaline-vascular form of lesion

Tissues from five cases of angiofollicular lymph node hyperplasia have been studied. All had the histological structure of the hyaline-vascular type of lesion; large numbers of very compact lymphoid follicles were distributed evenly throughout a highly vascular tissue. The follicles were characterized by their small size, a vascular poorly cellular and frequently hyalinized centre, and a 'tight' concentric mantle of small lymphocytes arranged in layers producing an 'onion-skin' appearance. The interfollicular tissue was characterized by the large numbers of small vessels mainly hyalinized capillaries and a few high endothelial venules and the presence of variable numbers of lymphocytes, plasma cells, immunocytes and immunoblasts. The immunoperoxidase method demonstrated polytypic cytoplasmic immunoglobulin in the small numbers of centroblasts and plasma cells within the follicle centres and in the plasma cells and immunocytes in the interfollicular tissue. Large numbers of suppressor T cells were present in the interfollicular areas and only scattered helper T cells were seen within the lymphocyte mantles. A strong reaction for factor VIII-related antigen was seen in the endothelium of the interfollicular high endothelial venules but only a weak reaction in the vessels in the follicle centres. A concentric distribution pattern of the dendritic reticulum cells was seen with the metalophil impregnation method of Marshall and with the enzyme histochemical methods for acid alpha-naphthyl acetate esterase and 5'-nucleotidase. This pattern differs from the zonal distribution of these cells seen in reactive lymphoid follicles. The nature and possible pathogenesis of AFLNH are discussed and contrasted with reactive hyperplasia.     

Angiolymphoid hyperplasia with eosinophilia associated with tetanus toxoid vaccination

Three cases of angiolymphoid hyperplasia with eosinophilia are reported at the site of tetanus toxoid vaccination. All presented as nodules on the upper arm, a known adverse effect of the vaccine following superficial inoculation. Histologically the nodules showed numerous small and medium sized vessels, some lined by hyperplastic endothelial cells with perivascular lymphocytic cuffing and an inflammatory infiltrate of plasma cells, eosinophils and scattered mast cells. Lymphoid follicles replete with germinal centres were identified in the dermis and subcutaneous tissue. Immunostaining revealed a polytypic lymphoplasmacytic infiltrate and a curious IgE reticulated pattern within the germinal centres, a feature described in both Kimura's disease and angiolymphoid hyperplasia. The present study suggests that atopic reaction to tetanus toxoid may be an aetiological factor in some cases of angiolymphoid hyperplasia.     

Intravascular (”intimal”) epithelioid angiosarcoma: clinicopathological and immunohistochemical analysis of three cases

Angiosarcomas are rare malignant mesenchymal tumours, characterized morphologically by anastomosing vascular channels lined by atypical and proliferative active endothelial cells. An epithelioid cytomorphology of tumour cells is often seen focally in angiosarcoma, whereas purely epithelioid angiosarcomas are rare. Although angiosarcomas show a vascular differentiation they are almost never confined to pre-existing blood vessels. We describe three cases of intravascular epithelioid angiosarcoma arising in the carotid artery of a 60-year-old man, in the infrarenal part of the abdominal aorta and both renal arteries of a 69-year-old woman, and in the abdominal aorta of a 68-year-old man. In all cases malignant tumour tissue was found incidentally after disobliteration of thrombosed vessels. Histologically, purely epithelioid angiosarcoma composed of solid sheets of epithelioid tumour cells was seen; immunohistochemistry confirmed the endothelial differentiation of neoplastic cells. The reported cases show that angiosarcoma can occasionally arise within a pre-existing vessel. Received: 18 May 1999 / Accepted: 8 June 1999