小肠巨大海绵状淋巴管瘤1例

患者女性 ,15岁。因阵发性腹痛入院。患者 10余年前在无明显诱因下出现脐周疼痛 ,阵发性 ,持续数日、数月发作1次 ,伴轻度恶心、呕吐、大便减少。近 1月来发作频繁。体检 :腹部可触及一包块。ＣＴ及ＭＲＩ示 :盆腔及中下腹区巨大囊实性占位。临床诊断 :回肠系膜淋巴管瘤。硬膜外麻醉下行回肠部分切除吻合术 ,术中见回肠起始段肠系膜增厚 ,内有大量乳白色液体 ,部分肠管呈乳白色 ,病变肠管切除。病理检查　眼观 :回肠一段 ,长 2 5ｃｍ ,外径 3ｃｍ。距一端 8ｃｍ处黏膜面呈乳白色 ,长径 7ｃｍ。肿块占据黏膜层、肌层及肠系膜 12ｃｍ× 7ｃｍ…     

关节旁黏液瘤临床病理特点

目的探讨关节旁黏液瘤(Juxta-articular myxoma,JAM)临床病理特点和鉴别诊断。方法对1例JAM进行组织形态学观察、免疫组化标记并复习文献。结果肿块位于左胫骨内侧髌骨下缘,不规则组织7cm×3cm×2cm,境界不清,切面周围为淡黄色脂肪组织,中央大部分区域呈黏液胶冻状。镜检:梭形、星芒状纤维母细胞样瘤细胞稀疏散在分布于丰富的黏液样基质中,细胞形态良善,间质血管稀少。部分区域血管丰富。散在有形状、大小不同的囊性腱鞘囊肿样腔隙。肿瘤界限不清,内有脂肪组织陷入。特殊染色:黏液样基质阿辛蓝弥漫(+)。免疫表型:瘤细胞Vim(+),部分瘤细胞α-SMA、CD34(+)。随访8个月未见复发。结论 JAM为良性病变,组织形态和免疫表型类似于肌内黏液瘤,见于膝、肩、肘、踝、髋等大关节旁。约有1/3病例复发,故长期随访是必要的。需与黏液性脂肪肉瘤、黏液纤维肉瘤、骨外黏液性软骨肉瘤、低度恶性纤维黏液样肉瘤等鉴别,避免过度治疗。     

小肠卡波西型血管内皮瘤临床病理特征

目的：探讨小肠卡波西型血管内皮瘤( kaposiform he-mangioendothelioma, KHE)的临床病理学特征、鉴别诊断和治疗方法。方法回顾性分析2例小肠KHE患儿的临床表现、组织学形态和免疫表型,并复习相关文献。结果患儿以肠梗阻、肠套叠为主要表现,不伴有Kasabach-Merritt综合征。镜下可见肿瘤细胞呈多结节状或多小叶状排列,向肠壁浸润生长,可见梭形细胞区、毛细血管瘤样结构和特征性的肾小球样结构。免疫表型：肿瘤细胞中CD31、CD34均强阳性, SMA 灶阳性, GLUT1、D2-40、S-100、CKpan、CK5/6、CR、desmin、DOG1和CD117均阴性。结论小肠KHE是一种罕见的中间型血管肿瘤,好发于婴幼儿和儿童,应与婴幼儿毛细血管瘤、丛状血管瘤、卡波西肉瘤、胃肠道间质瘤和梭形细胞血管内皮瘤等鉴别,诊断依靠典型的组织学形态和免疫表型,完整切除是治疗小肠KHE的有效方法。     

睾丸精母细胞性精原细胞瘤合并癌肉瘤一例

患者男，45岁，因左侧睾丸缓慢增大12年，突然增大4个月入院，患者1991年左侧睾丸无痛性增大，手术取睾丸组织一块，诊断为“精原细胞瘤”，并于1992年手术探查左侧睾丸，取活检未发现精原细胞瘤。此后无症状。1995年左侧睾丸缓慢增大，2002年1月左侧睾丸突然疼痛明显，诊为“附睾炎”予抗生素治疗20d缓解，2002年10月再次出现左侧睾丸增大伴疼痛，抗生素治疗无效，于2003年2月收入院，患者既往除1989年因右侧隐睾行隐睾切除术外无特殊。     

直肠间质瘤的临床病理特点及诊治

直肠间质瘤发病率低,确诊依赖于组织病理和免疫组化检查。目前治疗趋向于手术和分子靶向治疗等综合治疗。术后易复发,需密切监测与随访。     

升结肠自主神经瘤的临床病理诊断

目的：报道1例升结肠自主神经瘤（GANT）的临床病理特征及诊断和鉴别诊断。方法：通过HE、免疫组化及电镜观察1例结肠GANT并复习文献。结果：主要由梭形、多角形或上皮样细胞组成、胞质丰富、淡嗜酸性、核仁清楚,间质血管较丰富。瘤细胞免疫表型为vimentin,NSE,S-100蛋白及CD34阳性;CgA弱阳性;而actin,desmin,HHF35,Mac387,Syn,CK及HMB45阴性。电镜观察瘤细胞类似神经元、见细长的细胞突起和致密轴心神经分泌颗粒,瘤细胞间可见丝团样纤维。结论：GANT是胃肠道间质瘤中具有神经元分化的一种特殊类型、发生在结肠罕见。其组织形态应与平滑肌性肿瘤、神经鞘瘤等鉴别。     

脾脏淋巴管瘤的临床病理观察

目的探讨脾脏淋巴管瘤的临床病理特征和鉴别诊断特点。方法结合文献回顾,对18例脾脏淋巴管瘤进行临床资料分析、病理形态学观察及免疫表型检测。结果18例淋巴管瘤患者年龄9～72岁,中位年龄40岁;男13例,女5例;可无明显临床症状,也可表现以腹痛为主的消化道症状或脾功能亢进的相关症状。18例患者随访率72．2％,随访时间从5个月到15年不等,患者均无瘤生存,无复发及转移。脾脏多有肿大,病灶大体形态多样,包括囊性（8例）、实性（5例）和蜂窝状（5例）,可单发（5例）或多发（13例）;组织形态上分为海绵状淋巴管瘤（9例）、囊状淋巴管瘤（5例）及混合型（4例）。16例（100％）均表达CD9;7例（43．8％）表达D2-40。结论发生在脾脏的淋巴管瘤非常少见,应注意与脾脏血管瘤等鉴别。     

小肠间质瘤的免疫组织化学特征其意义

目的探讨小肠间质瘤的临床病理诊断及免疫组织化学特征。方法收集我校附属医院临床病理科6年来对外科手术切除的小肠肿瘤标本,光镜观察、免疫组织化学方法检测Vimentin、desmin、S-100、actin、CD117、CD34和SMA的表达。结果13例肿瘤中良性3例,潜在恶性4例,恶性6例;其中黏膜下生长3例,浆膜下生长5例,肠系膜处生长2例,肌壁内生长3例,常见症状是上腹部肿块和上消化道出血,免疫表形特征为:CD11713例(100%)胞质阳性表达;CD3410例阳性(76.9%)表达,S-100蛋白2例(15.4%)呈局灶表达,10例SMA阴性表达。结论小肠间质瘤发生率较高,多为恶性,有必要和肠的雪旺细胞瘤、平滑肌瘤相区别,CD117、CD34可作为诊断小肠间质瘤免疫标记物,而肿瘤大小、有无出血和坏死、核分裂像等均可作为良恶之判断的参照指标。     

原发小肠恶性间质瘤伴腹腔广泛转移一例

患者男 ,6 6岁。因间断左中上腹痛二周并发现腹部有包块而于 2 0 0 0年 2月 16日入院。体检 :左中上腹部压疼 ,左侧脐旁可触及一肿物 ,但大小及边界触摸不清。腹部Ｂ超、ＣＴ均显示 :左中上腹部占位性病变 ,肝脏内多发占位性病变 ,考虑为 :转移癌。手术所见 :全腹腔及盆腔的腹膜、肠壁、网膜、系膜上密布大小不等的球形肿物 ,肿瘤部分融合成块 ,有的象葡萄串样 ,肿瘤数目多达近万枚。切除一段空肠上可见一与肠壁紧密黏连的外凸性肿物 ,肿物边界清楚 ,检查肝脏表面及膈面均未见肿瘤组织。临床诊断 ,恶性间皮瘤。病理检查 :送检空肠组织一段 ,…     

Molecular cytogenetic analysis of human spermatocytic seminomas

We performed comparative genomic hybridization (CGH) on 8 formalin-fixed, paraffin wax-embedded primary spermatocytic seminomas (SS) from 7 patients, one of whom developed metastatic disease. In general, this tumour type is not associated with development of metastases. Since there are only few reported cases of metastatic SS in the literature, this study is the first report of chromosomal constitution in a patient with metastatic disease. Chromosomal imbalances were observed in all 8 tumours analysed by CGH. Frequent copy number alterations were enh(9), dim(16 or 16p), enh(20) and enh(X), each in 6 samples, followed by dim(7) in 4, and enh(1), enh(18) and dim(15), each in 3 samples. In addition to the CGH analysis, interphase fluorescence in situ hybridisation (I-FISH) was applied to evaluate the CGH results and to define the size of the aberrant cell population. Interphase cytogenetics showed gain of material on chromosomes 9 and X in all tumours analysed. Overall, the I-FISH results were in agreement with the CGH data. In conclusion, gain of chromosome 9 seems to be restricted to SS and point to an important role for this aberration in the development of this tumour type.     

1例气球细胞痣临床及病理分析

目的：学习气球细胞痣的临床病理诊断及鉴别诊断。方法：对1例气球细胞痣进行临床资料、病理形态及免疫组织化学观察,并结合文献对其诊断及鉴别诊断进行学习。结果：前胸壁皮肤先天性色素痣,近1年来增大,其组织学上表现为混合痣伴多量细胞胞浆丰富,呈气球样。免疫组织化学标记气球样细胞Melan A、S100、HMB45阳性,CD68阴性。结论：气球细胞痣是较为罕见的一种黑素细胞痣,根据其临床及组织学特征,结合免疫组织化学方法,有助于其诊断及鉴别诊断。     

Mixed testicular germ cell tumor in an adult with cryptorchidism and Down's syndrome

We here present a case of mixed testicular germ cell tumor in an adult with cryptorchidism and Down's syndrome. A 20-year-old Japanese man with a mass in the left inguinal region underwent orchidectomy as a left testicular tumor was suspected. Histology showed a mixed germ-cell tumor with embryonal carcinoma and yolk sac tumor with syncytiotrophoblastic giant cells occurring in a cryptorchid testis. Chromosomal analysis of peripheral lymphocytes disclosed a karyotype of 47,XY,+21[20]. Our case provides further evidence that these three conditions-Down's syndrome, cryptorchidism and testicular germ cell tumor-may be closely associated. To our knowledge this is the first case of mixed germ cell tumor arising in a patient with Down's syndrome and cryptorchidism.     

75例原发性胃肠道淋巴瘤的临床病理分析

目的:探讨原发性胃肠道淋巴瘤(primary gastrointestinal lymphoma,PGIL)的临床特点、诊断、病理特征和治疗.方法:收集2011年1月至2017年1月就诊于武汉大学人民医院75例PGIL患者的临床及病理资料,并进行回顾性分析.结果:75例PGIL患者的男女性别比为1.5:1,年龄(56.96±14.51)岁,其中43例(57.33％)患者以腹部隐痛为主要症状.51例(68.00％)患者CEA升高,17例(32.69％)患者行大便隐血试验(+).胃镜/肠镜诊断阳性率为74.58％.9例直肠淋巴瘤患者之中,有5例直肠指诊阳性,直肠指诊的阳性率为55.56％.75例PGIL患者均已行病理检查,45例弥漫性大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL),13例黏膜组织相关淋巴瘤黏膜组织相关淋巴瘤(mucosa-associated lymphoid tissue,MALT),6例间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL),4例外周T细胞淋巴瘤(peripheral T-cell lymphoma,PTCL),7例为其它病理类型.按照Ann Arbor标准分期,ⅠE期7例(9.33％),ⅡE期25例(33.33％,ⅢE 11例(14.67％),IVE期32例(42.67％).41例患者行CHOP化疗方案,12例患者行R-CHOP化疗方案,3例行手术治疗,19例患者放弃治疗.结论:PGIL好发于中老年男性,临床症状以腹痛为主,确诊主要依靠内镜下活检,内镜下诊断不明确的患者可行腹腔探查术明确诊断.CEA、大便隐血试验对PGIL的辅助诊断有一定价值,而直肠指诊对直肠淋巴瘤的初诊有重要意义.早期PGIL患者以PGL多见,而中晚期PGIL患者以PIL多见.病理类型以DLBCL和MALT为主.DLBCL型PGIL治疗首选CHOP/R-CHOP,MALT型PGIL患者首选HP根除疗法,手术适用于PGIL的并发症治疗.     

低蛋白性肥厚性胃病临床病理分析

目的探讨低蛋白性肥厚性胃病(Menetrier病)临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析3例Menetrier病的临床特征、组织学形态及免疫表型,并复习相关文献。结果 3例患者中,成人女性2例,小儿1例,临床表现均有腹痛及低蛋白血症,内窥镜显示胃窦及胃体胃壁明显增厚,表面高低不平,局部呈脑回样改变,局部结节状及息肉样改变,组织学显示小凹上皮增生明显,延伸并局部扩张,固有腺体萎缩,固有层水肿及急慢性炎细胞浸润。免疫表型:3例EGFR表达均增强,HP及CMV均阴性。3例患者出院后1例成人及小儿恢复良好,另1例成人病情反复,3年后因其他相关疾病死亡。结论Menetrier病为罕见疾病,临床表现及检查无特异性,易误诊为其它疾病,需结合组织学形态并排除其他鉴别诊断,必要时行内镜黏膜切除来明确诊断。     

Immunohistochemical characterization of the leucocytic infiltrate associated with canine seminomas

Both canine and human seminomas are typically associated with leucocytic infiltration, the role of which remains poorly understood. In this study, leucocytes infiltrating 10 canine seminomas were characterized. Monoclonal antibodies directed against CD18, CD11a, CD11b, CD11c, CD21, CD3, CD4, CD8 and Major Histocompatibility Complex class I and II (MHC I and MHC II) were also employed. Infiltrating leucocytes were located around vessels, adjacent to the thin fibrous septa between neoplastic lobules, and were also scattered singly amongst neoplastic cells. The more profuse infiltrates often had the appearance of follicular aggregates. Immunohistochemically, all the samples showed generally similar results. Most of the infiltrating cells were positive for CD18 and CD11a. Infiltrating cells were mainly T lymphocytes (CD3+), particularly of the CD8+ subset. B lymphocytes (CD21+) were detectable in almost all infiltrates; in the follicular aggregates they were centrally located, whereas T lymphocytes (CD3+) lined the periphery. CD11c+ cells were always more numerous than CD11b+ cells, demonstrating that if macrophages and antigen-presenting cells (APCs) were well represented, monocytes and granulocytes were practically absent. Almost all of the infiltrating cells were positive for both MHC I and MHC II antigens and, in nine samples, a large number of neoplastic cells expressing MHC I were detected. A few neoplastic cells expressing MHC II were observed in seven cases. The presence of CD8+ lymphocytes, together with the large number of both infiltrating and neoplastic cells expressing MHC I, suggests that the lymphocytes mediate a cytotoxic reaction against the neoplastic cells. This hypothesis may underlie the favourable prognosis frequently associated with canine seminomas.     

小肠原发性绒毛膜癌临床病理分析并文献复习

目的:探讨小肠原发性绒毛膜癌的临床特征、病理特点、诊断及治疗方法.方法:分析8例小肠原发性绒癌的临床病理特征,复习相关文献.结果:8例病例的典型症状为黑便,5例病变部位位于空肠.所有病例血清β-hCG均异常升高.主要病理特点为细胞滋养细胞及合体滋养细胞呈片状分布,伴出血及坏死.免疫组织化学结果示肿瘤细胞β-hCG为阳性.结论:小肠原发性绒毛膜癌十分罕见,但仍应纳入鉴别诊断.通过形态学特点、免疫表型及检测血清β-hCG水平可确诊.     

Cryopreservation of intact testicular tissue from boys with cryptorchidism

BACKGROUND: Boys with cryptorchidism often face fertility problems in adult life despite having orchiopexy performed at a very young age. During this operation, a biopsy of the testis is normally taken in order to evaluate their infertility potential and the presence of malignant cells. This study evaluated the morphology and functional capacity of cryopreserved testes biopsies and their possible use in fertility preservation. METHODS: Biopsies from 11 testes (eight boys) were obtained. Each biopsy was subdivided into six pieces and two pieces were frozen in each of two different cryoprotectants. One fresh and two cryopreserved pieces were cultured for 2 weeks. All pieces were prepared for histology. Used culture media were analysed for testosterone and inhibin B concentrations. RESULTS: The morphology of the fresh and frozen-thawed samples was similar, with well-preserved seminiferous tubules and interstitial cells. A similar picture appeared after 2 weeks of culture, but a few of the cultured biopsies contained small necrotic areas. The presence of spermatogonia was verified by c-kit-positive immunostaining. Production of testosterone and inhibin B (ng/mm(3) testis tissue) in the frozen-thawed pieces was on average similar to that of the fresh samples. CONCLUSIONS: Intact testicular tissue from young boys with non-descended testes tolerates cryopreservation with surviving spermatogonia and without significant loss of the ability to produce testis-specific hormones in vitro. It may be an option to freeze part of the testis biopsy, which is routinely removed during the operation for cryptorchidism, for fertility preservation in adult life.     

A comparative morphological and immunohistochemical study of testicular seminomas and intracranial germinomas

A series of 10 classical testicular seminomas and 10 intracranial germinomas were reviewed and examined using immunohistochemical techniques. In particular, the staining profiles of the tumour cells and the nature of the mononuclear cell infiltrate at the two sites were studied and compared. The tumour cells in eight of the 10 intracranial germinomas and nine of the 10 seminomas exhibited positive staining with placental alkaline phosphatase. Only one intracranial germinoma showed evidence of human chorionic gonadotrophin expression. Tumour cells in all cases at both sites were negative for cytokeratin and vimentin. The lymphocytic infiltrate in both the testicular and intracranial tumours was similar, comprising T-cells and B-cells, the former predominantly. The presence of macrophages and granulomas in tumours at both sites was noted. These findings confirm the high degree of similarity of germinomas of intracranial and testicular origin, and support the hypothesis of a common derivation. We could find no evidence of differentiation of tumour cells at either site.     

Clinicopathological analysis of 143 primary malignant lymphomas in the small and large intestines based on the new WHO classification

AIM: To study the clinicopathological and immunohistochemical features of 143 cases of primary small and large intestinal non-Hodgkin's lymphoma (NHL) in Japanese patients who presented between 1981 and 2000. METHODS AND RESULTS: The new World Health Organization (WHO) classification was used to classify NHL. The patients included 109 males and 34 females, with an average age of 54.1 years. Tumour sites were as follows: ileocaecal (n = 51, 35.7%), ileum (n = 29, 20.3%), rectum (n = 13, 9.1%), and duodenum (n = 11, 7.7%). Macroscopically, 124 cases (86.7%) were classified as tumorous type, 12 (8.4%) as diffuse infiltration type (erosion, superficial ulceration), five (3.5%) as polyposis type, and only two cases (1.4%) as ulceration type. Immunohistochemically, 122 lesions (85.3%) were of B-cell phenotype and 21 lesions (14.7%) were of T-cell phenotype. According to the WHO classification, of the B-cell lymphomas, 84 cases (68.9%) were large cell, 16 (13.1%) were Burkitt, 10 (8.2%) were marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT), and seven (5.7%) were mantle cell tumours. Among the T-cell lymphomas, 15 (71.4%) were of unspecified type, two (9.5%) were natural killer type, two were anaplastic large-cell lymphomas, one was lymphoblastic, and one was an adult T-cell leukaemia lymphoma. The survival rate for T-cell lymphomas was poorer than for B-cell lymphomas. Among the B-cell lymphomas, mantle cell lymphoma tended to have a poorer prognosis, whereas MALT lymphomas had a better prognosis than other B-cell tumour types. CONCLUSIONS: Our retrospective study of patients with primary malignant lymphomas in the small and large intestines has illustrated the clinical features and outcomes of patients with this disease.