p63蛋白在软骨母细胞瘤中的表达及其价值探讨

目的探讨p63蛋白在软骨母细胞瘤中的表达水平及其诊断价值。方法 采用免疫组织化学法(Maxvision)技术对25例软骨母细胞瘤、52例骨巨细胞瘤、10例动脉瘤样骨囊肿和20例腱鞘巨细胞瘤中p63和S-100p蛋白的表达水平进行分析。结果 p63蛋白在软骨母细胞瘤中呈单核细胞的胞核阳性,阳性率为88.0%(22/25)。而骨巨细胞瘤中除呈单核细胞的胞核阳性,少数多核巨细胞的胞核亦呈阳性,阳性表达率为92.3%(48/52)。p63蛋白在软骨母细胞瘤和骨巨细胞瘤中的表达两者比较差异无统计学意义(P>0.05),而S-100p在软骨母细胞瘤和骨巨细胞瘤中阳性表达率分别为88.0%(22/25)和1.9%(1/52),两者差异有统计学意义(P<0.05)。p63蛋白和S-100p在动脉瘤样骨囊肿和腱鞘巨细胞瘤中均不表达。结论 p63蛋白在软骨母细胞瘤和骨巨细胞瘤中均呈高表达,并有相似的免疫表型,提示两者在发生分化上可能均来自相同的起源细胞或肿瘤干细胞。p63蛋白结合S-100p联合应用对鉴别软骨母细胞瘤及其相关性病变具有一定辅助诊断意义。     

骨外骨软骨瘤

骨外骨软骨瘤 (或软组织骨软骨瘤 )是指发生在骨以外软组织中与骨骼及骨膜无关联的良性肿瘤性骨软骨组织。软组织中成软骨、成骨性良性肿瘤较少 ,其中以软组织软骨瘤为多 ,其次为软组织骨瘤。在各种肿瘤专著中 ,不少有这两种疾病的专门章节 ,唯独不见骨外骨软骨瘤 ,可见其病理学地位和定义尚不明确。本病罕见 ,三十年代Ａｌｐｅｒｓ首先报道了起源于硬脑膜的颅内骨软骨瘤 ,Ｎｅｕｍａｒｋ则报告位于舌组织内的骨软骨瘤。八十年代初报告的有所增加[1～ 2 ] 。国内胡圣光首先报告 1例大网膜骨软骨瘤[3 ] 。八十年代后报告逐渐增加[4～ 6] 。这…     

大网膜原发性骨外软骨瘤1例并文献复习

目的:探讨大网膜原发性骨外软骨瘤的临床病理特征、鉴别诊断、治疗及预后。方法:分析一例33岁女性患者的临床资料,对病理标本进行常规HE染色和免疫组化染色,对染色结果进行分析判读,并检索国内外文献进行复习。结果:超声及CT均提示患者腹腔有包块;大体标本检查肿瘤组织部分呈分叶状,界清、质硬、灰白,周边见部分网膜组织;组织病理检查肿瘤由成熟透明软骨构成,呈小叶状排列,细胞无异型性;免疫组化检测肿瘤组织S-100阳性,Ki67阳性指数<1%;随访患者3年无复发或转移,各项结果共同支持大网膜原发性骨外软骨瘤的诊断,临床治疗方法首选完整切除。结论:大网膜原发性软骨瘤极其罕见,其发病机制尚不明确,治疗以手术完整切除为主。     

手部各骨的肿瘤与瘤样病变：附190例分析

作者复习了我院近18年收治的发生于手部各骨(指、掌与腕骨)的肿瘤与瘤样病变190例,每例均经病检证实。以软骨瘤最多(76.3%),其次是骨软骨瘤(11.5%).转移癌只有2.6%,骨巨细胞瘤(1.6%)与软骨肉瘤(1.1%)亦少见.作者对上述肿瘤与瘤样病变的临床,X线特点进行分析并结合有关文献探讨。     

单中心肩胛骨肿瘤166例流行病学分析

目的：分析肩胛骨肿瘤的流行病学特征及影像学特点,提高对肩胛骨肿瘤的认知及疾病诊断水平。方法回顾1980年至2014年,北京积水潭医院骨肿瘤科收治的资料完整的166例肩胛骨肿瘤病例。总结分析患者的性别、年龄、病理类型、肿瘤部位及影像学特点。结果原发肿瘤133例,转移瘤33例。男111例,女55例,男女比例2.02∶1,年龄5～80岁。原发恶性肿瘤74例,占44.58%;转移瘤33例,占19.88%;中间型肿瘤18例,占10.84%;良性肿瘤41例,占24.70%。原发恶性肿瘤中位发病年龄为31(6～80)岁,常见肿瘤有软骨肉瘤、Ewing 肉瘤、骨肉瘤;转移瘤中位发病年龄为54(36～79)岁,常见原发部位为肺癌、乳腺癌、前列腺癌;中间型肿瘤中位发病年龄为18(5～62)岁,常见病种有软骨母细胞瘤、朗格汉斯细胞组织细胞增生症、骨巨细胞瘤;良性肿瘤中位发病年龄25(10～62)岁,常见病种有骨软骨瘤、内生软骨瘤、单纯性骨囊肿。结论肩胛骨肿瘤中,恶性占大多数,转移瘤、软骨肉瘤、Ewing 肉瘤、骨肉瘤较常见,良性肿瘤中以骨软骨瘤最多。     

颌骨软骨肉瘤cyclin D1和cdk4的表达及意义

 目的　分析细胞周期素D1(cyclinD1)和细胞周期素依赖激酶 (cdk4 )在颌骨软骨肉瘤的表达及意义。方法　免疫组化ABC法检测cyclinD1和cdk4在 2 0例颌骨软骨肉瘤、8例骨软骨瘤和 4例软骨瘤的表达。结果　软骨肉瘤cyclinD1和cdk4阳性表达率分别为 70 % (14 /2 0 )和 6 5 % (13/2 0 ) ,二者的阳性表达存在正相关 (rs=0 .5 2 6 ,P <0 .0 5 ) ;而它们在骨软骨瘤的阳性表达率均为 12 .5 % (1/8) ,在软骨瘤无表达 ,与软骨肉瘤相比有显著性差异 (P <0 .0 5 )。结论　CyclinD1和cdk4在颌骨软骨肉瘤过表达且与其发生和发展有关。     

骨软骨瘤平片、CT表现及其比较分析(附19例报告)

目的分析骨软骨瘤的平片和CT表现,提高对其的认识.方法检查19例骨软骨瘤,4例做CT检查,15例做X-ray检查.结果19例骨软骨瘤,多发者2例,单发者17例.长骨干骺端共计18例,其他骨16例.X-ray表现:骨性突起,以蒂或宽阔的基底与骨皮质相连,呈圆形或菜花状.CT表现:与骨皮质相连的骨性突起,以蒂、宽或窄的基底连于骨皮质,局部骨皮质可增厚,密度不均匀,可见软骨帽低密度影,无骨质破坏,骨膜反应及软组织肿块.结论CT是一种有效的检查手段,不但可以观察肿瘤与周围组织的关系,更重要的是可以显示软骨帽.     

软组织软骨瘤临床病理学特征分析

目的:探讨软组织软骨瘤(STC)临床病理学特征及其组织学发生机制.方法:对11例STC的临床表现、组织学形态及免疫组化表型进行分析.结果:临床上主要表现为局部缓慢性生长的无痛性肿块,少数伴有局部压痛,术后罕见复发.影像学显示,与骨无关联的软组织内肿块伴有程度不等的钙化.镜下肿瘤组织境界清楚,分叶状,由成熟透明软骨或纤维软骨构成,可伴有程度不等的钙化、黏液变及囊性变;软骨瘤细胞形态不一,多无异形性,核分裂像罕见;少数软骨小叶周边梭形间质细胞密集,无胞周空晕或陷窝状结构.免疫组化显示,所有成分均表达vimentin,成熟软骨细胞及梭形软骨细胞还部分表达S100及CD34;软骨结节周边梭形间质细胞部分表达a-SMA、MSA及CD34,散在表达CD163;透明软骨基质CollagenⅡ表达程度不等,Collagen Ⅰ及CollagenⅢ呈阴性表达或局灶弱阳性表达;纤维软骨基质及钙化区表达Collagen Ⅰ及CollagenⅢ,CollagenⅡ表达阴性或局灶弱阳性表达.结论:STC是一种较为少见的软组织良性软骨性肿瘤,STC的形成可能最初由间充质细胞演化为具有CD34阳性表达的梭形间质细胞,继而向软骨方向分化形成STC,但这一演化机制有待深入研究.     

软骨肿瘤中骨形成蛋白的原位杂交定位和定量分析

目的 探讨骨形成蛋白２．３,４．５在软骨肉瘤和软骨瘤形成中的作用。方法 运用原位杂交技术,检测软骨肉瘤和软骨瘤中ＢＭＰ２、３、４、５ｍＲＮＡ的分布和定位,并采用图像分析对原位杂交的阳性程度进行图像分析。结果 软骨肉瘤中分化较差的梭形细胞中检测有ＢＭＰ２,３,４ｍＲＮＡ的表达,而分化状态较好的圆形、卵圆形细胞中未见阳性信号,软骨基质中未见阳性信号,而软骨瘤中的瘤细胞中只检测到ＢＭＰ３ｍＲＮＡ的表达。     

手足部内生软骨瘤及原发性软骨肉瘤的临床病理分析

目的 探讨手足短管状骨内生软骨瘤及原发性软骨肉瘤的影像学及临床病理学特征,为二者的诊断及鉴别诊断提供依据.方法 回顾性研究手足短管状骨的内生软骨瘤204例,年龄6～72岁（平均34岁）,高峰年龄20～40岁,女∶男约为2∶1.其中指骨166例,掌骨34例,足趾骨4例.原发性软骨肉瘤2例,均发生于指骨,年龄分别为52岁、76岁,男女各1例.对上述病例的临床影像学资料进行分析,病理学特征通过石蜡切片HE染色进行观察.结果 手足短管状骨的内生软骨瘤临床多无症状,影像学呈现膨胀性的骨破坏,组织学表现为分化较好的软骨组织伴不同程度黏液变、钙化及细胞轻度非典型性,未见浸润骨及软组织.软骨肉瘤临床多伴有疼痛,影像学可见皮质破坏、软组织包块形成.2例病理组织学分级均为II级,其肿瘤细胞较内生软骨瘤更为丰富,非典型性及黏液变显著,可见肿瘤性软骨浸润宿主骨、侵破皮质进入软组织.结论手足短管状骨以内生软骨瘤多见,尽管组织学具有非典型特征,但其呈现＂软骨岛＂结构及为宿主骨小梁包绕的生长方式与软骨肉瘤的浸润性生长不同,手足短管状骨软骨肉瘤罕见,其组织学显示显著的恶性特征,二者诊断时应结合临床、影像学特征及病理学特点进行综合分析.     

Immunohistochemical distribution of S-100 protein in tumors and tumor-like lesions of bone and cartilage

Various tumors and tumor-like lesions of bone and cartilage were examined for S-100 protein using the avidine-biotin-peroxidase complex (ABC) immunostaining method. The most intense reactivity for S-100 protein was found in well-differentiated chondrocytes of enchondromas, osteochondromas and chondrosarcomas, and in normal epiphyseal cartilage. S-100 protein was positive in both polygonal stromal cells and chondrocytes of chondroblastomas and in chondrocytes of mesenchymal chondrosarcoma. In osteosarcomas not only chondroblastic areas but also osteoblastic areas showed positive cells. Reticulum histiocytic cells of eosinophilic granulomas and chordoma cells were positive for S-100 protein. The study yielded three main conclusions: (1) S-100 protein could be the marker for tumors of cartilaginous origin and differentiation, notochord origin, and T-zone histiocyte origin; (2) chondroblastoma can be distinguished from other histologically confusing giant cell lesions by using ABC to detect S-100 protein; and (3) S-100 protein has some relationship with tumoral calcification not only in cartilaginous tumors but also in osteosarcoma.     

S-100 protein in tumors of cartilage and bone. An immunohistochemical study

Using the peroxidase-antiperoxidase (PAP) immunohistochemical method, S-100 protein was found in well-differentiated chondrocytes of chondroma, chondroblastoma, mesenchymal chondrosarcoma, and osteosarcoma. It was not detected in osteoma, osteoblastoma, giant cell tumor, and Ewing's tumor. The presence of S-100 protein in tumorous chondrocytes and chondroblasts suggests that this protein may be a marker of chondrocyte origin and should not be considered a specific marker for nerve tissue.     

CLEAR CELL CHONDROSARCOMA OF BONE (A REPORT OF THREE CASES WITH IMMUNOHISTOCHEMICAL AND AFFINITY HISTOCHEMICAL OBSERVATIONS)

Clear cell chondrosarcoma, a subtype and separate entity from the traditional chondrosarcoma, is characterized by its special histologic features, site of predilection, slow growth and better prognosis. Three cases are presented with elucidation of clinicopathologic correlation and detection by the ABC immunohistochemical method using several antibodies. The observation of positive reaction to S-100 pretein (S-100), vimentin(Vim), alpha 1-antichymotrypsin (AACT) and lysozyme (Lyso) by the tumor cells of clear cell chondrosarcoma, similar to traditional chondrosarcoma and chondroblastoma, proves that this tumor has its origin from the cartilaginous tissue. It was found for the first time that the clear cell chondrosarcoma was positive for wheat germ agglutinin (WGA) and concanavalin A (Con A). The authors believe that clear cell chondrosarcoma may result from the anaplastic changes of chondroblastoma cells Into another subtype of that tumor. The osteoclast-like multinucleated giant cells (MGC), retaining     

Clear cell chondrosarcoma of bone

Clear cell chondrosarcoma, a subtype and separate entity from the traditional chondrosarcoma, is characterized by its special histologic features, site of predilection, slow growth and better prognosis. Three cases are presented with elucidation of clinicopathologic correlation and detection by the ABC immunohistochemical method using several antibodies. The observation of positive reaction to S-100 pretein (S- 100), vimentin (Vim), alpha 1-antichymotrypsin (AACT) and lysozyme (Lyso) by the tumor cells of clear cell chondrosarcoma, similar to traditional chondrosarcoma and chondroblastoma. proves that this tumor has its origin from the cartilaginous tissue. It was found for the first time that the clear cell chondrosarcoma was positive for wheat germ agglutinin (WGA) and concanavalin A (Con A). The authors believe that clear cell chondrosarcoma may result from the anaplastic changes of chondroblastoma cells into another subtype of that tumor. The osteoclast- like multinucleated giant cells (MGC), retaining the antigens of phagocytes, are not considered to be neoplastic.     

Effect of aromatic retinoids on rat chondrosarcoma glycosaminoglycan biosynthesis.

Synthetic aromatic analogs of retinoic acid were administered i.p. and p.o. to Fischer F344 rats bearing a transplantable chondrosarcoma. 35CO4 incorporation into glycosaminoglycans were compared for neoplastic and normal cartilage explants after removal from animals given various analogs. There was a direct relationship between [35S]glycosaminoglycan synthesis by chondrosarcoma chondrocytes and inhibition of tumor growth. The degree of inhibition of [35S]glycosaminoglycan synthesis in the neoplastic cartilage was dependent on the dose of the retinoid administered. At 20-mg/kg/day doses of retinoid for 4 weeks, 35SO4 incorporated into glycosaminoglycan by treated tumor explants was reduced as much as 95%. There was no reduction of [35S] glycosaminoglycan produced in normal costal cartilage of the same animals. Retinoid treatment of 20-mg/kg/day doses for 4 weeks resulted in a 75% reduction in glycosaminoglycan per mg of chondrosarcoma; there was no reduction in costal cartilage glycosaminoglycan. Retinoid (10- to 20-mg/kg/day doses) elevated collagen levels per mg of chondrosarcoma but had no effect on costal cartilage collagen. Combined in vitro and in vivo studies showed that retinoid administration modified neoplastic chondrocyte function but had no measurable effect on normal chondrocyte function.     

Immunohistochemical study on clear cell chondrosarcoma]

Clear cell chondrosarcoma, a subtype and separate entity from the conventional chondrosarcoma, is characterized by its special histologic features, site of predilection, slow growth and better prognosis. Three cases are presented with elucidation of clinicopathologic correlation and detection by ABC immunohistochemical method using several antibodies. The observation of positive reaction to S-100 protein, vimentin, anti-alpha-chymotrypsin and Lysozyme by the tumor cells of clear cell chondrosarcoma, similar to chondrosarcoma and chondroblastoma, proves that this tumor has its origin from the cartilaginous tissue. It was found for the first time that the clear cell chondrosarcoma was positive for wheat germ agglutinin and concanavalin A. The authors believe that clear cell chondrosarcoma may result from the anaplastic change of chondroblastoma cells into another subtype of that tumor. The osteoblastlike multinucleated giant cells, retaining the antigens of phagocytes, are not considered to be neoplastic.     

Phenotypic characterization of chondrosarcoma-derived cell lines

Gene expression profiling of three chondrosarcoma derived cell lines (AD, SM, 105KC) showed an increased proliferative activity and a reduced expression of chondrocytic-typical matrix products compared to primary chondrocytes. The incapability to maintain an adequate matrix synthesis as well as a notable proliferative activity at the same time is comparable to neoplastic chondrosarcoma cells in vivo which cease largely cartilage matrix formation as soon as their proliferative activity increases. Thus, the investigated cell lines are of limited value as substitute of primary chondrocytes but might have a much higher potential to investigate the behavior of neoplastic chondrocytes, i.e. chondrosarcoma biology.     

Immunohistochemical study of the histogenesis of esophageal carcinosarcoma.

In order to clarify the histogenesis and the direction of differentiation of spindle-cell and sarcomatous components of esophageal carcinosarcoma, 20 cases of the disease were reviewed histologically and immunohistochemically using the avidin-biotin-peroxidase complex method with monoclonal and polyclonal antibodies to various keratins, vimentin, desmin, muscle specific actin and S-100 protein. A gradual transition between carcinomatous and spindle cell sarcomatous components was present histologically in all 20 cases. Positive immunoreactivity for keratins was found in carcinomatous areas in all cases. Spindle cells in the transitional areas were positive for keratins in nine cases and for vimentin in five. Two cases demonstrated trace positive reactions to both keratin and vimentin in the same areas of transitional spindle cells between carcinomatous and sarcomatous components. The sarcomatous component showed an immunohistochemically positive reaction for vimentin in ten cases and for desmin in two. In one of the 20 cases, chondrosarcomatous cells were seen which showed a positive reaction to S-100 protein but were negative to keratin. The findings strongly suggested that neoplastic epithelial cells may show dedifferentiation to transforming spindle cells and also disdifferentiation to non-epithelial sarcoma like chondrosarcoma and leiomyosarcoma.