多发性子宫平滑肌瘤的克隆性

目的 探讨磷酸甘油酸激酶（PGK）位点克隆性分析技术对于确定局灶性或结节性病变的克隆组成的意义,以及子宫平滑肌瘤的克隆性及多发性肌瘤不同瘤结节之间的关系。方法 提取103例子宫平滑肌肿瘤新鲜组织DNA,HpaⅡ消化,巢式聚合酶链反应扩增PGK基因,产物经BstⅪ消化后,琼脂糖凝胶电泳显示结果。结果 103例组织标本中,32例（31％）具有BstⅪ酶切位点的多态性。对其中29例共89个瘤结节进行了检测,显示所有瘤结节均为单克隆性;1例多结节的平滑肌肉瘤中,检查过的7个瘤结节均为同一PGK等位基因被灭活;而多发性子宫平滑肌瘤不同瘤结节之间的关系较复杂,其酶切带型可以完全桢相同（8／15）或大部分相同（2／15）,也可以完全不同（5／15）。这种差别与瘤组织核分裂象指数无关。结论 PGK克隆性分析技术可用于确定局灶性或结节性病变的克隆组成;子宫平滑肌瘤是单克隆起源;多结节的子宫平滑肌瘤可能有各自独立型、局部侵袭型以及二者的混合型三种。     

子宫平滑肌瘤细胞核体的定量分析

人体子宫平滑肌瘤的发病机理尚未完全阐明,许多研究认为与雌激素长期大量持续刺激有关,并认为核体是雌激素刺激靶细胞的结构指示物。为探讨人体子宫平滑肌瘤与雌激素的关系,本研究用立体学的方法,对此肌瘤细胞核体作定量分析。结果表明子宫平滑肌瘤细胞核体的数密度显著高于正常平滑肌细胞核体的数密度,而体积大小无显著变化。这一发现证实人体子宫平滑肌疚的发生与雌激素的刺激有关。     

卵巢浆液性囊腺瘤合并平滑肌瘤附壁结节

卵巢浆液性囊腺瘤合并平滑肌瘤附壁结节吕翔张丽华周祀桥朱永斌作者单位：南京市鼓楼医院病理科２０００２４作者简介：吕翔，男，４２岁，博士，副主任医师。主要从事淋巴瘤、中枢神经系统肿瘤及肾上腺肿瘤方面的研究卵巢囊性肿瘤伴附壁结节（ｓｐｉｎｄｌｅｃｅｌｍｕｒ...     

起源未定的交界性和中间型软组织肿瘤

一、侵袭性血管粘液瘤 (ａｇｇｒｅｓｓｉｖｅａｎｇｉｏｍｙｘｏｍａ)1983年 ,Ｓｔｅｅｐｅｒ和Ｒｏｓａｉ首先提出了侵袭性血管粘液瘤的概念。本病主要见于女性。发病年龄 18～ 70岁 ,平均年龄 36岁 ,80 %的病例在 2 0～ 49岁年龄组。发生部位主要在盆腔和会阴。除盆腔外 ,女性主要见于外阴和阴道 ,男性则见于精索 ,腹股沟、阴囊、肛周和会阴。大体表现 :瘤体通常比较大 ,大小范围 3～ 6 0ｃｍ ,平均12ｃｍ。本瘤为一浸润性生长的肿瘤 ,但大体上境界尚清。切面鱼肉状 ,胶冻状 ,具有光泽。镜下表现 :肿瘤富于血管 ,血管一般不分支连成网 ,…     

交界性胃肠道间质瘤279例临床病理学分析

目的 阐述交界性胃肠道间质瘤(gastrointestinal stromal tumors,GISTs)的特征并重新评估目前广泛应用于GISTs的美国国家卫生研究所(NIH)共识标准.方法 回顾性分析840名患者的病史资料及手术切除标本.结果 筛除485例恶性GISTs及76例假定的良性GISTs,剩余的279例GISTs归为交界性GISTs,其中223例随访1～31年.2例患者局部复发,随后经过单纯手术切除治愈.5年无瘤生存率和总生存率分别为99%和100%.形态学上,交界性GISTs的典型特征为富于细胞、中度异型性、低核分裂活性(＜10个/50 HPF)或较大肿瘤体积.依据NIH共识标准,279例中,极低危险度组13例,低危组164例,中危组71例,高危组31例,但各组间的无瘤生存率差异无显著性(P=0.681).结论 交界性GISTs具有介于良恶性GISTs之间的临床及形态学特征.部分交界性GISTs表现出恶性潜能,需长期随访.NIH 共识标准基于肿瘤体积及核分裂象划分的危险度等级并不适用于评估交界性GISTs的生物学行为.     

外分泌胰腺的交界性肿瘤

传统上外分泌胰腺的肿瘤一般分为良性和恶性两大类 ,但随着对胰腺肿瘤认识的深入 ,也提出了胰腺交界性肿瘤的概念 ,新版ＷＨＯ肿瘤分类[1] 也列出了外分泌胰腺的交界性肿瘤的类型。目前认为外分泌胰腺的交界性肿瘤包括 :交界性粘液性囊腺瘤 ,导管内乳头状粘液腺瘤伴中度不典型增生 ,胰腺的实性 假乳头瘤。胰腺导管上皮的中度不典型增生也应属交界性病变。一、粘液性囊性肿瘤 (包括交界性粘液性囊腺瘤 )为一类由产生粘液的上皮细胞形成的囊性胰腺肿瘤。过去一直分为良性的粘液性囊腺瘤和恶性的粘液性囊腺癌。1978年Ｃｏｍｐａｇｎｏ和Ｏｅｒ…     

子宫奇异型平滑肌瘤的临床病理分析

目的探讨子宫奇异型平滑肌瘤组织形态、免疫组织化学及生物学行为特征.方法对25例子宫奇异型平滑肌瘤进行瘤体大体及镜下观察;20例行平滑肌肌动蛋白(SMA)、增殖细胞核抗原(PCNA)、雌激素受体(ER)、孕激素受体(PR)免疫组织化学SP和ABC法染色,20例子宫富于细胞性平滑肌瘤作为对照组;并行临床资料分析及跟踪随访.结果临床主要表现为阴道不规则出血、腹痛和盆腔包块,1例合并大量腹水,2例合并妊娠,无应用孕酮类药物者.镜下部分或全部胞核奇异,双核或多核易见,核内多见1至数个较大红染包涵体样物(直径7～26 μm),核分裂象0～2/10高倍视野(HPF).20例奇异型平滑肌瘤做 SMA染色,全部奇异瘤细胞胞质呈肌源性表达,与对照组相同;PCNA染色,观察组15例(75.0%)呈弱阳性,与对照组相比,差异有显著意义(P=0.027);在观察组中18例(90.0%)ER呈阴性表达,表现有ER丢失现象,对照组则全部阳性,两组相比差异有极显著性意义,P＜0.005;PR两组均呈阳性;同时发现大部分核内包涵体样物呈肌源性表达,即SMA阳性.长期随访无复发.结论子宫奇异型平滑肌瘤虽形态奇异,但属良性肿瘤.本组形态学变化与外源性孕酮药物无关,与妊娠关系应予以关注.免疫组织化学表达有一定特征,应与平滑肌肉瘤及恶性潜能待定平滑肌瘤进行鉴别.     

子宫肌层平滑肌瘤样血管瘤性神经内分泌肿瘤超微结构研究

Vajtai等（2006年）首次报道了1例发生于脑垂体的平滑肌瘤样血管瘤性神经内分泌肿瘤（leiomyomatoid angiomatous neuroendocrine tumor，LANT），并指出这种肿瘤可能是二态脑垂体肿瘤的变异体，与无功能细胞腺瘤有关。作者从组织学、免疫表型和超微结构方面报道了第2例LANT的特征。患者女性，45岁，子宫肌层肿瘤，临床诊断子宫平滑肌瘤，经腹腔镜行子宫肌瘤切除术。肉眼观肿瘤表面菜花样，灰白色。组织学主要有两种成分，一种为具有较小管腔，     

子宫上皮样平滑肌瘤的临床病理观察

目的 探讨子宫上皮样平滑肌瘤(epithelioid leiomyoma，EL)的临床病理特征和生物学特性。方法 收集14例子宫EL的临床和病理资料。随机抽取同期l0例子宫低度恶性间质细胞肉瘤作为对照。采用二步法进行actin、Des、EMA和CDl0免疫组化染色。结果 子宫EL临床表现无特征性。镜下显示肿瘤细胞呈类似上皮细胞样的圆形、卵圆形或多边形，核呈圆形或卵圆形，胞质嗜伊红染色或部分伴空泡化改变似核周晕，也可表现为富含糖原的透明细胞。平均核分裂象0～1个10HPF。14例EL的actin、Des全部阳性表达，EMA9例阳性，CDl0有2例呈弱阳性。l0例子宫低度恶性间质细胞肉瘤actin、Des、EMA和CDl0分别有5例、3例、2例和7例阳性表达。结论 子宫EL有特殊的病理形态特征；免疫组化有助于鉴别诊断。     

交界性肿瘤的临床和病理

1　交界性肿瘤的概念肿瘤从良性到恶性是一个连续的谱带 ,它需要一个漫长且极为复杂的细胞生物学演变过程。临床上所见到的一些肿瘤正是处于良性病变或良性肿瘤向恶性转化的某一个阶段 ,即为交界性病变和交界性肿瘤。交界性肿瘤的提出 ,最早始于Ｒｉｂｂｅｒｔ(1 91 4 )和Ｅｗｉｎｇ(1 938) ,他们认为将肿瘤截然划为良性、恶性两类是不够确切的。Ｗｉｌｌｉｓ(1 950 )指出 ,肿瘤的良恶性分类是相对的 ,在两类间有中间类型或边界肿瘤。 1 953年Ｗｉｌｌｉｓ正式提出了交国际妇女医师联盟 (ＦＩＧＯ) 1 961年对卵巢浆液性和黏液性肿瘤中新增了…     

The first case of gastric leiomyosarcoma developed through malignant transformation of leiomyoma

Malignant transformation of gastric leiomyoma has not been reported, and therefore it is considered to have virtually no malignant potential. We report a case of gastric leiomyosarcoma arising from leiomyoma. The patient is a 72-year-old man with a submucosal mass measuring 20 mm in diameter, which was incidentally identified by an endoscopic surveillance. A biopsy suggested a diagnosis of leiomyosarcoma, and local excision was performed. Pathological examination revealed that the tumor was composed of two distinct components: typical leiomyoma-like area in the periphery and leiomyosarcoma component exhibiting higher cellularity, prominent nuclear atypia, necrosis, and increased mitosis. Immunohistochemically, in the latter, p53 overexpression, increased Ki-67 labeling index, and attenuated expression of smooth muscle markers were noted. This is the first report to demonstrate the presence of leiomyoma–leiomyosarcoma sequence in the stomach that is well recognized in the uterus. Our observation highlights the potential occurrence of malignant transformation of gastrointestinal leiomyoma.     

Uterine leiomyosarcomas are characterized by high p16, p53 and MIB1 expression in comparison with usual leiomyomas, leiomyoma variants and smooth muscle tumours of uncertain malignant potential

AIMS: It has been suggested that p16 is overexpressed in uterine leiomyosarcomas in comparison with leiomyomas. In this study, p16 immunohistochemical expression was assessed in a variety of uterine smooth muscle tumours, including usual leiomyomas, leiomyoma variants, smooth muscle tumours of uncertain malignant potential (STUMPs) and leiomyosarcomas. The aim was to ascertain whether there are differences in p16 expression between these groups and whether p16 is of potential value in the assessment of problematic uterine smooth muscle neoplasms. p16 expression was also compared with that of p53 and MIB1. METHODS AND RESULTS: Cases of usual leiomyoma (n = 10), leiomyoma variants (n = 27), STUMP (n = 4) and leiomyosarcoma (n = 22) were subject to p16, p53 and MIB1 immunohistochemistry. For p16, cases were evaluated with respect to both staining distribution and intensity. There was a statistically significant difference in p16 distribution (P < 0.001) and intensity (P = 0.001) between leiomyosarcomas and the other groups. There was no difference in p16 expression between usual leiomyomas, leiomyoma variants and STUMPs. There were also statistically significant differences in p53 (P = 0.014) and MIB1 (P < 0.001) immunoreactivity between leiomyosarcomas and the other groups. CONCLUSIONS: p16 is overexpressed in uterine leiomyosarcomas compared with leiomyomas, benign leiomyoma variants and STUMPs, suggesting that p16 may be implicated in the pathogenesis of malignant uterine smooth muscle neoplasms. p16, in combination with p53 and MIB1, may be of value as an adjunct to morphological examination in the assessment of problematic uterine smooth muscle tumours, although further large-scale studies with follow-up are necessary to confirm this.     

Uterine leiomyosarcoma arising in leiomyoma: Clinicopathological study of four cases and literature review

In this study we examined four cases of leiomyosarcoma (LMS) arising in leiomyoma (LM). The patients ranged from 40 to 64 years old. Symptoms were unspecific and preoperative diagnosis of LMS had not been made. Size of the tumors ranged from 4 cm to 20.5 cm. All patients were in stage I and no patients developed recurrent disease. Histologically, the LMS component showed moderate to severe cytological atypia and an increased mitotic count in all cases. Tumor cell necrosis in the LMS was seen in three cases. Associated LM component was cellular in one case. The LMS component showed p53 expression in three of four cases and p16 expression was seen in two cases. The LM component lacked p53 expression in all cases and demonstrated p16 expression in two cases, including one case of cellular LM. In all cases, Ki‐67‐positive cells were less than 5% in the LM component and more than 25–30% in the LMS component. This study reveals that: (i) malignant transformation can occur even in relatively small LM; (ii) patients with LMS with an LM component have a favorable prognosis; and (iii) immunohistochemical examination of p53, p16 and Ki‐67 is useful for identification of malignant focus.     

原发性皮肤恶性淋巴瘤的临床病理和免疫组化研究

研究原发性皮肤恶性淋巴瘤的类型及临床病理特点,方法对４２例ＰＣＭＬ作了形态学观察,免疫表型标记和组织学分型。结果原发性皮肤Ｔ细胞性淋巴瘤（ＰＣＴＣＬ）占６９．０％,其中蕈样霉菌病占７．２％（ＭＦ）、血管中心性淋巴瘤占９．５％皮下指明脂膜炎样Ｔ细胞巴瘤占１９．０％,原发性皮肤Ｂ细胞性淋巴瘤（ＰＣＢＣＬ）占３１．０％;ＰＣＭＬ男女之比为２：１,中位年龄３７岁,ＰＣＴＣＬ的发病年龄小于ＰＣＢＣＬ（     

Uterine leiomyoma versus leiomyosarcoma: a new attempt at differential diagnosis based on their cellular characteristics

AIM: To differentiate uterine leiomyoma (LM) with 'pseudosarcomatous' features from leiomyosarcoma (LMS). We hypothesized that because pseudosarcomatous LM is only a benign variant of conventional LM, it may have some characteristics different from LMS. METHODS AND RESULTS: Fourteen uterine smooth muscle tumours, which had been diagnosed as LMS previously, were reviewed and divided into adverse outcome (true LMS; seven cases) and excellent outcome groups (presumed pseudosarcomatous LM; seven cases) based on their clinical course. The tumours of the adverse outcome group were composed of rather uniform long spindle cells similar to those of leiomyosarcoma arising from the soft tissue. All tumours in this group had coagulative necrosis and all but one case were diagnosed preoperatively as malignant. In contrast, the tumours of the excellent outcome group showed significant histological diversity both between tumours and also in the same tumour and areas indistinguishable from conventional LM were often seen. All the tumours of this group were diagnosed preoperatively as benign. Immunohistochemically, all tumours of the excellent outcome group expressed progesterone receptor, often intensely, and also frequently expressed oestrogen receptor. In contrast, all the tumours of the adverse outcome group lacked them completely. CONCLUSION: Pseudosarcomatous LM has characteristics useful for differentiating it from LMS, which are common in conventional LM.     

肺良性转移性平滑肌瘤1例报道及文献复习

目的 分析肺良性转移性平滑肌瘤(benign metastasizing leiomyoma,BML)的临床和组织病理学特点,研究其免疫组化表型,讨论诊断和鉴别诊断.方法 对1例BML进行光镜观察及免疫组化标记,并复习文献.结果 中年女性患者,有子宫平滑肌瘤手术史,胸部CT示右肺多发性结节.切除标本见2个灰白色结节,直径分别为6 cm、1.2 cm,边界清楚,镜下见肿瘤细胞较丰富,并见上皮衬覆的管腔样结构,未见坏死及核分裂象,与原发子宫平滑肌瘤形态相似.免疫组化证实为平滑肌源性,且表达雌激素受体.结论 BML是一种罕见的疾病,多发生在有子宫平滑肌瘤病史的女性,转移部位主要是肺,组织学形态表现良性,手术切除是主要治疗方法 .     

Primary Smooth Muscle Tumor of the Pleura: A Clinicopathological Case Report with Ultrastructural Observations and a Review of the Literature

Primary smooth muscle tumor of the pleura is exceptionally rare. The authors describe a primary smooth muscle tumor of the pleura that was discovered incidentally on chest X-ray in a 73-year-old man. Magnetic resonance imaging demonstrated a 12 × 18 × 15-cm pleura-based mass arising from the posterior mediastinum. Computerized tomography (CT) guided needle cores from the pleura showed a primary smooth muscle tumor of undetermined malignant potential. Further excision of the whole tumor showed an intimate relation to pleura, and the diagnosis of leiomyosarcoma was made. The clinical, radiological, histopathological, immunohistochemical, and ultrastuctural findings were consistent with a primary smooth muscle tumor of the pleura. This is the seventh case in the literature of a primary smooth muscle tumor of the pleura, which, to the best of the authors' knowledge, is the first such case of the pleura to be diagnosed on CT-guided needle biopsy. In conclusion, this method of investigation is recommended since it is minimally invasive but has a rewarding yield in providing the most likely diagnosis, predicting prognosis, and management planning.     

Primary smooth muscle tumor of the pleura: a clinicopathological case report with ultrastructural observations and a review of the literature

Primary smooth muscle tumor of the pleura is exceptionally rare. The authors describe a primary smooth muscle tumor of the pleura that was discovered incidentally on chest X-ray in a 73-year-old man. Magnetic resonance imaging demonstrated a 12 × 18 × 15-cm pleura-based mass arising from the posterior mediastinum. Computerized tomography (CT) guided needle cores from the pleura showed a primary smooth muscle tumor of undetermined malignant potential. Further excision of the whole tumor showed an intimate relation to pleura, and the diagnosis of leiomyosarcoma was made. The clinical, radiological, histopathological, immunohistochemical, and ultrastuctural findings were consistent with a primary smooth muscle tumor of the pleura. This is the seventh case in the literature of a primary smooth muscle tumor of the pleura, which, to the best of the authors' knowledge, is the first such case of the pleura to be diagnosed on CT-guided needle biopsy. In conclusion, this method of investigation is recommended since it is minimally invasive but has a rewarding yield in providing the most likely diagnosis, predicting prognosis, and management planning.     

Myxoid leiomyoma of the vulva mimicking aggressive angiomyxoma

A case of vulvar leiomyoma with extensive myxoid change in a 40 year old female is described. The tumor had a unique connection with a non-degenerative leiomyoma that compressed the rectum and the bladder. Scattered smooth muscle cells in a loose myxoid stroma were immunoreactive for desmin. Fibroblast-like spindle cells were immunoreactive for vimentin but not for desmin. The initial, although incorrect, pathological diagnosis of the tumor was aggressive angiomyxoma based on the similarity in both clinical and pathological aspects with this more invasive tumor. Myxoid vulvar leiomyoma should also be differentiated from angio-myoflbroblastoma. The key to the differential diagnosis is the presence of interlacing smooth muscle cells and an awareness of tendency toward myxoid change in vulvar leiomyomas.