肛管直肠原发性恶性黑色素瘤临床病理分析

目的 探讨肛管直肠原发性恶性黑色素瘤的临床表现、手术方式、病理特征、免疫组化特点及误诊原因.方法 分析13例肛管直肠原发性恶性黑色素瘤的临床及病理资料,所有标本均行HE及免疫组织化学染色,免疫组织化学采用SP法.结果 本组男4例,女9例,平均年龄56.5岁.主要临床症状为排便习惯改变、便血、肛门口脱出肿物、肛门坠胀和肛周疼痛.临床误诊率为100%.肿物距肛缘1～4 cm,肉眼呈结节状、息肉状、菜花状、溃疡型及平坦型,镜下形态多样,以上皮细胞及梭形细胞为主,11例检见黑色素颗粒.结论 肛管直肠原发性恶性黑色素瘤是一种相对少见、恶性度极高的肿瘤,临床误诊率高,病理活检难以准确分型,S-100、HMB-45免疫组织化学染色有助于诊断,且特异性高,特别是对于无色素者.     

直肠肛管测压研究的进展

直肠肛管测压是诊断直肠肛门疾病及评价肛门功能的重要方法之一。近年来其检测范围不断扩大,主要包括:直肠肛管压力测试、直肠肛门松弛反射、肛门内括约肌节律性和直肠顺应性等。肛管压力不能单独评价肛门功能,直肠肛门松弛反射不可忽视。检测中三维压力向量分析、容量分析及梯度分析等方法可发现肛管球面或扇面压力缺陷,更好地反映排便机制和肛门功能,使直肠肛门生理学研究进入一个新阶段。直肠肛管测压与肛门内超声描记术相结合能更好地评价肛门括约肌状态,在肛门括约肌研究方面,动态直肠肛管测压是静态测压的有益补充,直肠肛管测压已用于生物反馈治疗,成为肛门外括约肌重复训练的物理疗法之一。计算机的应用使直肠肛管测压形象化、标准化,提高了科学性,是直肠肛管测压技术的重要发展。     

恶性黑色素瘤组织学变异型与鉴别诊断

目的研究恶黑的临床病理特点、组织学变异型及鉴别诊断。方法应用HE染色、免疫组化标记对130例恶黑形态学进行观察、分析临床资料，并划分组织学变异型。结果根据病理形态学发现及免疫组化标记，130例恶黑组织学变异被分为上皮样型、肉瘤样型、癌肉瘤样型、促结缔组织增生型、小细胞型、血管周细胞样型、假腺样型、浆细胞样型、横纹肌样型、巨细胞样型、印戒细胞型、透明细胞型、气球样细胞型及炎性MFH样型。结论恶黑的组织病理结构和形态变异复杂，细胞类型多，少色素、无色素及核仁模糊占大多数，有不同的病理类型，有时诊断很困难，应特别注意与多种类型肿瘤相鉴别。     

2.154 51例便秘小儿肛门直肠测压分析

目的通过对51例便秘小儿肛门直肠测压,了解便秘小儿肛门直肠压力与先天性巨结肠等疾病的关系.方法使用MMS(胃肠动力监测系统)UPS-2020系统,对51例便秘小儿进行肛门直肠测压,检查时患者处于清醒、非麻醉状态,不用影响直肠功能的药物,不进行肠道准备.测量肛管的静息压、长度及其直肠肛门抑制反射(RAIR)、直肠敏感性和顺应性.结果男性30例,女性21例;年龄最小40天,最大16岁,平均年龄3.7岁; 经外科手术及病理结果证实为先天性巨结肠共19例,其中男性12例,女性7例;其肛管的静息压(平均)为220mmHg,平均高压带长度为3.1cm,直肠肛门抑制反射(RAIR)减弱或消失, 直肠敏感性减低;平均气囊注气96mL.其余32例病人,其肛管的静息压(平均)为120mmHg, 平均高压带长度为2.1cm,直肠肛门抑制反射(RAIR)存在,直肠敏感性和顺应性好;平均气囊注气46mL出现不适感觉,平均气囊注气66mL不能耐受.结论先天性巨结肠肛管的静息压升高,直肠肛门抑制反射(RAIR)减弱或消失,是诊断短型和常规型先天性巨肠可靠方法,在手术前进行肛门直肠测压对指导诊断和治疗有很大的帮助.     

肛管直肠恶性黑色素瘤一家系四例

肛管直肠恶性黑色素瘤一家系四例马胜军宋代波先证者男，７４岁。因血便伴肛门肿物３个月于１９８０年６月１８日入院。查体：肛门处坠出一６ｃｍ×６ｃｍ×５ｃｍ大小肿物，暗紫色，触之易出血，行肿物切除，术后病理报告为恶性黑色素瘤，术后４个月死于多脏器转移。先证...     

2.92直肠肛门反射的表达及其对特发性便秘的诊断价值

目的研究直肠肛管压力在直肠肛门反射(RAR)生理功能中的表达,提出其对特发性便秘(CIC)的诊断意义. 方法用直肠肛管测压法检测30例正常人和32例特发性便秘患者直肠肛门反射曲线的肛门外括约肌收缩压(EASCP),肛门内括约肌舒张压(1A-SDP),肛门外括约肌收缩压与肛门内括约肌舒张压的压力差(PD),反射时间(RT)包括直肠肛门收缩反射时间(RACRT)和直肠肛门抑制反射时间(RAIRT)6项技术指标.     

直肠肛门反射的表达及其对特发性便秘的诊断价值

目的　研究直肠肛管压力在直肠肛门反射(RAR)生理功能中的表达 ,提出其对特发性便秘(CIC)的诊断意义。方法　用直肠肛管测压法检测 30例正常人和32例特发性便秘患者直肠肛门反射曲线的肛门外括约肌收缩压 (EASCP) ,肛门内括约肌舒张压 (1A SDP) ,肛门外括约肌收缩压与肛门内括约肌舒张压的压力差 (PD) ,反射时间 (RT)包括直肠肛门收缩反射时间 (RACRT)和直肠肛门抑制反射时间 (RAIRT)6项技术指标。结果　 30例正常人均能导引直肠肛门反射 ,32例特发性便秘患者的肛门外括约肌收缩压(EASCP) ,肛门内括约肌舒张压 (1ASDP) ,肛…     

功能性便秘和IBS便秘型在肛管直肠运动和感觉上的异同

目的　观察不同的慢性便秘患者在肛管直肠运动、扩张时的直肠感觉上有无异同。方法　根据RomeⅡ诊断标准选择 16例功能性便秘 (FC)和 2 2例IBS便秘型 (IBS C) ,均了解患者便秘的相关症状 ,运用液体灌注测压系统测量直肠、肛管静息压 ,肛管松弛率等指标 ,利用电子恒压器检测直肠敏感性及顺应性。直肠感觉用压力表示 ,采取时相性增压上升扩张的方式 (充气速率 38mL s)检测。同时以 10例正常健康人为对照。结果　两组中直肠肛管静息压、直肠肛门抑制反射与对照组无明显差异。直肠顺应性FC组明显低于IBS C (P <0 0 5 )及对照组 (P <0 …     

2.137 长期使用泻剂对肛门直肠动力的影响

目的对使用泻剂的便秘病人进行肛门直肠压力、直肠敏感性的检测,探讨泻剂对肛门直肠动力的影响.方法采用美国Sandhill公司生产的BioLAB动力学参数监测系统、二通道环状固态探头肛门直肠压力测压导管对40例服用泻剂的便秘病人进行直肠静息压、肛管静息压、肛管的功能长度、肛门括约肌最大榨缩压、最大榨缩间期;模拟排便动作时直肠的收缩压、肛门括约肌的松弛压、排便指数;直肠感知性的检测.结果必须服药组肛管的长度、肛管静息压、肛管直肠压力差明显低于有时服药组(P＜0.05);肛门括约肌最大榨缩压、最大榨缩间期、模拟排便动作时直肠的收缩压、肛门括约肌的松弛压、排便指数、直肠最大耐受容量低于有时服药组(P＞0.05) ,直肠开始感觉阈值、排便感阈值、排便紧迫感阈值高于有时服药组(P＞0.05);统计学意义不显著.结论服用泻剂的程度与肛门直肠功能的改变有密切的联系,长期服用泻剂可影响肛门直肠的动力,治疗时应根据不同的分型采用不同的治疗方法.     

肛管界限的讨论

<正> 肛管是指消化管末段,但其界标以及各结构的命名相当混乱,有时是互相矛盾的。关于肛管的定义,大体有两种看法:第一种是从发生的角度看,把齿状线作为肛管上端的界标,即把从齿状线至肛门一段长约2厘米的肠管称为肛管;第二种是从形态、功能的角度看,把直肠穿过盆膈处(也有把提肌板内侧缘、肛直肠环或直肠柱上端连线——Herrmann氏线)作为肛管上端的界标,即把此处以下至肛门一段长约4厘米的肠管称为肛管。     

Composite paraganglioma and ganglioneuroma in the retroperitoneum: a case report

Reports of a composite paraganglioma (PG) and ganglioneuroma (GN) in the retroperitoneum are rare. In the present case, dynamic computed tomographic (CT) findings showed a 30 × 22 × 20 mm tumor that was located in the retroperitoneum and which was dissociated from pancreatic tissue and the left adrenal gland. The markedly reddish tumor showed a clear margin and central multicystic changes on the cut surface. The tumor was composed of two major components, the PG and the GN. The paraganglionic cells in the PG component, which were arranged in a nested pattern, occupied the main and central part of the tumor. Both ganglionic cells and Schwann cells in the GN were located in a unorganized pattern in the periphery. The paraganglionic cells exhibited a Zellballen pattern, which consisted of an association of edematous vascular-rich stroma and focal hemorrhage, resulting in multicystic changes. These centrally located tumor cells were pleomorphic in part and did not have mitotic figures. In the periphery, Schwann cells, which were arranged in an obscure and fascicular pattern that was intermingled with large ganglionic cells, were located adjacent to the PG component with a mostly sharp margin. With higher magnification, the border was not as sharp, as revealed especially with chromogranin-A immunostain, in which both the PG and GN components were focally intermingled with each other. The histogenesis of the composite PG and GN was thought to be extraadrenal neural crest cells in the retroperitoneum because the tumor was not located in the adrenal gland or the Zuckerkandl organ, according to the CT findings. The immunohistochemical findings of this rare case of a composite PG and GN in the retroperitoneum are reported with a focus on the peculiar arrangement of these two components.     

Case of clear cell ependymoma of medulla oblongata: clinicopathological and immunohistochemical study with literature review

Clear cell ependymoma has been included in the WHO classification of the central nervous system in 1993, after the first report by Kawano et al. Since then, only a few cases have been reported. Most clear cell ependymoma cases reported in the literature so far were located in the supra-tentorial compartment and/or cerebellum, and one case was in the cervical spinal cord. We report a case of clear cell ependymoma whose histological features were sufficient for the diagnosis and was unusually located in the fourth ventricle originating from the medulla oblongata. The tumor showed uniform tumor cells with perinuclear halo, nuclei being centrally located. Most of the tumor cells were arranged as perivascular pseudorosettes, and no ependymal canals or rosettes were evident. Mitotic figures were not frequent. Immunohistochemically, the tumor cells were strongly reactive for glial fibrillary acidic protein and vimentin, and weak and dot-like positive for epithelial membrane antigen. Clear cell change of the tumor cells appeared to be fixation artifact because this feature was not evident in the frozen section.     

Merkel cell tumor of the skin. Ultrastructural and immunohistochemical studies

A skin tumor of a 66-year-old female was investigated morphologically and immunohistochemically. The tumor was located within the dermis and comprised of rounded cells with scanty cytoplasm, which proliferated forming a small nest or trabecular arrangement. Electron microscopic observation indicated the presence of dense-core granules within the tumor cell cytoplasm suggesting that the tumor was derived from Merkel cells. Occasionally clusters or bundles of the intermediate filaments were found in the perinuclear cytoplasm of the tumor cells. Each tumor cell was connected with desmosomes. Immunohistochemical staining with anti-keratin antiserum showed positive reaction at the perinuclear cytoplasm of the tumor cells indicating that the cluster of the microfilaments presumably contains keratin. Conversely S-100 protein was negative in the tumor cells. The results obtained strongly suggest that the tumor or Merkel cell was considered to be derived from the epidermal immature cells rather than from the neural crest.     

Infratentorial giant cell ependymoma: a rare variant of ependymoma

We describe a giant cell ependymoma occurring in a 50-year-old man. The mass was located in the posterior aspect of the foramen magnum, extending from the cerebellar tonsil to the upper cervical spine. The tumor was a highly cellular neoplasm showing biphasic histology. Diffuse sheets of non-cohesive atypical giant cells, having eccentrically located single or multiple nuclei and plump eosinophilic cytoplasm, partly infiltrated the desmoplastic inflammatory stroma. Parts of perivascular pseudorosette-forming or pseudopapillary areas were composed of atypically elongated cells, which looked like conventional anaplastic ependymoma. There was a transitional area between two patterns. Numerous mitoses and focal necrosis were observed. Immunohistochemically, the tumor cells were immunoreactive for glial fibrillary acidic protein, vimentin, S-100 protein, and CD99. None of the tumor cells showed immunoreactivity for epithelial membrane antigen except for the intracytoplasmic lumen of a few vacuolated cells. Ultrastructurally, tumor cells were ependymal in nature; we noted cytoplasmic intermediate filaments and intercellular microrosettes with microvilli, cilia, and long zonula adherens. The features of this tumor, e.g. its superficial location, mixed giant cells, perivascular pseudorosettes or papillaries, complicated its differentiation from rhabdoid/papillary meningioma. However, immunohistochemistry and electron microscopy confirmed the diagnosis of ependymoma. The giant cell variant should be included in the subclassification of the ependymoma.     

MERKEL CELL TUMOR OF THE SKIN Ultrastructural and Immunohistochemical Studies

A skin tumor of a 66-y-ear-old female was investigated morphologically and immunohistochemically. The tumor was located within the dermis and comprised of rounded cells with scanty cytoplasm, which proliferated forming a small nest or trabecular arrangement. Electron microscopic observation indicated the presence of dense-core granules within the tumor cell cytoplasm suggesting that the tumor was derived from Merkel cells. Occasionally clusters or bundles of the intermediate filaments were found in the perinuclear cytoplasm of the tumor cells. Each tumor cell was connected with des-mosomes. Immunohistochemical staining with anti-keratin antiserum showed positive reaction at the perinuclear cytoplasm of the tumor cells indicating that the cluster of the microfilaments presumably contains keratin. Conversely S-100 protein was negative in the tumor cells. The results obtained strongly suggest that the tumor or Merkel cell was considered to be derived from the epidermal immature cells rather than from the neural crest. ACTA PATHOL. JPN. 34: 1433–1440. 1984.     

Mixed neuronal–glial tumor of the digestive tract: Distinctive entity from gastrointestinal stromal tumor?

A 53-year-old-woman presenting with pelvic discomfort was found to have a 9.5 cm tumor located in the wall of the ileon. Light microscopy showed that the tumor was made of fascicles of plump spindle cells and bizarre epithelioid cells. A cuff of lymphoid cells was also present at the tumor margin. The tumor cells strongly expressed tau protein, neuron-specific enolase, protein green product 9.5 and glial fibrillary acid protein (GFAP), but did not show positive immunostaining for S-100 protein, CD34 or CD117. The tumor showed unequivocal ultrastructural evidence of neural differentiation. Skeinoid fibers were scattered throughout the tumor. This is the first mixed neuronal-glial tumor of the digestive tract to be described in the literature. Such histological and immunohistochemical features could be misinterpreted as features of digestive schwannoma. We suggest that this tumor is distinct from gastrointestinal stromal tumors in lacking CD34 and CD117 expression.     

Low-grade renal cell carcinoma arising from the lower nephron: a case report with immunohistochemical, histochemical and ultrastructural studies

Most renal cell carcinomas (RCC) are composed of clear cells with sinusoid-like vasculatures and originate from the proximal tubule. On the other hand, collecting duct carcinoma (CDC) and chromophobe RCC are thought to originate from the lower nephron. In the present study, we present a case of unusual RCC. The patient was a 68-year-old Japanese woman who had developed general fatigue with hematuria. Computed tomography revealed a left renal tumor suggesting sarcoma. The resected tumor was located in the renal parenchyma, measuring 12 x 10 x 8 cm in size. Histologically, the tumor consisted principally of cuboidal cells forming parallel or radiating arrays, continuous with the spindle-shaped cells. Most parts of the tumor showed hemorrhagic necrosis. Immunohistochemically, tumor cells were positive for high molecular weight cytokeratins, vinculin, vimentin, CD15 and epithelial membrane antigen, and showed affinities with some kinds of lectins. N- and E-cadherins and beta-catenin were diffusely positive in tumor cells. Nuclear positivity for Ki-67 and p53 protein were approximately 2.0 and 1.7%, respectively. Considering its morphological and histochemical natures, this tumor is considered to have originated from the lower nephron, which is unique for a tumor of low-grade malignancy.     

Juvenile granulosa cell tumor of the testis

Juvenile granulosa cell tumor occurred in a newborn. The tumor presented with testicular torsion, and no malformations were observed. The karyotype was normal. The occurrence of initial tumoral lesions in the seminiferous tubules located in the vicinity of the tumor suggests that the tumor originated from immature Sertoli's cells. To our knowledge, this is the tenth case reported in a newborn and the second associated with testicular torsion.     

伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤的临床病理观察

目的 探讨伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤的病理学特点、诊断及鉴别诊断要点.方法 采用HE及免疫组织化学(EnVision法)方法 ,对7例分别发生于眼眶及眶外的伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤进行病理学分析.结果 2例为发生于眼眶的肿瘤,其中1例为复发病例;5例为眶外肿瘤.镜下特征:肿瘤境界清楚,由无明显异型性的圆形或梭形的肿瘤细胞与胶原混杂组成,富含血管,部分区域形成假血管腔隙样结构,多核巨细胞衬于腔隙的内壁或散布于间质中.免疫组织化学标记显示肿瘤细胞和巨细胞表达CD34.7例均行肿块切除术,术后随访4例,均无复发.结论 伴有巨细胞的血管外皮细胞瘤/孤立性纤维性肿瘤是一种眼眶和眶外组织的中间型软组织肿瘤,局部切除多可治愈,组织学上需与巨细胞纤维母细胞瘤、软组织多形性透明变性血管扩张性肿瘤、血管瘤样型纤维组织细胞瘤相鉴别.     

Analysis of tumor suppressor gene on human chromosome 9 in mouse x human somatic cell hybrids

Deletions of the short arm of human chromosome 9 (9p) are common in human leukemia and solid tumors. The minimum region of overlap of these deletions, located between the interferon genes and the methylthioadenosine phosphorylase gene, is partially syntenic with a region of mouse chromosome 4 that has tumor suppressor activity. Somatic cell hybrids between tumorigenic, MTAP-deficient, mouse L cells, and MTAP-competent human cells containing either a normal copy of 9p or a 9p with a deletion involving band 9p21 were selected in culture conditions that require MTAP activity for continued growth. Somatic cell hybrids that contained a normal copy of 9p rarely formed tumors in nude mice. Cells from the rare tumors that grew had lost the normal 9p. Hybrid cells that contained a 9p with deletions formed tumors more frequently, and cells from these tumors retained the 9p deletion chromosome. These results provide evidence that a tumor suppressor gene (or genes) is located on human chromosome 9 within the region of deletion.