骨原发恶性纤维组织细胞瘤（附15例报告）

骨恶性纤维组织细胞瘤是一种罕见的肿瘤，春特点是兼有组织细胞性和纤维性成分，男性发病多于女性，无明显好发年龄，以中老年稍多。病损主要位于和骨骨端，红占全部病例的７５％。病程较长，疼痛和逐渐长大的肿块是国早出现的临床症状。Ｘ线表现溶骨性病变。诊断时要与骨纤维肉瘤鉴别．单纯手术治疗效果不满意，应辅以化疗或放疗。早期病例可在有效化疗放疗控制下，行广泛病灶切除，保留肢体预后比骨肉瘤、骨纤维肉瘤好。     

含软骨成份骨肿瘤的X线诊断

X线诊断在骨肿瘤的诊断中有它独特的长处，它可以观察到肿瘤的全貌，避免病理取材的局限，骨肿瘤内含软骨细胞成份则表现为成簇分布的斑点状或环状钙化阴影，仔细观察很具特征性。现就含软骨细胞成份的骨肿瘤种类探讨如下。1．起源于软骨组织的骨肿瘤有：软骨瘤，骨软骨瘤，良性软骨母细胞瘤，软骨粘液纤维瘤、软骨肉瘤。2．起源于骨组织的肿瘤有：成骨肉瘤，骨分骨肉瘤，恶性巨细胞瘤，骨旁骨肉瘤可能含大量软骨细胞成份。3．起源于骨纤维组织的肿瘤有：骨纤维肉瘤部分病例可见到。4骨组织肿瘤样病变：骨纤维异常增殖症，特别是下颌和多骨…     

蹠骨纤维肉瘤误诊骨结核1例报告

骨纤维肉瘤好发于四肢长骨和干骺端或骨干部.手、足骨发病报道甚少,我院于2006-07-23收治1例发生在蹠骨的纤维肉瘤,由于对该病认识不足,误诊为骨结核,现将本病例报告如下.     

骨外尤文氏肉瘤15例临床及病理分析

目的探讨骨外尤文氏肉瘤的临床及病理学特点。方法对15例骨外尤文氏肉瘤患者的临床资料进行回顾性分析。结果15例中,男9例,女6例,发生于腰背部软组织者5例。镜下见小圆细胞,胞浆少,富含糖原,免疫组化CD99阳性。15例均采用手术切除,多数辅以化疗和(或)放疗。7例生存期>5年。结论骨外尤文氏肉瘤好发于青少年,男性多见,好发于躯干深部软组织,病理学特征与原始神经外胚叶瘤有共同之处,治疗上以手术切除,放疗、化疗综合治疗为主。预后较差。     

股骨近端平滑肌肉瘤1例并文献复习

目的探讨骨平滑肌肉瘤的临床表现、诊断和治疗。方法对1例骨原发性平滑肌肉瘤进行临床和病理资料观察。复习有关文献,就骨原发性平滑肌肉瘤发病情况、组织来源、病理、诊断、鉴别诊断、治疗和预后进行了讨论。结果骨原发性平滑肌肉瘤好发于股骨远端和胫骨近端,尤其是膝关节附近。临床最常见症状是疼痛,可伴有关节活动受限。X线显示长骨干骺端不规则低密度影。组织学、免疫组织化学标记表达具有特征。结论骨原发性平滑肌肉瘤比较少见,诊断主要靠免疫组化。宜采取手术治疗,可辅以放疗或化疗。     

骨纤维异常增殖症恶变1例报告并文献复习

骨纤维异常增殖症(简称骨纤)可恶变为骨肉瘤、纤维肉瘤及软骨肉瘤，我们遇见1例，报告于后。     

继发于骨纤维结构不良的未分化多形性肉瘤1例

结合1例继发于桡骨骨纤维结构不良的未分化多形性肉瘤的影像学表现,并进行鉴别诊断。其CT表现为桡骨近段膨胀性骨质破坏,伴周围软组织影,临近骨皮质明显变薄、缺损,MRI表现为病灶信号混杂,增强扫描病变呈不均匀强化,内见纤维分隔。长骨未分化多形性肉瘤影像学特点缺乏特异性,鉴别诊断较困难,通过该病例加深对骨纤维结构不良恶变及未分化多形性肉瘤的认识,以期提高对骨纤维结构不良随访的重视程度。     

综合治疗骨肉瘤保留肢体30例临床观察

目的 观察综合治疗四肢骨肉瘤保肢的临床疗效。方法 对30例四肢骨肉瘤病人，根据病变部位、范围、Enneking分期，分别进行了瘤骨灭活再植、在体灭活、局部扩大切除、骨水泥填充、瘤骨切除后自体骨移植关节重建，以及术前、术后局部化疗及放疗。结果 随访0．5～11年，发现30例病人中感染3例，病理骨折1例，腓总神经损伤2例，局部复发6例；2年无瘤生存24例(80％)，3年无瘤生存20例(66．7％)，5年无瘤生存16例(53．3％)。结论 手术及手术前、后采用局部动脉化疗及局部放疗，是四肢骨肉瘤保肢术的较为理想方法。     

横纹肌肉瘤的治疗（附20例报道）

对２０例经病理证实为横纹肌肉瘤的患者，采用单纯手术、单纯放疗或化疗，放疗＋化疗，手术＋放疗＋化疗等方案。结果表明手术＋放疗＋化疗进行治疗的患者，生存时间最长，同时对横纹肌肉瘤的病因及诊治进行了讨论。     

滑膜肉瘤的现代诊疗分析

目的：分析滑膜肉瘤的现代诊断与治疗方法。方法；对8例滑膜肉瘤病例进行回顾性分析。结果：手术配合化疗、放疗后，远处转移5例，术后复发4例 ，死亡3例。结论：病理诊断具有特异性，染色体检查有特殊意义。手术配合化疗、放疗为有效的治疗手段，几种因素可指导预后。     

骨原发性恶性纤维组织细胞瘤影像分析

目的探讨骨原发性恶性纤维组织细胞瘤的影像学表现,以提高对该病的诊断水平。方法回顾性分析该院收治的资料完整并经病理证实的骨原定性恶性纤维组织细胞瘤10例的影像学表现。结果骨原发性恶性纤维组织细胞瘤好发于昧关节上下股骨和胫骨的干骺端,10例中,发生于股骨远端4例,胫骨近端3例,腓骨近端、骶骨、右侧髂骨各1例。X线和CT影像学表现为形态不一的溶骨性骨质破坏,边界清楚或模糊,无明显或轻微线状骨膜反应,伴软组织肿胀或肿块,肿块较小而局限,可发生病理性骨折。MRI检查边界清,信号混杂,增强扫描呈不均质中等或明显强化。结论骨原发性恶性纤维组织细胞瘤在临床及影像学方面缺乏特征性,应综合分析其临床及影像表现,结合病理检查及免疫组化标记分析,才能对该病作出正确的诊断。     

An Unusual Case of Elastofibroma in the Neck

We report the case of a 45-year old woman with a painless soft tissue mass on the left side of her neck. At surgery, the lesion was solid and rubbery and revealed grey-white fibrous areas. It was pathologically confirmed as an elastofibroma, a rare, benign soft tissue tumour that is most commonly encountered in patients more than 55 years of age. Elastofibroma arising from connective tissue and usually found in the subscapular region is extremely rare in the neck. The pathogenesis of elasto-fibroma is still unclear but increased mechanical stress or nutritional derangement consequent to vascular involvement and hereditary factors may predispose to this lesion. Marginal excision is the treatment of choice in symptomatic patients.     

Fibrous dysplasia on the skull

A young rickshaw puller admitted in Neurosurgery ward of Mymensingh Medical College Hospital due to ugly looking skull swelling on right side and iatrogenic ulceration on the postero-superior surface of the swelling. He also mentioned that he was not socially well-excepted and facing difficulty in getting married for that ugly lesion and was very unhappy at that time. CT scan was suggestive of fibrous dysplasia of skull bone and no other abnormalities were present on other part of the body. Then complete excision of the mass was done and fibrous dysplasia was confirmed by histopathological study. Two months after surgery he got married and expressed his happiness. In fibrous dysplasia, normal bone is replaced by fibrous connective tissue with varying degrees of osseous metaplasia. It is a benign disorder of bone commonly seen from childhood to the third decade. Virtually any bone in the body can be affected. Regarding investigations, plain radiography in the first line study. CT may be required to assess complex region such as the spine, pelvis, chest and facial skeleton. Histologically, fibrous dysplasia appears as multiple areas of fibrous tissue contained within islands of bone with evidence of both blastic and clastic activity.     

阿伦膦酸钠治疗23例骨纤维结构不良的临床观察

目的探讨阿伦膦酸钠治疗骨纤维结构不良的临床疗效。方法选取我院骨科2009年7月至2012年7月诊治的骨纤维结构不良患者23例,给予口服阿伦膦酸钠70 mg每周1次维持治疗,连续服用1年以上。观察治疗前及治疗6个月、12个月以后患者疼痛、病变部位X线表现及阻射密度、生化指标（ALP）的变化。结果 23例骨纤维结构不良患者经阿伦膦酸钠治疗后,疼痛症状明显改善：显效18例、有效4例、无效1例,总有效率95.63%。19例患者病变部位X片检查发现病损区密度增高、骨皮质增厚及溶骨区域出现明显钙化沉积并且大小未见继续扩大。2例X片检查发现病损区无任何变化。1例病损区有进行性发展的趋势,总有效率82.64%,骨病损区X线平均阻射密度较前明显升高（P〈0.05）。治疗前后血清ALP明显降低,治疗前ALP为（110.25±3.21）U/L;治疗后为（40.35±3.17）U/L,差异有统计学意义（P〈0.05）。结论阿伦膦酸钠可有效抑制骨吸收,促进骨生成,从而抑制骨纤维结构不良的病情发展。     

下颌骨复发性造釉细胞瘤

本文报告20例下颌骨复发性造釉细胞瘤。虽然肿瘤复发大多数出现于病变剜除之后,但受累颌骨切除后亦有复发。我们强调术中应彻底清除可能隐匿在病变周围松质骨中的残余瘤,并且尽量保存下颌骨的连续性以维持其形态和功能。     

Study of 7 Cases of Giant Cell Tumor of Soft Tissue

Background

Primary giant cell tumour of soft tissues is a distinct but uncommon group of neoplasms morphologically identical to osseous giant cell tumor.

Methods

7 patients with painless growing soft tissue mass, having no attachment to underlying bone, were identified in a four years retrospective study from two zonal hospitals of armed forces. Histopathology of these lesions revealed admixture of multinucleated giant cell with mononuclear cells. All patients were treated by surgical resection and followed up for recurrence. Results : There were 5 male and 2 female patients in the age group of 18 to 56 years. All lesions were superficial, circumscribed and involved extremities except one. Histologic transition between benign and malignant lesion was present in only one of the 7 patients that recurred after three months of surgery for which she had to be operated again. 2 of our 7 cases were lost in follow up.

Conclusion

Primary giant cell tumour of soft tissues usually present as a painless mass and needs to be differentiated from other giant cell rich soft tissue tumors. Benign clinical course is expected if the lesion is excised adequately. Its biological behaviour to have low malignant potential is recognized; but this cannot be predicted and metastasis does occur rarely.Key Words: Giant cell tumour of soft tissue, Giant cell tumour of bone, Malignant fibrous histiocytoma     

Paratesticular fibrous hamartoma in an infant

Fibrous hamartoma of infancy is a rare benign tumour with local infiltration. The lesion is usually found in the upper torso and rarely occurs in the genital region. Diagnosis before surgery is rare, and complete excision is essential to prevent recurrence. We present a case in which fibrous hamartoma of infancy involving the spermatic cord was found. A pre-operative clinical diagnosis could not be made. On inguinal exploration, the tumour could not be dissected away from the testicular vessels, which necessitated an orchidectomy for complete removal. The case is presented due to its rarity and successful management.     

Imaging diagnosis of monostotic fibrous dysplasia in thoracic and lumbar spine vertebrae

Summary The X-ray radiograph, CT scan and MRI appearance of 5 patients with pathologically proven fibrous dysplasia in thoracic and lumbar spine vertebrae were retrospectively analyzed. Plain radiographs, CT scans and MR images showed the presentation of eccentric lesion with intact cortex bone and marginal sclerosis in vertebral bodies without involvement of vertebral appendix and extraosseous soft tissue. The lesion masses were round (one being oval-shaped) and radiolucent in plain radiographs and CT scans. Homogeneous long signal was observed on T1 weighted image and strongly enhanced when gadolinium was administered. On T2 weighted MRI, short signal was found in the anterior part of the mass, long signal in the posterior part, and short and slight long signal in the middle part, without partitioning and laminating change. There was a good correlation between radiological features and surgical findings. These findings may be useful to diagnose fibrous dysplasia in spine.     

Imaging Diagnosis of Monostotic Fibrous Dysplasia in Thoracic and Lumbar Spine Vertebrae

The X-ray radiograph, CT scan and MRI appearance of 5 patients with pathologically proven fibrous dysplasia in thoracic and lumbar spine vertebrae were retrospectively analyzed. Plain radiographs, CT scans and MR images showed the presentation of eccentric lesion with intact cortex bone and marginal sclerosis in vertebral bodies without involvement of vertebral appendix and ex- traosseous soft tissue. The lesion masses were round （one being oval-shaped） and radiolucent in plain radiographs and CT scans. Homogeneous long signal was observed on Tl weighted image and strongly enhanced when gadolinium was administered. On T2 weighted MRI, short signal was found in the anterior part of the mass, long signal in the posterior part, and short and slight long signal in the middle part, without partitioning and laminating change. There was a good correlation between radiological features and surgical findings. These findings may be useful to diagnose fibrous dysplasia m spree.     

巨颌症的临床病理研究

目的对巨颌症的临床病理特点进行研究分析。方法对1例巨颌症标本进行常规石蜡切片、HE染色、光镜观察。结果镜下见增生的纤维性组织代替了正常骨组织,纤维呈纵横交叉排列,多量多核巨细胞散在于纤维组织之间或围绕在血管周围。结论巨颌症是一种少见的良性病变,该病的诊断主要依靠组织病理学、临床表现及影像学检查。