骨巨细胞瘤84例临床病理分析

报道84例骨巨细胞瘤(GCT),20～39岁患者占75%,95.2%位于长骨,其中膝关节附近占64.3%。X线表现为长骨骺端偏心位溶骨性病变。63例随访1～16年,33例复发(52.4%)。按照Jaffe病理分级,Ⅰ、Ⅱ、Ⅰ～Ⅱ和Ⅲ级的复发率各为66.7%、40%、44.4%和50%。Ⅰ～Ⅱ级和Ⅲ级肺转移各为2例和1例。结果表明分级与预后无明确关系。     

骨的恶性巨细胞瘤临床病理观察

目的 探讨骨的恶性巨细胞瘤临床病理学特征及其诊断和鉴别诊断.方法 对13例恶性巨细胞瘤的临床及病理学资料进行回顾性分析.结果 13例恶性巨细胞瘤中原发性恶性巨细胞瘤6例,发病年龄21-71岁,平均年龄39.5岁.复发后继发性恶性巨细胞瘤7例,发病年龄27-52岁,平均年龄36.7岁.6例原发性恶性巨细胞瘤除可见到骨巨细胞瘤区域外,还可见到高度恶性的梭形细胞肉瘤区域,7例继发性恶性巨细胞瘤其原发性肿瘤均为骨巨细胞瘤,而复发性肿瘤则呈恶性纤维组织细胞瘤/未分化肉瘤形态.结论 诊断恶性巨细胞瘤时需将临床、影像及病理结合,并除外其他肉瘤如富含巨细胞的骨肉瘤、富含巨细胞的恶性纤维组织细胞瘤等.     

骨巨细胞瘤诊断中值得注意的几个问题

骨巨细胞瘤诊断中值得注意的几个问题朱雄增骨巨细胞瘤是一种常见的原发性骨肿瘤，也是骨肿瘤诊断中问题存在最多的一种肿瘤。骨巨细胞瘤在组织发生、生物学行为、临床病理诊断和治疗等诸方面都有较大争议［１］。近年来尽管在某些方面已取得较一致的意见，但在一些研究中...     

右胫骨骨巨细胞瘤1例

临床资料 患者,男,35岁,无明显诱因出现右小腿部位不适一周,走平路时表现明显。门诊查体：右胫骨胫前畸形,骨面不光滑,约3cm,表面皮温较高,压痛（＋）,无红肿。初步诊断为右胫骨骨髓炎。X线提示：右胫骨纤维异常增生,骨膜无异常,右膝关节无异常。行ECT检查,全身骨显像示：右股骨上段可见局部轻度浓聚,右股骨下端及右胫骨可见异常浓聚,     

AgNOR在骨巨细胞瘤分级及预后判定中的价值

对４２例骨巨细胞瘤（ＧＣＴ）患者进行ＡｇＮＯＲ计数，其中Ｉ级１８例，Ⅱ级１４例，Ⅲ级１０例，结果发现ＡｇＮＯＲ在Ｉ级与Ⅱ级瘤间差异无显著性（Ｐ〉０．０５）而在Ⅱ级与Ⅲ级瘤间差异有高度显著性（Ｐ〈０．０１）。提示ＡｇＮＯＲ的表达与Ｊａｆｆｅ分级不太一致，因而，我们倾向于将骨ＧＣＴ只分为良，恶性两肿，而且ＡｇＮＯＲ的高低与骨ＧＣＴ的预后有关。     

骨巨细胞瘤TIMP-3启动子甲基化的研究

目的 检测骨巨细胞瘤(GCT)中TIMP—3启动子甲基化及其蛋白表达，探讨该肿瘤组织TIMP—3蛋白表达缺失的原因和TIMP-3启动子甲基化与GCT分级和复发的关系。方法 用免疫组织化学SP法和蛋白免疫印迹检测TIMP—3在GCT组织中的表达，用甲基化特异的PCR(MSP)法检测TIMP—3基因启动子的甲基化状态。结果 TIMP—3主要在单核基质细胞和多核巨细胞的胞质表达，后者的表达具有明显的极向性。17例GCT中有5例(29．4％)TIMP—3蛋白丢失，其中4例的TIMP—3启动子发生异常甲基化，且均为组织学分级为Ⅱ级的病例。结论 GCT的局部骨质破坏，可能与TIMP—3丢失有关；而TIMP—3启动子的异常甲基化，是TIMP—3基因失活和蛋白丢失的重要机制。     

骨巨细胞瘤中PTEN基因与PCNA的表达

目的 探讨PTEN与骨巨细胞瘤中破骨样多核巨细胞的关系。方法 采用免疫组化染色方法检测27例骨巨细胞瘤PTEN蛋白与PCNA的表达、PCR检测骨巨细胞瘤PTEN基因的外显子。结果 77．8％(21／27例)骨巨细胞瘤中仅破骨样多核巨细胞PTEN蛋白呈强表达，PCNA不表达；而间质细胞基本不表达PTEN蛋白，但具有不同比例的PCNA阳性细胞。在所分析的8例骨巨细胞瘤中，PCR均检测到PTEN基因的外显子。结论 PTEN在GCT破骨样多核巨细胞的过表达可能是骨巨细胞瘤形成原因之一。     

骨巨细胞瘤生物学行为的相关因素分析

目的 探讨骨巨细胞瘤的组织发生以及与肿瘤复发、侵袭、恶变等生物学行为相关的因素.方法 从临床、影像学、手术方式、病理形态、免疫表达和随访结果等方面对2005至2011年间收集的123例骨巨细胞瘤进行分析和研究.结果 骨巨细胞瘤的术后复发率与术前骨巨细胞瘤的影像学分级(P=0.032)、CD147 (P=0.034)和p53(P =0.005)的过表达以及手术方式(P =0.048)有明显相关性,但与骨巨细胞瘤的髓内浸润、骨皮质浸润、骨旁软组织浸润、脉管内瘤栓、单核间质细胞的梭形变、核分裂象多少、Ki-67阳性指数、凝固性坏死、继发性动脉瘤样骨囊肿、出现反应性骨等形态学改变均缺乏明显相关性,骨巨细胞瘤中单核间质细胞p63阳性率(79.7％,94/118)明显高于软骨母细胞瘤(44.7％,21/47)、骨肉瘤(22.2％,10/45)等其他富于巨细胞的骨肿瘤.结论 骨巨细胞瘤是一种有低度恶性潜能的肿瘤,形态学特征难以预测其生物学行为.p63阳性、PGM-1阴性的单核间质细胞是其肿瘤成分,此种细胞p53的过表达以及骨巨细胞瘤各种细胞成分(包括反应性单核巨噬细胞和多核巨细胞)CD147的过表达与骨巨细胞瘤的侵袭、复发和恶变有关.术前根据骨巨细胞瘤的影像学分级选择合适的手术方式是减少复发最重要的因素.     

骨巨细胞瘤外科治疗的随访

目的探索骨巨细胞瘤术前X线表现、病理分级与预后的关系,寻求最有效的治疗方法。方法对资料完整的60例骨巨细胞瘤的X线、手术方法、病理结果进行回顾性分析。结果骨巨细胞瘤的X线分度在一定程度上反映病理分级及预后,以瘤段骨切除体外灭活再植术疗效最佳,但手术复杂。结论术前可根据骨巨细胞瘤的X线分度或结合病理活检结果选择合适的手术方法。     

Osteoclast Origin of Giant Cells in Giant Cell Tumors of Bone: Ultrastructural and Cytochemical Study of Six Cases

To clarify the histogenesis of giant cells appearing in giant cell tumors of bone (GCTB), an ultrastructural and cytochemical study of six cases was performed with both acid phosphatase (ACPase) and tartrate-resistant acid phosphatase (TRACPase) as marker enzymes. TRACPase is considered a specific marker for osteoclasts. ACPase was demonstrated in the macrophagelike stromal cells, the multinucleated giant cells, and the infiltrating macrophages. The enzyme reaction was localized in lysosomal dense bodies and Golgi areas. Intense TRACPase activity was demonstrated in the multinucleated giant cells, whereas a weak reaction was found in the macrophagelike stromal cells. The multinucleated giant cells and macrophagelike stromal cells resembled osteoclasts with regard to the subcellular localization of TRACPase. The present results suggest that the giant cells in GCTB are indeed derived from osteoclasts.     

Osteoclast Origin of Giant Cells in Giant Cell Tumors of Bone: Ultrastructural and Cytochemical Study of Six Cases

To clarify the histogenesis of giant cells appearing in giant cell tumors of bone (GCTB), an ultrastructural and cytochemical study of six cases was performed with both acid phosphatase (ACPase) and tartrate-resistant acid phosphatase (TRACPase) as marker enzymes. TRACPase is considered a specific marker for osteoclasts. ACPase was demonstrated in the macrophagelike stromal cells, the multinucleated giant cells, and the infiltrating macrophages. The enzyme reaction was localized in lysosomal dense bodies and Golgi areas. Intense TRACPase activity was demonstrated in the multinucleated giant cells, whereas a weak reaction was found in the macrophagelike stromal cells. The multinucleated giant cells and macrophagelike stromal cells resembled osteoclasts with regard to the subcellular localization of TRACPase. The present results suggest that the giant cells in GCTB are indeed derived from osteoclasts.     

An Unusual Form of Endoplasmic Reticulum in Mononuclear Cells of a Giant Cell Tumor of Bone

A peculiar configuration of endoplasmic reticulum (ER) was observed by electron microscopy in some mononuclear cells from a malignant giant cell tumor of bone that had been removed from a cervical vertebral body in a 54-year-old female. The unusual ER consisted of looped, smooth tubules, which originated from poorly developed rough ER present in the tumor cells. The tubular structures were approximately 23 ran in diameter and contained paracrystalline material. The curvilinear tubular ER closely resembles the vermiform tubular structures that have been observed mainly in histiocytes from different clinical forms of histiocytosis X.     

右美托咪定对骨巨细胞瘤细胞增殖、侵袭及迁移的影响

目的探究右美托咪定(dexmedetomidine,DEX)对骨巨细胞瘤细胞增殖、侵袭及迁移的影响.方法将细胞分Control、DEX 0.01、0.1及1μmol/L组.BrdU检测细胞增殖,transwell检测细胞侵袭,划痕实验检测细胞迁移,免疫印迹检测Ki67、PCNA、Bcl-2、VEGF、MMP-2、MMP-9、NF-κB p65及其下游蛋白表达水平、p38 MAPK表达及其下游蛋白磷酸化比值.结果与Control组比较,DEX 0.1、1μmol/L组BrdU阳性细胞百分比、侵袭细胞数、划痕闭合率和Ki67、PCNA、Bcl-2、VEGF、MMP-2、MMP-9表达水平显著降低,抑制p38 MAPK、NF-κB信号通路的激活.结论DEX通过调控p38 MAPK及NF-κB表达抑制骨巨细胞瘤细胞增殖、侵袭及迁移.     

Multinucleated Giant Stromal Tumor of the Omentum: Report of a Case with Immunohistochemical and Ultrastructural Investigation

Multinucleated giant stromal cells (MGSC) have been described in a variety of lesions of various anatomical sites. They are generally believed to be derived from fibroblasts or myofibroblasts. Their size and bizarre appearance may lead to an erroneous interpretation of infiltrating malignant cells, but they are regarded as reactive in nature. MGSC also seem to participate in a neoplastic process and form a part of tumors called giant cell fibroblastomas (GCF). In GCF, multinucleated giant cells are sparsely scattered throughout the tumor, which is composed of loosely arranged spindle cells. Thus far, no tumor composed of MGSC entirely, to the best of the authors' knowledge, has been reported. This study involved an 80-year-old female with an omental tumor, which is believed to represent the first case of tumor of MGSC. The patient developed abdominal pain; a large abdominal tumor measuring 18 × 15 × 5 cm by computerized tomography was found located between the left lobe of the liver, the transverse colon, and the greater curvature of the stomach. Although the tumor was adherent to the above organs and infiltrating the omentum, it was resectable. Grossly, the tumor was highly vascular and the surface was shaggy with no recognizable capsule. The cut surfaces were red to tan with frequent cystic spaces containing bloody material. Microscopically, the tumor cells were large and multinucleated (2-6 nuclei) with prominent nucleoli. The cytoplasm was abundant and stained amphophilic. These tumor cells formed moderately cellular sheets filling the spaces between the varying sized vessels. There was prominent vascularity throughout the tumor. DNA study by image analysis revealed aneuploidy peaks. On immunohistochemis-try, the tumor cells were strongly positive for vimentin, moderately positive for actin along the periphery of the cytoplasm, and negative for cytokeratin, EMA, myoglobin, S-100, CEA, Factor Xllla, HMB-45, and HAM56 and KP-1. Ultrastructurally, the cytoplasm contained rich profiles of RER with scattered lysosomes. The cell borders were slightly irregular with occasional subplasmalemmal densities facing loosely arranged collagenous stroma. The light microscopic, immunohistochemical, and electron microscopic features of tumor cells were remarkably similar to MGSC. The tumor size and gross appearance suggested a malignancy, but it was a diploid tumor and the patient remains disease free 5 years after a complete resection.     

A Small Aneurysmal Bone Cyst Restricted to the Cortical Bone of the Femur Resembling So-called Subperiosteal Giant Cell Tumor or Subperiosteal Osteoclasia

We report a 16-year-old Japanese girl with a cystic lesion restricted to the cortical bone under the periosteum of the diaphysis of the left femur. Roentgenograms showed a long, oval translucent lesion in frontal view and an eccentric erosive lesion in lateral view. Computed tomography showed a distinct intracortical lesion. The lesion, which was excised en bloc , measured 3×2×2 cm. The outer layer of the cortical bone was eroded eccentrically. From the margin of the eroded bone, thin fragile bony tissue and preserved periosteum extended like the roof of a dome. Multicystic structures, filled with blood, were lined with fibrous granulation and occasional giant cells. Histologically, this lesion falls within the category of aneurysmal bone cyst. However this case is of a rare type, since the lesion was relatively small, and showed a very specific intracortical location, in marked contrast to typical aneurysmal bone cyst. Additionally, this lesion is similar to so-called subperiosteal giant cell tumor or subperiosteal osteoclasia described in the literature. Acta Pathol. Jpn. 39: 539∼544, 1989.     

Ovarian Sertoli-Leydig Cell Tumor with Rhabdomyosarcoma: An Ultrastructural Study

The clinical and light and electron microscopic findings of a moderately differentiated, virilizing, Sertoli-Leydig cell tumor (SLT) with pleomorphic rhabdomyosarcoma of the ovary are presented. The tumor recapitulates the primitive embryonal testis and rhab-domyogenesis, respectively. The natural history, including pathogenesis, of this peculiar and rare tumor is discussed in the light of the pertinent literature on SLT and ovarian rhabdomyosarcoma. It seems that when rhabdomyosarcoma is a significant or predominant component of SLT, as occurred in this case, the prognosis is poor and is that of rhabdomyosarcoma in general.     

Ovarian Sertoli-Leydig Cell Tumor with Rhabdomyosarcoma: An Ultrastructural Study

The clinical and light and electron microscopic findings of a moderately differentiated, virilizing, Sertoli-Leydig cell tumor (SLT) with pleomorphic rhabdomyosarcoma of the ovary are presented. The tumor recapitulates the primitive embryonal testis and rhab-domyogenesis, respectively. The natural history, including pathogenesis, of this peculiar and rare tumor is discussed in the light of the pertinent literature on SLT and ovarian rhabdomyosarcoma. It seems that when rhabdomyosarcoma is a significant or predominant component of SLT, as occurred in this case, the prognosis is poor and is that of rhabdomyosarcoma in general.     

癌基因C-erbB-2和H-ras在骨巨细胞瘤中的表达

目的　探讨 C- erb B- 2和 H- ras癌基因在骨巨细胞瘤 (GCT)的表达及与 GCT有关临床指标的关系。方法　应用 SP免疫组织化学方法检测 C- erb B- 2和 H- ras在 5 2例 GCT(GCT按 Jaffe分级 : 级 15例、 级 2 5例、 级 12例 )中的表达。结果　 11例 C- erb B- 2表达阳性 ,阳性率 2 1.2 % ,7例 H- ras表达呈阳性 ,阳性率 13.5 %。其阳性表达与病理分级均无显著性差异 (P >0 .0 5 )。 C- erb B- 2和 H- ras在复发和无复发的病例中 ,阳性表达率分别为 38.5 %、15 .4%和 2 3.1%、10 .3% ,两者同时阳性表达的有 2例。C- erb B- 2和 H- ras的阳性表达与 GCT复发无显著性差异。结论 C- erb B- 2和 H- ras在 GCT中的表达与病理分级和复发无关