婴儿色素性神经外胚瘤的临床病理研究

目的 探讨婴儿色素性神经外胚瘤的临床病理特点。方法 对2例婴儿色素性神经外胚瘤进行临床资料分析和组织病理学、免疫组织化学及电镜观察。结果 该肿瘤多发生于1岁以内的婴幼儿。主要发生在上、下颌骨，呈浸润性生长、溶量性破坏。组织学特征为：肿瘤由含色素的上皮样细胞和小的神经母细胞样细胞构成。免疫组化结果为：上皮样瘤细胞表达CK、EMA、HMB45、S－100蛋白及Vim，小的瘤细胞则表达NSE，Syn及Vim。超微结构观察：大的瘤细胞质内含黑色素小体，小的瘤细胞质内见神经内分泌颗粒。结论 婴儿色素性神经外胚瘤起源于原始的神经外胚层细胞，为低度恶性肿瘤。     

黑色素性神经外胚瘤临床病理学观察

 目的　探讨黑色素性神经外胚瘤（MNT）的临床病理特征和鉴别诊断。方法　收集2例MNT资料并复习文献。结果　MNT多发生于1岁内的婴儿，个别年龄较大。上下颌骨多见。肿瘤由含色素的上皮样细胞和小圆形神经母细胞样细胞构成。上皮样瘤细胞常表达Vimentin、CK、EMA、HMB45，小圆形神经母细胞样瘤细胞表达CD57、Syn，两类瘤细胞S-100通常阴性。结论　MNT是良性肿瘤，起源于神经外胚层，很罕见，具有典型的临床特征和组织学形态。需要警惕的是婴儿MNT呈浸润性生长，速度惊人，溶骨性破坏，术后复发率高。     

胃肠道间质瘤87例临床病理分析

  目的 探讨胃肠道间质瘤（GISTs）的临床病理特点。方法 应用常规病理及免疫组化观察87例胃肠道间质瘤，并复习相关文献。结果 本组GISTs均为成年人，年龄28 ~ 73岁，平均53岁。发生于胃56例，小肠29例，结肠2例。镜下肿瘤由梭形细胞和上皮样细胞以不同的比例构成。瘤细胞vimentin均阳性，CD117，CD34多为弥漫强阳性，SMA、S-100在肿瘤向平滑肌和神经方向分化时阳性。结论 GISTs好发于中老年人，肿瘤由梭形细胞和上皮样细胞构成，免疫组化特征为CD117，CD34阳性。     

黑色素性神经鞘瘤的组织发生、鉴别诊断与预后

目的：探讨黑色素性神经鞘瘤组织发生、鉴别诊断及预后。方法：3例患者年龄,分别4、17、28岁,黑色素性神经鞘瘤用组织学（HE）、免疫组化（ABC法）和电镜技术进行检测。结果：肿瘤直径为2、4、8cm。组织学：由黑色素性梭形细胞和黑色素性多边形细胞组成。梭形细胞呈束状、交织状、小区呈栅状排列,多边形细胞呈疏松片或网状。退色素后可见核呈卵圆形,核仁明显,核分裂相可见,部分细胞有异型。2例有小区浸润。免疫组化：S-100蛋白、HMB45、Vim、Leu-7、NSE等阳性。电镜观察见肿瘤胞质内大量Ⅱ-Ⅳ期黑色素小体及细胞膜外少量基膜。结论：①黑色素性神经鞘瘤好发于青年人,多数为良性或潜在恶性,少数为恶性,需要随访。②黑色素性神经鞘瘤来自神经嵴具有双向分化潜能的细胞。     

伴上皮样细胞分化的周围神经肿瘤

周围神经肿瘤起源于原始神经嵴。由于原始神经嵴细胞的多向分化潜能，使得周围神经肿瘤中常常出现一些异常分化成分，如上皮样细胞、腺体、横纹肌、骨、软骨和黑色素等，从而给肿瘤的诊断和分类造成困难。本文报告腺性神经纤维瘤，上皮样恶性神经鞘膜瘤和婴儿黑色素性神经外胚叶瘤各1例。重点讨论有上皮细胞分化特征的周围神经肿瘤的诊断和鉴别诊断。临床和病理资料3例伴上皮样细胞分化的周围神经肿瘤。例1男，46岁，右胸壁肿块2×2cm，无包膜。病理形态，在神经纤维瘤背景中出现成团分化成熟的粘液腺泡。免疫组化为棱形细胞S-100（＋），S…     

肝脏血管平滑肌脂肪瘤临床病理分析

目的 探讨肝脏血管平滑肌脂肪瘤(HAML)的临床病理特征.方法 结合文献对1例HAML的临床资料、免疫组化、超微结构和随访结果进行分析.结果 HAML女性好发,成人多见,常无症状,体检发现:影像学因肿瘤成分构成比和分布不同而异;肿瘤由厚壁畸形血管、成熟脂肪和多形肌样细胞构成,并常见有单核、多核和巨核细胞呈散在巢团状集聚和灶性髓外造血灶;上皮样肌样细胞HMB45、CD117阳性及超微结构见不同成熟时期黑色素小体;治疗以手术为主.结论 HAML主要由血管、脂肪、平滑肌3种成分组成,其中变化最大的是肌样细胞,肌样细胞HMB45标记及电镜检查不同发育时期黑色素小体是诊断HAML的关键.HAML多为良性,首选手术,但有恶性HAML的报道.     

肝脏血管平滑肌脂肪瘤的临床病理与免疫组织化学特征

目的探讨肝血管平滑肌脂肪瘤（HAML）的临床病理及免疫组织化学特征。方法对4例HAML进行病理组织学观察，用免疫组织化学检测HMB45等10种抗体在瘤细胞中的表达。结果其中3例肿瘤组织由平滑肌、脂肪和血管按不同比例构成，1例由单一的上皮样细胞构成。免疫组织化学检测4例均显示黑色素标记HMB45阳性和肌源性标记SMA阳性。结论HAML由平滑肌、脂肪及血管按不同比例构成，形态变异大，瘤细胞表达黑色素标记，HMB45阳性具有诊断价值。     

《天津医药肿瘤学》附刊第10卷索引

病理分类痛疤痕癌恶性淋巴瘤恶性纤维组织-细胞瘤恶性肿瘤非何杰金氏淋巴瘤非移行细胞上皮癌骨髓瘤海绵状血管瘤黑色素瘤黑色素神经外胚瘤横纹肌肉瘤滑膜肉瘤化质骨质纤维瘤肌瘤脊索瘤卡波西氏肉瘤类癌鳞状细胞癌烦咽管瘤粘液纤维瘤囊肿内胚窦瘤平滑肌肉瘤胚瘤(髓上皮瘤)其它韧带样瘤乳头涎腺瘤软纤维瘤神经源性肿瘤嗜伊红细胞增生性-淋巴肉芽肿性索瘤星形细胞瘤血管瘤炎性假瘤隐球菌病尤文氏启肿瘤转移癌组织细胞增生症X病理分类索引瘤血液稀释自家输血在食管(责门)癌根治术中 的应用(附30例报告)、20(l):11结肠食代管手术治疗晚期食管癌3…     

胃肠道间质瘤53例临床病理及免疫组化分析

目的探讨胃肠道间质瘤的临床病理及免疫组化特点.方法结合免疫组化和病理形态学观察,对53例胃肠道间质瘤进行分析.结果胃肠道间质瘤最常见于胃,其次为回肠、直肠.其中胃31例伴其中肝转移1例,空肠1例,十二指肠、空肠多发1例,回肠14例,直肠6例.镜下瘤组织主要由梭形细胞和或上皮样细胞构成.CD 34、CD 117有很高的阳性表达率,SMA、S-100、NSE在向平滑肌、神经各自的分化有一定的表达率.结论胃肠道间质瘤由非上皮性梭形细胞和(或)上皮样细胞构成,免疫组化CD 34、CD 117有助于做出正确的病理诊断.     

肾外恶性横纹肌样瘤临床病理分析

目的探讨肾外恶性横纹肌样瘤的临床病理学特征及诊断和鉴别诊断。方法对2例肾外少见部位恶性横纹肌样瘤术后标本行HE、免疫组织化学染色（EnVision法）并结合文献复习。结果光镜下见瘤细胞椭圆形或多边形,胞浆丰富嗜酸性,似横纹肌母细胞;核卵圆形或圆形,呈泡状、核仁明显,部分细胞核偏位,似印戒细胞;少数瘤细胞胞浆内见嗜酸性小体样结构,免疫组化见瘤细胞CK、EMA及Vim均阳性,部分瘤细胞Syn阳性。结论该肿瘤是一种少见、高度恶性、病理形态独特的肿瘤。     

Melanotic neuroectodermal tumor of infancy. A review of seven cases

The melanotic neuroectodermal tumor of infancy (MNTI) is a rare, usually benign, pigmented neuroectodermal tumor which most often involves the maxilla. The authors reviewed seven cases of MNTI, with patient ages of our patients ranged from nine weeks to 18 months; six of the seven were less than six months old at initial diagnosis. Four patients were males, and all were white. One tumor each was located in the femur, the temporal bone, and the epididymis; the remaining lesions occurred in the maxilla. Three of the four maxillary tumors recurred locally; the epididymal and femoral tumors metastasized. Two of these cases had unique clinical or pathologic features. The case of the femoral tumor is remarkable in that it is the first reported one of MNTI presenting in a long bone. This tumor was aggressively malignant; within two months after its discovery, a large mass of similar tumor was formed in the pelvis, and the tumor resulted in the patient's death. To the authors' knowledge, the case of the temporal bone tumor is the first one of MNTI in which neuronal differentiation of the neuroblastic cells is convincingly demonstrated. This finding provides additional evidence in support of the neuroectodermal theory of origin of these neoplasms.     

Melanotic neuroectodermal tumor of infancy: Report of a case with ganglionic differentiation

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare but well-documented lesion of neuroectodermal derivation. Maturation of the neural elements has been reported only occasionally. We report a case of MNTI of the maxilla showing maturation of neural elements to ganglionic cells. © Wiley-Liss, Inc.     

Malignant melanotic neuroectodermal tumor: light and electron microscopic study

An 11-year-old white boy had a melanotic neuroectodermal tumor of infancy (MNTI) in his right mandible. Gross-examination showed that the tumor had originated in the right lower dental nerve, destroyed the right mandible, infiltrated the surrounding soft tissues, and metastasized to several lymph nodes. The typical alveolar pattern was observed in most of the tumor mass; however, solid areas with neuroblastic features were present in the infiltrating and metastasizing portions of the tumor. Ultrastructural study demonstrated unequivocal neuroblastic and melanocytic differentiation.     

Large cell medulloblastoma with myogenic and melanotic differentiation: a case report with molecular analysis

We present a case of large cell medulloblastoma with myogenic and melanotic differentiation arising in the cerebellar vermis of a 2-year-old boy and following an aggressive clinical course. Histologic and immunohistochemical features of this tumor include primitive neuronal, rhabdomyoblastic, and pigmented epithelial elements, along with large cell features. Immunohistochemical and molecular data (c-myc gene amplification and the presence of isochromosome 17q) support the contention that this histologically diverse tumor represents a pattern of medulloblastoma with striated muscle and pigmented epithelial differentiation, rather than a teratoma or a cerebellar variant of melanotic neuroectodermal tumor of infancy (‘progonoma’).     

Malignant melanotic neuroectodermal tumor of infancy: a clinical, pathologic, ultrastructural and tissue culture study.

The melanotic neuroectodermal tumor of infancy is an uncommon neoplasm typically of early childhood which has a predilection for the head and neck region, particularly the maxilla. Except for one previous example in the literature, this tumor has consistently behaved in a benign fashion. This study documents the clinical course and pathologic findings of a tumor which began in the maxilla of a 4-month-old boy, followed by a local recurrence, metastasis to a cervical lymph node and finally, widespread dissemination and death at 18 months, 24 months and 38 months, respectively. The tumor was initially composed of nests consisting of melanin-containing cells and small dark cells. An elevated vanillylmandelic acid level was recorded during the course of the disease. At autopsy, the tumor in lymph nodes, liver, bone and soft tissues had a monotonous pattern of small dark cells similar to a conventional neuroblastoma. Previous ultrastructural studies indicate that the melanotic neuroectodermal tumor of infancy is composed of melanocytes and neuroblast-like cells. Our case provided the unique opportunity to examine in sequence the ultrastructural and in vitro characteristics of a recurring and eventually metastasizing melanotic neuroectodermal tumor. Although the neuroblast-like cells were initially difficult to identify by electron microscopy, a melanin-producing cell line and a separate nonpigmented cell line were successfully isolated from various tumor explants. Various stages of melanosome development were identified in the pigmented cells from the local recurrences and in vitro. Dibutyryl cAMP accentuated the formation of pigment and dendritic development in the melanocytes and dendrites only in the small nonpigmented cells. Electron dense granules were observed in the cultured smaller cells and also in the lymph node and soft tissue metastases. Tyrosine hydroxylase activity was demonstrated in the neuroblast-like cells. In the final biopsy and autopsy material, only the neuroblast-like cells remained and the tumor resembled a conventional neuroblastoma.     

Establishment and characterization of malignant rhabdoid tumor of the kidney

Malignant rhabdoid tumor of the kidney (MRTK) is a highly aggressive tumor which occurs in childhood and which is histologically characterized by the existence of eosinophilic intracytoplasmic inclusions. We established and characterized a cell line from this tumor with histological, immunohistochemical and cytogenetical analysis. Histologically, the tumor cells demonstrate typical eosinophilic inclusions, while immunohistochemically the cells demonstrate common mesenchymal and epithelial differentiation. Although the conventional karyotyping of this tumor lacked the abnormalities of 22q chromosome, Southern blot analysis and microsatellite analysis verified abnormalities of the BCR gene and of the hSNF5/INI1 gene. Despite the variety of locations, these common genetic abnormalities appear to contribute to distinguish rhabdoid tumor from such other small round cell tumors as primitive neuroectodermal tumor, rhabdomyosarcoma, poorly differentiated synovial sarcoma and desmoplastic small round cell tumor.     

小腿软组织促纤维组织增生性小圆细胞肿瘤临床病理观察(附1例)

目的:探讨促结缔组织增生性小圆细胞肿瘤(DSRCT)的临床病理特点及免疫组化表型及鉴别诊断。方法:对1例DSRCT患者的临床特征、组织学形态及免疫组化表型进行研究,并复习有关文献。结果:DSRCT由成巢的小圆细胞组成。核深染、浆少。纤维组织增生性间质围绕肿瘤细胞巢,可伴透明变性或黏液样变。间质内可有明显血管:毛细血管丛,较大厚壁血管。提示是对肿瘤的增生性反应。核分裂像多。免疫组化多种表型,可表达上皮性、肌性和神经性分化CKAE1/AE3(+)、Vim(+)、Des(+)、NSE(+)、CD99(+)、EMA灶(+)、Myoglobin(+)等。结论:DSRCT为罕见的、侵袭力强的高度恶性肿瘤,瘤细胞具有神经、间叶和上皮标记复合表达。主要累及儿童和年轻男性,一般表现为广泛的腹腔浆膜受累,发病年龄20-30岁。95%发生在腹腔内,常位于后腹膜、盆腔、网膜和肠系膜。临床上位于腹腔外的病例非常罕见,主要在胸腔和睾丸旁区域,个别病例发生在肢体,头颈部和大脑。侵袭性高,预后差,70%因广泛转移而亡。     

Morphological and immunohistochemical studies of the estrogen-induced Syrian hamster renal tumor: probable cell of origin

Chronic natural or synthetic estrogen treatment of Syrian golden hamsters leads to the development of malignant renal neoplasms. In the present study, morphological and immunohistochemical studies were performed to further characterize the estrogen-induced hamster renal tumors. The neoplasms were composed of two distinct cell populations: a large-cell component that appeared highly epithelial, and a poorly differentiated small-cell component. Importantly, both cell types had epithelial characteristics, since they contained desmosomes at their cell surfaces. However, the large-cell component possessed additional epithelial features such as microvilli, intracytoplasmic lumens, and cilia. Comparative studies of renal tumors and developing renal tissue from fetal and newborn hamsters revealed remarkable histological similarities. Morphologically, the large tumor cells resembled early metanephric tubules and the small tumor cells were very similar to the blastemal cells of the developing kidney. The earliest tumor foci were found after 4.5 months of treatment. They were consistently found in the kidney interstitium in proximity to large arteries. Immunohistochemical staining for intermediate filaments in developing fetal and newborn kidneys demonstrated cytokeratin in renal tubules, desmin in blastemal cells, and vimentin in stromal cells. Estrogen-induced renal tumor cells uniquely possessed reactivity for all three intermediate filaments, clearly demonstrating their epithelial and mesenchymal characteristics. Based on their morphological resemblance to developing embryonic kidney cells and the presence of both epithelial and mesenchymal intermediate filaments, our findings provide strong evidence that the cell of origin of this malignant tumor is a precursor cell that is committed to an epithelial differentiation pathway.     

外周原始神经外胚层肿瘤形态学免疫表型及临床预后研究

目的：探讨外周原始神经外胚层肿瘤(PNET)的临床表现，病理及免疫组织化学特点及预后。方法：对15例外周原始神经外胚层肿瘤进行临床资料及预后，病理组织形态学和超微结构特点及免疫组化表型研究。结果：患者以男性为主，男女之比约为6．5：1，年龄6～35岁，年龄中位数为16，发生部位较多，可发生在直肠，腹膜后，腹股沟，淋巴结，胸壁，睾丸，鼻腔及骨组织等；免疫组化：肿瘤均弥漫表达CD99，并不同程度地表达NSE，SYN，CgA等，但不表达CK，Desmin，LCA等，其中1例表达组织化学染色PAS，随访最长时间为24月。结论：PNET是一种发生在年轻男性，进展迅速，预后非常差的恶性小圆细胞肿瘤，认识其临床病理特点及免疫组化表型对于该恶性肿瘤的诊断及临床治疗意义重大。     

胸腺非典型性类癌的临床病理及免疫组化表型

目的:了解胸腺非典型性类癌的临床病理特征。方法:收集2例胸腺非典型性类癌的临床资料,手术切除组织光镜切片观察,另作10项免疫组织化学标记:CKp,EMA,Ki-67,CD3,CD20,CD5,TdT,NSE,CgA,Syn。结果:2例胸腺非典型性类癌均为男性,纵膈肿瘤,由小园形细胞构成。瘤细胞CKp、EMA、NSE、CgA、Syn阳性,CD3、CD5、CD20、TdT阴性,Ki-67核阳性细胞指数>20%,肿瘤有显著坏死。结论:非典型性类癌是一种罕见的胸腺肿瘤,诊断靠病理组织学和免疫组织化学标记,手术切除为主要治疗方法。