骨和累及骨的外周性原始神经外胚层瘤的临床病理与影像学表现

目的探讨骨和累及骨的外周性原始神经外胚层瘤(pPNETs)的临床病理与影像学表现。资料与方法7例中6例有X线检查、2例有CT检查、4例有MR检查,分析其临床病理和影像学特点。结果6例X线片上均呈溶骨性骨质破坏,其中2例病变区存在骨质硬化,5例合并软组织肿块,均未见骨膜反应。2例CT像上均为溶骨性骨质破坏合并软组织肿块形成,软组织内未见钙化或骨化。MRI检查4例中有3例病变在T1WI呈中等信号,1例在T1WI呈低信号,4例在T2WI上均呈中、高信号,信号不均匀,增强扫描呈中度不均匀强化。4例均合并软组织肿块,其中3例有囊变坏死区。结论影像学上骨内病变呈溶骨性破坏,可伴有病变区的骨质硬化而一般无骨膜反应,并伴有较大软组织肿块者应考虑到pPNETs的可能。     

外周原始神经外胚叶肿瘤CT、MRI及病理分析

目的 研究外周原始神经外胚叶肿瘤的CT、MRI表现及病理特征.方法 回顾性分析经病理证实的9例外周原始神经外胚叶肿瘤的临床、影像及病理资料.4例行CT检查,5例行MRI检查.结果 本组9例,上颌窦2例,胸壁2例,股骨、眼眶、椎管、肩部及腋窝各1例.8例肿瘤为单发,类圆形多见,5例为软组织肿块伴邻近骨质破坏,囊变坏死少见,未见钙化,CT平扫密度多均匀,MRI平扫为T1等肌肉信号、T2不均匀高信号,增强扫描多为明显强化.2例出现淋巴结转移.肿瘤免疫组化均表达CD99,不表达白细胞共同抗原(LCA).结论 外周原始神经外胚叶肿瘤的CT及MRI表现为非特异性,但能很好地显示肿瘤及侵袭范围,其最终诊断依靠病理和免疫组织化学检查.     

骨外周性原始神经外胚层瘤的影像学表现

目的探讨骨外周性原始神经外胚层瘤(pPNETs)的临床和影像学表现。方法分析10例骨pPNETs的临床和X线、CT及MR影像资料。结果pPNETs多以局部疼痛(9例)伴肿块(7例)为主诉。X线示溶骨性骨质破坏8例，伴有不规则硬化和轻度膨胀的溶骨性骨质破坏1例，未见异常1例；7例见软组织肿块；均未见骨膜反应。7例CT扫描中，边界不清的溶骨性骨质破坏6例。伴有不规则硬化和轻度膨胀的溶骨性骨质破坏1例，均有软组织肿块形成，2例伴有细小的点状钙化，均未见骨膜反应；10例MR扫描中，9例在T1WI呈等信号，1例呈中等偏高信号；在T2WI和短时反转恢复(STIR)序列，8例呈不均匀中、高信号，2例呈均匀高信号；均见明显软组织肿块形成；4个疗程化疗以后，肿瘤有明显缩小。结论骨pPNETs以溶骨性骨质破坏伴明显的软组织肿块、无骨膜反应为主要表现，缺乏特征性，但影像学检查有助于了解病变的范围、治疗措施的制定和治疗效果的评价。     

富于巨细胞的骨肉瘤影像学诊断分析(附7例报告)

目的分析总结7例富于巨细胞的骨肉瘤(GCRO)的影像学表现,并探讨其与典型骨肉瘤、骨巨细胞瘤的鉴别诊断。方法回顾性分析经病理证实的7例GCRO的影像学表现。结果 GCRO病变大部分发生于长骨干骺端,个别病例发生于不规则骨。X线表现为溶骨性骨质破坏,长骨病变呈偏心性生长,骨内病变纵径大于横径,少见骨膜反应,未见肿瘤骨形成。CT及MRI平扫显示软组织病变替代正常骨组织,骨外肿块较大;MRI信号混杂,T2WI见低信号边界,周围骨髓及软组织水肿明显,T1WI显示骨质破坏病变内不规则斑片状高信号骨髓组织残留。增强见丰富肿瘤血管,肿块实质成分不均匀明显强化。结论 GCRO影像学表现为溶骨性骨质破坏,与典型骨肉瘤及良性骨巨细胞瘤有区别,与恶性骨巨细胞瘤鉴别困难。     

脊索瘤的影像学分析

目的：分析脊索瘤的影像学表现，探讨其影像学诊断价值。资料与方法：收集经手术病理证实的12例脊索瘤，所有病例均摄X线平片，其中CT检查8例，MRI检查4例。结果：骶尾部脊索瘤7例，表现为囊性膨胀性及溶骨性骨质破坏，骨壳完整或不完整，骨破坏区内可见散在分布的斑片状、斑点状钙化，周围可见软组织肿块；颅底部脊索瘤4例，表现为溶骨性膨胀性骨质破坏及软组织肿蚨，内可见囊变、出血及钙化灶；腰椎1例，表现为椎体溶骨性骨质破坏及软组织肿块，内可见斑点状钙化。MRI表现为病变呈不均匀长T1、长T2信号，增强扫描表现为不均匀“蜂窝样”强化。结论：通过影像学检查，并结合临床，大部分脊索瘤术前能够正确诊断；CT较X线平片能够更好的显示病变结构及邻近软组织改变；MRI能够精确显示病变范围及周围血管神经的关系，有助于制定合适的手术方案和预后的估计。     

骶椎肿瘤的CT表现及其临床意义

目的探讨骶椎肿瘤的CT表现及其临床意义.方法回顾性分析行CT检查并经临床病理证实的14例(男9例,女5例)骶骨肿瘤,就骨质破坏,钙化,软组织肿块和病变与邻近组织的关系等方面进行分析.结果转移瘤CT表现为椎体和附件骨质破坏,无钙化.脊索瘤亦表现为溶骨性骨质破坏,伴有软组织肿块,常有钙化.巨细胞瘤呈膨胀性生长,压迫周围组织或器官.神经纤维瘤、骨软骨瘤及软骨肉瘤的CT表现典型.结论CT扫描在骶椎肿瘤的发现和估价病变方面具有较高的敏感性,对治疗计划的制订有重要的参考作用.     

外周性原始神经外胚层瘤的影像表现

目的:探讨外周性原始神经外胚层肿瘤的影像学表现.方法:回顾性分析经病理证实的12例外周性神经外胚层瘤的影像学资料.9例行CT扫描,4例行MR扫描,其中1例行DSA检查.结果:胸壁软组织5例,右肺下叶1例,肱骨2例,股骨1例,胫骨1例,脊椎2例.胸壁软组织的pPNETs CT平扫表现为大而边界欠清的软组织肿块,内见坏死,无钙化,增强后明显不均匀强化.右肺下叶的pPNETs CT平扫表现为分叶状肿块,内见少量出血,边界清晰,增强后明显强化.长骨pPNETs表现为溶骨性骨质破坏伴巨大软组织肿块,无钙化,无骨膜反应.T1WI上表现为等或低信号,T2 WI上表现为不均匀高信号,增强后明显强化.脊椎pPNETs表现为椎体或附件骨质破坏伴软组织肿块,不累及椎间盘,其中1例骶骨pPNETs DSA检查示肿瘤由髂内动脉供血和大量新生血管.结论:CT和MRI均能很好地显示pPNETs的内部结构、病变范围、有无侵犯毗邻组织器官或远处转移.DSA可明确肿瘤供血动脉和反映新生肿瘤血管丰富的特点,可作为一种补充检查方法.确诊仍需依靠病理和免疫组化检查.     

骶骨神经源性肿瘤的X线平片及CT分析（附6例报告）

目的：通过分析骶骨神经源性神经肿瘤的Ｘ线和ＣＴ表现，提高其诊断水平，材料与方法，６例骶骨神经源性肿瘤，其中神经鞘瘤４例，恶性肿瘤鞘瘤１例，神经纤维瘤１列，对其平片及ＣＴ表现进行分析，结果：平片表现，骶骨神经源性肿瘤多位于上部骶椎，偏侧性生长，骶孔或骶骨内奢望生扩大破坏或变形及多囊状膨胀溶骨性骨质破坏，边缘骨质硬化，ＣＴ表现，膨胀的溶骨破坏区被软组织肿块充填，骨壁变薄，肿块无钙化，肿瘤破坏骨壁或骶孔     

外周性原始神经外胚层肿瘤的影像学诊断

目的:探讨外周性原始神经外胚层肿瘤(pPNET)的影像学诊断价值,提高对本病的认识.方法:搜集我院经手术病理及免疫组化证实的pPNET 9例.CT检查6例,其中4例增强扫描;MRI平扫 增强检查3例;超声检查4例,分析pPNET的影像学表现特点.结果:发生于后胸壁2例,腰骶部、肩胛骨、鞍结节、鼻腔、颈部、右上臂及卵巢各1例.pPNET多表现为较大实体软组织肿块,成分混杂,血供相对较丰富,呈不均匀性强化;起源骨组织者以溶骨破坏为主,周围见明显软组织肿块;起源于胸壁pPNET常伴有胸膜侵犯及邻近肋骨骨质的破坏.结论:影像学检查能较好地显示pPNET大小、范围及其毗邻关系,对于青少年出现溶骨性破坏伴较大软组织肿块或软组织内见较大异质性肿块应考虑本病的可能,定性诊断仍依赖于病理学检查.     

小儿外周原始神经外胚层肿瘤的MSCT诊断

目的分析小儿外周原始神经外胚层肿瘤(pPNET)的MSCT表现,提高对该病的认识。方法回顾性分析11例经病理确诊的pPNET的MSCT表现。结果 8例巨大软组织肿块伴骨质破坏,其中3例表现为骨质放射状增生伴局部骨质破坏,其中2例位于胸壁,1例位于肩胛骨;5例为溶骨性骨质破坏,其中2例位于后纵隔、1例发生于副鼻窦、1例发生在腰椎和1例发生在骶椎。3例为未伴骨破坏的软组织肿块,其中2例发生于腹膜后,1例发生于皮下脂肪层内。除发生在皮下脂肪层内病灶较小者,其余病灶都较大,平均直径为8 cm。软组织肿块以压迫推移邻近组织的方式生长。瘤体内钙化少见。结论 pPNET的MSCT典型表现为巨大软组织肿块,伴或不伴骨质破坏,增强呈均匀或非均匀的团絮状强化,大血管穿行于瘤体内较少见。有一定的特点,需与其他恶性软组织肿瘤鉴别。MSCT能较好显示病变内部结构,血供状况以及与邻近组织的关系,有助于术前分期、手术方案的确定、有无远处转移和治疗效果评价。     

原发性长骨横纹肌肉瘤的X线诊断

Four cases of primary rhabdomyosarcoma of long bone were reported, all confirmed by operation and pathology. There were 3 male and 1 female. The femur was involved in 1 case and tibia in 3. Based on the radiologic manifestations the tumor could be divided into two different types: (1) Osteolytic form: Presenting as large area of osteolytic destruction without periosteal reaction; (2) Mixed form: In addition to patchy osteolytic destruction, calcification or dense tumor bone in the medullary cavity or soft tissue can be seen, often associated with periosteal reaction.     

软骨母细胞瘤的影像学表现及对比研究

目的分析对比软骨母细胞瘤的X线、CT及MR影像学特征。方法分析经病理证实且于2周内行X线、CT和MR检查的22例软骨母细胞瘤病人的影像学表现。2例同时行CT平扫和增强扫描,7例同时行MR平扫和增强扫描。结果病灶位于胫骨上端11例,股骨下端4例,髌骨2例,肱骨上端、股骨上端、颞骨和下颌骨各1例。1例恶性者发生于髂骨并同时累及骶骨。X线表现为骨质破坏(21),病灶内斑片状、斑点状和(或)条状致密影(5),病灶周围边界不清的斑片状硬化(10)。CT上,病灶呈类圆形分叶状(14),内为软组织密度(7),伴有斑片状、斑点状和(或)结节状钙化(15),病灶周围出现边界不清的斑片状硬化(15),病变周围软组织肿胀(18)。病灶在T2WI和脂肪预饱和T2WI均呈混杂信号(22),病灶周围髓腔内有边界不清的斑片状长T1长T2信号(20)。结论良性软骨母细胞瘤的影像学表现多具有特征性,X线平片、CT和MRI多数征象相互对应。     

Imaging of non-central nervous system primitive neuroectodermal tumours: diagnostic features and correlation with outcome

AIM: To document the varied radiological features before, during, and after treatment of non-Central Nervous System Primitive Neuroectodermal Tumours (PNETs), which are rare tumours of childhood.MATERIALS AND METHODS: Thirty-three children with PNETs have been treated at our institution between 1990 and 1999. Full radiological and clinical follow-up was obtained in 29 (17 females, 12 males). Imaging was retrospectively reviewed, with particular attention to Computed Tomography (CT) and Magnetic Resonance Imaging (MRI).RESULTS: Age range at diagnosis was 0-16 years old (mean 4.4 years). There were five main sites of tumour: head and neck (n = 7), scapula/axilla (n = 2), chest (n = 11), abdomen (n = 3), and spinal/paraspinal (n = 6). Overall mortality was 62%. Tumours of the scapula or paraspinal region appear to show better survival than other sites. Of 23 patients who had Tc99m-methylene diphosphonate (MDP) bone scans at diagnosis, four patients showed widespread distant metastases, seven showed focal increased uptake in an adjacent bone only, and 12 had normal examinations. CT was performed in 25 patients and MRI in 20, both at diagnosis and follow-up. Average size of tumours at presentation was 4.5 cm in the paraspinal, head and neck and scapular regions and 7.5 cm in the chest and abdomen. Tumours were typically of soft tissue density on CT with the larger (>5 cm) masses tending to be more heterogeneous in character. The lesions were slightly higher signal than muscle on T1-weighted (T1W) MRI and all masses were heterogeneous on T2W sequences. Calcification was uncommon (n = 6) and generally sparse. Tumours tended to displace adjacent soft tissue structures such as vessels and bronchi rather than invade or encase them. Tumours rarely crossed the midline. Local or bony invasion was seen in 12 patients at diagnosis. Metastases were identified in the lung (n = 5), pleura (n = 2), brain (n = 4), bone (n = 4), lymph nodes (n = 2), liver (n = 2), subcutaneous tissues (n = 2), kidney (n = 1) and peritoneum (n = 1).CONCLUSIONS: Imaging characteristics of non-CNS PNETs are described. Tumours tend to displace rather than encase adjacent structures; local invasion occurred in 43%. Tumour calcification is uncommon. Poor prognostic features included the presence of distant metastases at diagnosis (all four patients with distant metastases at diagnosis died), but even patients without metastatic disease have a relatively poor prognosis.     

骨韧带样纤维瘤的X线诊断(附6例报告)

目的　探讨骨韧带样纤维瘤的临床与X线特点。方法　回顾性分析经手术病理证实的 6例骨韧带样纤维瘤的临床及X线表现。结果　 6例病变位于股骨 2例 ,胫骨 2例 ,桡骨 1例 ,肱骨 1例。骨质呈囊状膨胀性改变 ,内见粗细不等骨小梁影 6例 ,伴有骨嵴 3例 ,边缘不同程度硬化 6例 ,软组织肿胀 4例 ,未见钙化和骨膜反应。结论　长骨干骺端为好发部位 ,以骨内具有骨小梁结构的囊状膨胀性改变 ,瘤壁硬化 ,无钙化及骨膜反应是该病的主要X线表现 ,而“根须”状肿瘤性骨小梁形成具有一定特征性     

长骨骨干骨肉瘤X线、CT及MRI表现

目的 分析长骨骨干骨肉瘤X线、CT和MRI表现,探讨有关的临床特点和鉴别诊断.方法 28例长骨骨干骨肉瘤患者,均经手术与病理证实,其中病变位于股骨干18例、腓骨干4例、肱骨干4例、胫骨干2例.所有患者均行X线、CT和MR检查,对其影像学表现与手术病理结果进行对照,并由双盲法分析确认.结果 28例中,X线和CT显示广泛骨质破坏16例,骨膜反应22例.X线显示软组织肿块18例,肿瘤骨和瘤样钙化12例.CT平扫显示软组织肿块22例,增强扫描显示软组织肿块24例,肿瘤骨和瘤样钙化16例.MRI显示骨质破坏和骨膜反应10例,软组织肿块26例,其周围可见软组织水肿及骨髓水肿.骨膜反应在SE T1WI上呈等低信号,T2WI呈等信号.软组织肿块在T1WI为等信号,T2WI及STIR呈等高信号.软组织水肿及骨髓水肿在T2WI及STIR呈高信号.MRI增强检查显示病灶均呈不均匀强化,骨髓水肿和软组织肿块均见强化.结论 X线、CT和MRI从不同方面反映长骨骨干骨肉瘤的影像病理特点,其发病率低,骨破坏范围大,无病理性骨折.成骨型骨干骨肉瘤较易诊断,溶骨型应与Ewing瘤、恶性巨细胞瘤等鉴别.

Abstract：

Objective To explore the findings of diaphysial osteosarcoma in long bone on X-ray,CT and MRI, and discuss their clinical features and manifestations for differential diagnosis. Methods Twenty-eight cases with diaphysial osteosarcoma in long bone proved by surgery and pathology were reviewed retrospectively. Eighteen tumors were located in the femur, 4 in fibula, 4 in humerus and 2 in tibia. All of the patients were examined by X-ray, CT and MRI. The imaging manifestations on X-ray, CT and MRI were analyzed, and the relationship of the imaging features with the pathological types was also observed. The imaging signs were correlated with the pathologic findings with a double blind method. Results Of the 28 cases, there were 16 cases with large bone destruction, 22 cases with periosteal reaction on X-ray and CT. On X-ray, 18 cases showed soft tissue mass and 12 cases with neoplastic bone and tumor calcification.While on CT, 22 cases showed soft tissue mass on plain scan and 2 more cases displayed soft tissue mass after the injection of contrast mediun. Sixteen cases showed neoplastic bone and tumor calcification on CT.On MRI, there were 10 cases with bone destruction and periosteal reaction with iso- and hypo-intense on T1WI and iso- signals on T2WI. Twenty-six cases showed soft tissue edema and bone marrow on MRI. The soft mass were iso-signals on T1 WI and iso-hyperintense signals on T2 WI or STIR. The soft tissue edema was found hyperintense signals on T2WI or STIR. The lesions had heterogeneous enhancement especially in bone marrow with edema and adjcent soft tissue. Conclusion The X-ray, CT and MRI can reflect the pathological changes of diaphysial osteosarcoma in long bone from different aspects. Lower incidence, large bone destruction and no pathological fracture were the features of diaphysial osteosarcoma. The osteogenic type is diagnosed easily, but the osteolytic lesion should be differentiated from Ewing sarcoma, malignant giant cell tumor of bone and so on.     

Vascular tumors of bone: Imaging findings

Purpose

To identify radiological features of malignant vascular tumors of bone, which can be used to avoid erroneously diagnosing metastases based on radiological multifocality, and histological epitheloid phenotype.

Materials and methods

From the databases of the Bologna & Netherlands Committee on Bone Tumors, 63 patients with a histological diagnosis of malignant vascular tumor of bone were retrieved. Epidemiological and imaging characteristics were recorded on a case record form.

Results

In 63 patients, 185 lesions were detected by radiographs (61 patients) and/or CT (30 patients) and/or MRI (19 patients). Multifocality was observed in 25 patients (40%), in these patients most lesions were located in the femur. Typically lesions were well-defined, osteolytic, had a geographically pattern of destruction and were also located in the femur. Most lesions showed cortical destruction (118 lesions). No periosteal reaction was seen in most cases (121 lesions). In 13 of 39 patients (33%) tumor extension was more advanced and/or (additional) lesions (29 lesions; 17%) were visible on MRI and CT. In 20 cases (51%) cortex destruction was better shown on CT or MRI. In six patients (15%) periosteal reaction was only seen on MRI or CT and not on radiographs. In 16 (41%) cases soft tissue extension was only seen on MRI or CT, and not on radiographs. Extensive reactive changes on T2-weighted images were seen in 11 patients (58%).

Conclusion

When single, or regional multifocal osteolytic, well-marginated lesions with cortical destruction are seen, in the femur, and with marked reactive soft tissue changes on MRI, a diagnosis of malignant vascular tumor should trigger the use of additional immunohistochemistry to confirm the vascular nature of the tumor.

Clinical relevance statement

Because of epithelioid phenotype at histology, radiological signs are key in entertaining a diagnosis of malignant vascular tumor of bone which should trigger the use of appropriate immunohistochemical stainings.     

恶性纤维组织细胞瘤的影像学诊断

目的探讨原发性恶性纤维组织细胞瘤影像学特征。材料和方法回顾性分析5例原发性恶性纤维组织细胞瘤的X线、CT或MRI影像表现特点。结果5例均经病理证实,分别发生于小腿软组织、胫骨干及腰椎各1例,髂骨2例。原发于骨者影像学主要表现为骨质破坏、软组织肿胀或巨大肿块、患骨膨胀,骨膜反应少见,病损部位可见钙化。发生于软组织者表现为非特异性肿块,邻近骨正常或受累。结论原发性恶性纤维组织细胞瘤的X线、CT或MRI均有特征性表现,CT或MRI在确定肿瘤侵犯范围与软组织肿块方面明显优于X线。     

骨纤维肉瘤的CT和MRI诊断

目的：评价CT和MRI在骨纤维肉瘤中的诊断价值。方法：回顾性分析经手术病理证实的7例骨纤维肉瘤的平片、CT和MRI表现。结果：7例均为中央型，其中股骨3例，胫骨2例，肱骨1例，肋骨1例。影像学表现主要为无膨胀性骨质破坏，4例边界清楚，3例边界模糊。CT能清晰显示骨皮质的侵蚀、断裂以及骨内膜的虫蚀状破坏，1例CT见散在颗粒状钙化，1例可见较大软组织肿块，7例CT均未见到骨膜反应。4例MRI检查T1WI上3例均匀低信号，1例稍低信号欠均匀，T2WI上随分化程度高低而不同，1例中心明显液性高信号，手术病理证实为坏死。结论：CT和MRI对骨纤维肉瘤有较高诊断价值，但其本身并无特异征象。     

胃肠道间质瘤的螺旋CT诊断

目的:探讨胃肠道间质瘤(GIST)的螺旋CT表现及诊断价值.方法:回顾性分析经手术和病理证实的胃肠道间质瘤患者18例,术前均行螺旋CT扫描,7例行双期增强扫描.结果:肿瘤位于胃部8例,食管1例,十二指肠2例,小肠4例,回肠末端小肠系膜、横结肠系膜各1例,未确定原发部位者1例.无论良、恶性,CT表现17例边界清楚;瘤体内多发坏死、囊变10例,无囊变者密度均匀,为软组织密度;肿瘤边缘分叶12例;病变腔内侧面溃疡7例,气体及对比剂进入其中;钙化1例,转移2例,未见淋巴结转移;增强扫描,肿瘤中度到明显强化;螺旋CT定位准确度为77.8%,判定良、恶性准确度为50%.结论:螺旋CT对于胃肠道间质瘤的定位和提示诊断都具有非常高的价值,对肿瘤良恶性的判断有一定限度,确诊有赖于病理学检查.