骨化性纤维瘤及纤维结构不良临床病理比较分析

收集骨化性纤维瘤20例和纤维结构不良48例,就其临床病理表现进行比较分析,探讨二者的关系。二者临床及X线表现相似,骨化性纤维瘤有局部侵裂行为,复发率较纤维结构不良高;组织学上,二者在一定程度有形态交叉。通过对比分析作者支持二者均为纤维—骨病变的论点。     

长骨骨纤维结构不良型釉质上皮瘤临床病理分析

目的探讨长骨骨纤维结构不良型釉质上皮瘤的临床病理特点、鉴别诊断及生物学特征。方法对2例长骨骨纤维结构不良型釉质上皮瘤进行病理学、免疫组织化学观察,结合临床资料进行分析并复习相关文献。结果2例长骨骨纤维结构不良型釉质上皮瘤患者均为年轻人,临床以小腿疼痛或无痛性肿胀为主,病程较长,影像学均示胫骨骨干病变。光镜下肿瘤主要由梭形细胞和散在的新生编织骨组成,梭形细胞密度较高,呈束状或编织状排列,纤维组织间见少数巢团状或裂隙状的上皮样细胞巢,梭形细胞和上皮样细胞均无明显异型性。免疫表型:上皮细胞巢表达CKpan、CK14和CK19,但不表达CK8、CK18。EGFR阳性表达主要见于上皮样细胞,Ki-67在上皮样细胞和梭形细胞均有少量散在阳性表达。结论长骨骨纤维不良型釉质上皮瘤是一种罕见的好发于年轻人胫骨骨干的低度恶性肿瘤,主要根据影像学、光镜及免疫组化确诊,应与骨纤维结构不良、转移癌、滑膜肉瘤等相鉴别。     

骨纤维结构不良临床病理分析

目的 探讨骨纤维结构不良的临床病理特点,提高对该疾病的认识.方法 观察6例骨纤维结构不良的临床特点及病理形态学特征,并进行组化和免疫组化染色.结果 该肿瘤特征性地累及婴儿和儿童的胫骨中段前面的皮质,影像学上病变境界清楚,骨皮质变薄、膨胀甚至消失,边缘有硬化带,组织学上骨纤维结构不良有不规则的编织骨碎片,其边缘常衬覆着板层骨,板层骨由轮廓分明的骨母细胞产生.免疫组化染色显示6例中3例有梭形细胞CK散在阳性,未见CK阳性的上皮细胞巢,S-100阴性.骨纤维结构不良的自然病程是在10岁以前逐渐生长,到15岁左右逐渐自行消退并康复.10岁以下术后复发率高.结论骨纤维结构不良有特征性的病理改变及临床表现,应注意与纤维结构不良等病变鉴别.     

纤维瘤病的临床病理和免疫组化分析

目的：探讨纤维瘤病的组织发生和临床病理特征。方法：对１６例手术切除的纤维病标本进行了光镜观察和免疫组织化学ＬＳＡＢ法染色，结果；腹壁及腹壁外的纤维瘤病表达ＡＳＭＡ和Ｓ－１００蛋白，Ｓ－１００蛋白阳性细胞呈散在分布，复发的２例ＰＣＮＡ呈弱阳性，肿瘤的主要成分为肌纤维母细胞，可以见到Ｓ－１００阳性表达。结论：纤维瘤病率来源皇多潜能的间叶细胞。     

四肢骨骨化性纤维瘤7例病理分析与鉴别诊断

本文报道四肢骨骨化性纤维瘤7例,男性4例,女性3例,年龄10～46岁,平均20岁。主要临床表现为局部肿块。镜下所见:肿瘤由肿瘤性纤维结缔组织和骨小梁组成,典型的骨小梁周边部呈平行的板层结构,中央呈不太清晰的编织状结构,骨小梁外周有密集的单行排列的骨母细胞和少数破骨细胞,所有病例均无出血、炎症和死骨片。本瘤应注意与非骨化性纤维瘤、骨纤维异常增殖症、骨囊肿和骨巨细胞瘤鉴别。     

儿童非骨化性纤维瘤X线-组织病理学

目的提高对儿童非骨化性纤维瘤的临床病理学认识,以能及时诊治. 方法分析了5例儿童非骨化性纤维瘤临床特点.诊疗途径和组织学特征. 结果 5例均发生于长骨,手术病理证实为非骨化性纤维瘤.随访最长5年7月,无一例复发. 结论本病发病率极低,易误诊,X线及病理均有特征性改变,手术预后良好.应与纤维性皮质骨缺损、骨囊肿等相鉴别.     

长骨骨化性纤维瘤的DR及CT诊断

骨化性纤维瘤又称为纤维骨瘤,该肿瘤好发于颌骨和颅骨,但近年来长骨发病也时有报道。本文收集经手术及病理证实的有CR和CT资料的骨化性纤维瘤18例,回顾性分析其影像学特征,探讨影像表现与病理基础之间的关系。     

儿童非骨化性纤维瘤X线——组织病理学

目的：提高对儿童非骨化性纤维瘤的临床病理学认识，以能及时诊治。方法：分析了5例儿童非骨化性纤维瘤临床特点。诊疗途径和组织学特征。结果：5例均发生于长骨，手术病理证实非骨化性纤维瘤。随访最长5年7月，无一例复发。结论：本病发病率极低，易误诊，X线及病理均有特征性改变，手术预后良好。应与纤维性皮质骨缺损、骨囊肿等相 鉴别。     

49例纤维结构不良的病理及免疫组化观察

纤维结构不良是发生于骨内的最常见的瘤样病变之一 ,临床上有较高的术后复发率。收集 4 9例纤维结构不良标本进行病理分析及免疫组化标记 ,以探讨复发病例的病理形态特点及其与ＨＳＰ70、ＥＲ、Ⅰ、Ⅲ型胶原的关系。1　材料与方法1.1　临床资料　收集西京医院病理科 4 9例纤维结构不良的档案病例 ,年龄最小 1岁 4个月 ,最大 4 2岁 ,14岁以下 2 2例 ,治疗后复发病例 12例。复发病例中 14岁以下 11例。所有标本均经 10 %福尔马林液固定、常规石蜡包埋、分别进行切片 ,切片厚 5 μｍ ,ＨＥ及免疫组化染色。1.2　方法与试剂　免疫组化采用ＳＡＢ…     

Fibro-osseous lesions of the head and neck

This review aims to overview key histopathological features and provide diagnostic clues for a selection of the fibro-osseous lesions of the jaws and facial bones. These form a diverse group of conditions with differing aetiologies but similar histological appearances. Some may be part of more generalized systemic disease. It will become apparent that for this group of conditions, diagnosis cannot be made by examination of biopsy material by a pathologist in isolation, and both clinical and radiological correlation is needed. However, there are some subtle histopathological features that can allow one diagnosis to be favoured over all others, and these will be outlined. Identification of these features in material sent for histopathological analysis should assist the multidisciplinary team in making a definitive diagnosis.     

Ultrastructure of Fibrous Dysplasia of Bone: A Study of Its Fibrous, Osseous, and Cartilaginous Components

Eight cases of fibrous dysplasia were evaluated by electron microscopy and the findings were correlated with the light microscopy. Fibroblasts and myofibroblasts were the cells seen in the fibrous component. The osseous component consisted of immature woven bone trabeculae lined by abnormal osteoblasts with a fibroblastlike appearance. The cartilaginous component resembled hyaline cartilage. The process of mineralization of both osseous and cartilaginous tissues appeared similar to normal bone and cartilage. In fibrous dysplasia there is a slow turnover of bone, and this correlates well with the flattened shape of the bone-lining cells. When compared with normal ossification, the process of bone formation appears to be arrested after an early stage resembling membranous ossification. It is possible that the abnormal osteoblastic maturation of the bone-forming mesenchyme is one of the most important alterations of this condition.     

Confocal Laser Scanning Microscopy Analysis of 10 Cases of Craniofacial Fibrous Dysplasia

Fibro-osseous lesions (FOL) represent a heterogeneous group of lesions that exhibit a variety of clinic-pathological features. Recently, based on the new World Health Organization classification system, these lesions were reclassified as follows: (1) fibrous dysplasia (FD), (2) osseous dysplasia, and (3) ossifying fibroma. Nevertheless, the nosologic placement of FOL may be problematic because of substantial overlap in the histopathological findings. In this study, we analyzed 10 cases of FD by both optical and confocal laser scanning microscopy, a research technique based on the laser light microscopic analysis of stained biological samples that allows improved tissue imaging and bidimensional pictures with high resolution at the cellular level to provide a better understanding of the diagnosis of this disease.     

Ossifying fibromyxoid tumor of the retroperitoneum

A case of ossifying fibromyxoid tumor (OFMT) in an unusual site, the retroperitoneum, in a 71 year old Japanese man is reported. The well demarcated tumor contained a large amount of mature bony tissue within the tumor. The tumor cells were round to spindle-shaped, and showed a high cellularity in some areas and were loosely arranged in other areas. Although the majority of tumor cells expressed vimentin, neuron specific enolase and Leu-7, there were no tumor cells positive for S-100 protein, myogenic or epithelial markers. We would like to emphasize that tumors classified as OFMT contain a wide spectrum of histologic features and heterogeneity.     

Ossifying subcutaneous tumor with myofibroblastic differentiation

Immunohistochemical, ultrastructural, and flow cytometric studies were performed on an ossifying soft-tissue tumor, presumed to be a variant of ossifying fibromyxoid tumor of soft parts, which was located in the subcutis of the left buttock of a 76 year old Japanese woman. Histologically, this was a benign-looking spindle, oval, or round cell lesion, having a fibrous capsule with a discontinuous rim of bone as seen in typical cases. However, this lesion was also characterized by a high degree of cellular proliferation in storiform and whorl arrangements, extensive ossification, osteoid and metaplastic bone formation and absence of myxoid features. In an immunohistochemical study using formalin-fixed, paraffinembedded sections, many tumor cells expressed vimentin, S-100 protein, Leu-7, neuron specific enolase, and desmin. Ultrastructuraliy, this neoplasm consisted of fibroblast-like cells and myofibroblast-like cells. This tumor had an aneuploid DNA content. No recurrence has been observed for 16 months. These results suggest that the neoplastic cells may show the phenotypic expressions of myofibroblast and also osteogenic differentiation.     

Histopathology of fibro-osseous and cystic tumors of bone

Fibro-osseous and cystic tumors are some of the more common tumors encountered in bone pathology. Although there is significant histologic overlap, fibro-osseous tumors of bone have distinct clinical, radiologic and pathogenetic features. Similarly, cystic lesions have characteristic radiologic and pathologic features that help distinguish them from tumor of bone that can have secondarily cystic components. More recently, specific genetic abnormalities have been identified in a subset of fibro-osseous and cystic tumors, such as fibrous dysplasia and aneurysmal bone cyst. Herein we discuss the clinical, radiologic, pathologic and genetic features of fibro-osseous and cystic tumors of bone.     

Ossifying Fibromyxoid Tumor: Invariable Ultrastructural Features and Diverse Immunophenotypic Expression

Ossifying fibromyxoid tumor (OFMT) is a rare enigmatic soft tissue tumor, the origin of which is still uncertain. The authors report on 3 cases of OFMT arising in the trunk and head and neck regions of adults. Two recurred and one was suspected to have metastasis. All tumors consisted of multiple nodules, in which round or polygonal tumor cells were arranged in sheets or cords within a fibromyxoid background. Characteristic shell-like bone tissues were recognized in all tumors. Based on the grading system proposed by Folpe et al., 2 cases were designated as malignant OFMT and 1 as typical. In addition to S-100 protein, cytokeratin and neuronal markers (neurofilament, CD56 or CD57) were detected in 1 and 2 tumors, respectively. The salient and invariable ultrastructural features included reduplicated basal laminas, which seem to be crucial for the diagnosis.     

Ossifying fibromyxoid tumor: invariable ultrastructural features and diverse immunophenotypic expression

Ossifying fibromyxoid tumor (OFMT) is a rare enigmatic soft tissue tumor, the origin of which is still uncertain. The authors report on 3 cases of OFMT arising in the trunk and head and neck regions of adults. Two recurred and one was suspected to have metastasis. All tumors consisted of multiple nodules, in which round or polygonal tumor cells were arranged in sheets or cords within a fibromyxoid background. Characteristic shell-like bone tissues were recognized in all tumors. Based on the grading system proposed by Folpe et al., 2 cases were designated as malignant OFMT and 1 as typical. In addition to S-100 protein, cytokeratin and neuronal markers (neurofilament, CD56 or CD57) were detected in 1 and 2 tumors, respectively. The salient and invariable ultrastructural features included reduplicated basal laminas, which seem to be crucial for the diagnosis.     

Benign fibro-osseous tumors of bone: clinicopathological findings and differential diagnosis

Benign fibro-osseous tumors of the skeleton are a diverse group of neoplasms with overlapping histologic features. They are characterized by an admixture of fibroblast-like spindle cells enmeshed in a collagenous stroma admixed with varying quantities of bone that can be deposited in different configurations. Some fibro-osseous tumors are predisposed to develop in specific bones, while others can arise in any bone; they may be solitary or multicentric. The treatment is based on their biological behavior and ranges from observation and medical therapy to surgical removal. Prognosis depends on the extent of the disease and the biological potential of the neoplasm. Because of overlapping clinicopathological features distinguishing one fibro-osseous tumor from another can be difficult and requires correlation with the imaging and clinical findings. This review discusses the features of benign fibro-osseous tumors of the skeleton and their important differential diagnoses.