Paget disease of bone

Paget disease of bone, also known as osteitis deformans, is a nonmalignant disease of bone that causes accelerated and abnormal bone remodeling. It is the second most common bone disorder, osteoporosis being first. It is a localized disorder, meaning it affects just 1 or several bones in the body. Paget disease of bone is characterized by enlarged and deformed bones and occurs mainly in the axial skeleton. The cause of the disease is still not known entirely, but genetics are certain to be involved, and viral factors also are suspected.     

Giant cell tumor complicating Paget disease of long bone

Giant cell tumor (GCT) is a rare complication of Paget disease of bone. It usually occurs in the skull or pelvic bones of patients with long-standing polyostotic disease. This report describes a 62-year-old patient who presented with monostotic Paget disease of the distal femur complicated by GCT. He had a 2-year history of discomfort and pain in his left knee. Conventional plain films and MRI demonstrated the characteristic bone changes of Paget disease and an associated lytic lesion involving the epiphyseal and metaphyseal regions of the distal femur. A diagnostic curettage showed the characteristic histopathologic features of Paget disease and GCT. There was no evidence of malignancy. The clinicopathologic features of this rare lesion are described and correlated with a review of the literature.     

Place of radiology in giant cell tumors of the bone

The authors present 60 cases of histologically confirmed giant cell tumor classified by a radiological grading as quiet, active and aggressive form. On the basis of our acquired experience and mainly from the follow-up films, all the pertinent radiological findings are analysed either for a more correct therapeutic guidance or for an adequate post-operative follow-up (favourable evolution, definitive recovery, local relapse, malignant degeneration).     

Fluid-fluid levels in giant cell tumors of bone: report of two cases

Fluid-fluid levels have been described in association with aneurysmal bone cysts, telangiectatic osteosarcoma, and a chondroblastoma. We report two cases of giant cell tumors of bone with fluid-fluid levels identified by computed tomography and, in one case, by magnetic resonance imaging. This finding has not previously been associated with giant cell tumors. The radiographic features of the fluid-fluid levels cannot be distinguished from those reported in other osseous neoplasms.     

颞骨骨巨细胞瘤的C T、MR I表现

目的：探讨颞骨骨巨细胞瘤(GCTTB)的CT、MRI影像学表现。方法回顾性分析经病理证实的5例 GCTTB 的影像学资料,分析其病灶的位置、大小、形态、边缘、密度、信号及强化等影像表现,并结合文献探寻其特征性影像表现。结果5例中病灶均位于颞骨的颞颌关节窝附近,病灶呈类圆形,膨胀性溶骨性骨破坏为主,脑侧部分较皮侧大,病灶破坏区内有线样高密度分隔,破坏区边缘颅骨无硬化边,病灶内有斑点状、条样及大片状高密度钙化灶,病变边缘有不连续骨包壳,有“交界角”征,病变周围无软组织肿块,MRI因病灶内成分不同而信号差异明显,增强扫描病灶内实性成分明显强化。结论颞骨骨巨细胞瘤发病率低,明显膨胀生长、有不连续骨包壳、病灶内分隔以及钙化灶多见、有“交界角”征等征象具有相对特征性。     

Sarcoma in Paget disease of bone: clinical, radiologic, and pathologic features in 22 cases

The clinical, radiologic, and histologic features of 22 cases of Paget sarcoma were reviewed to determine in which patients with Paget disease these tumors are most likely to develop and what radiologic findings suggest the diagnosis. Clinical findings at presentation included pain and/or a mass (11 patients), pathologic fracture (seven), and neurologic symptoms (four). Survival time in 20 patients ranged from 5 days to 2.5 years. Two patients were lost to follow-up: one at 2 years and one at 8 years. There were 16 high-grade osteosarcomas, three chondrosarcomas, two fibrosarcomas, and one malignant fibrous histiocytoma. The most common site was the femur. Tumors also were observed in unusual sites. In one case of multifocal osteosarcoma, the tumor involved only pagetic bone. In 15 patients, Paget disease was polyostotic, clinically significant, and had been documented previously. In four patients, a sarcoma developed near the site of a fracture that had occurred between 2 months and 15 years previously. All cases showed radiologic evidence of a destructive lesion; other findings included a mass and evidence of tumor mineralization. Periosteal reaction was not observed. All but one tumor developed in a site of osteoblastic or mixed osteoblastic and lytic Paget disease. Our results suggest that sarcomas can develop in any part of any bone affected by Paget disease but are more likely to occur with advanced disease and to present with a destructive lesion without periosteal reaction.     

Radiology of giant cell tumors of bone: Computed tomography,arthro-tomography,and scintigraphy

Radiologic studies of 50 giant cell tumors of bone in 48 patients were useful in assessing the anatomic extent for planning surgical treatment. Contrast-enhanced computed tomography (CT) provided the most useful and complete evaluation, including soft tissue extent and relationship to major vessels. Angiography was useful when the extraosseous extent and vascular relationships were not entirely clear on CT. Arthro-tomography was the best way to evaluate tumor invasion through subchondral cortex and articular cartilage. Reactive soft tissues, with edema and hyperemia, were difficult to distinguish from tumor tissue on CT and angiograms. Bone scintigrams often showed intense uptake beyond the true tumor limits.     

Untreated Paget disease of bone studied by scintigraphy

We determined that the concentration of radioactivity in a lesion of Paget disease correlates with the grade of radiological deformation and the frequency of pain; the total skeletal uptake correlates with the severity of the biochemical abnormalities. We suggest that the major determinant of uptake in untreated lesions is abnormal metabolic activity, and in lesions in remission it is structural deformation of mineralized tissue. It is likely that the metabolic activity, and possibly also the rate of progression of the individual lesions, will differ in the individual patient, and that metabolic activity determines the amount of deformation and the chance of pain. Lesions not visible on the radiograph usually show only low uptake of Tc-99m-Sn-EHDP; the majority of these lesions are asymptomatic and reflect low activity of the disease. Radiological differentiation between sclerotic and osteolytic lesions does not reflect differences in either scintigraphic uptake, metabolic activity, or pain.     

Paget disease of the spine: CT with clinical correlation

Thirty-six patients with pagetic involvement of the spine were evaluated clinically and by computed tomography (CT). Pagetic phase, modeling expansion, degree and site of spinal stenosis, and pagetic facet joint arthropathy were recorded for each involved vertebral segment. CT demonstrated spinal stenosis in 20 patients, 11 of whom exhibited spinal stenosis on plain films. Twenty-one patients had symptoms of neck or back pain, with associated neurologic dysfunction in 13. Spinal stenosis was present in 81% of the symptomatic patients and 20% of the asymptomatic patients. Severe pagetic facet arthropathy was present in 17 of the symptomatic patients. We conclude that spinal stenosis is an important cause of vertebral pain and neurologic dysfunction.     

58例骨与软组织恶性肿瘤介入治疗——临床,影像与病理综合评价

目的 探讨骨与软组织肿瘤介入治疗的技术和疗效及血管造影表现在评价疗效方面的意义。材料与方法 ５８例骨与软组织肿瘤患者接受介入治疗，并随访３～２４个月，观察临床及放射学改善情况，计算肿瘤坏死百分比并与血管造影表现对照，结果 治疗后大部分患者指标明显改善，肢体肿瘤患者９２％行保肢手术，术后局部复发率为１３．６％，手术病理示５３％（１０／１９）的患者肿瘤坏死率达９０％以上，术后血管造影表现与病理结果相符     

Carcinoid tumors of the stomach: a clinical and radiographic study

OBJECTIVE: Our purpose is to describe associated and coexistent diseases of gastric carcinoid tumors, the unique biologic behavior of these tumors, the appearance of these tumors on fluoroscopic and CT images, and the radiologic management of these neoplasms. CONCLUSION: First, multiple gastric carcinoid tumors are associated with enterochromaffin-like cell hyperplasia, chronic atrophic gastritis, and pernicious anemia and have a low risk of malignancy. Second, solitary gastric carcinoid tumors, or gastric carcinoid tumors associated with multiple endocrine neoplasia-type I (MEN-I) and Zollinger-Ellison syndrome, have a higher potential for metastatic disease. Third, the radiologic appearance and management of these tumors depend on the clinical background of the patient.     

58例骨与软组织恶性肿瘤的介入治疗——临床、影像与病理综合评价

目的 通过对５８例骨与软组织肿瘤与软组织肿瘤局部内行灌注化学药物治疗（简称化疗）和栓塞术后的临床、放射学变化及手术病理研究,探讨骨与软组织肿瘤介入治疗的技术和疗效及血管造影表现在评价疗效方面的意义。方法 ５８例骨与软组织肿瘤患者接受介入治疗,治疗后随访３￣２４个月,观察临床及放射学改善情况,计算肿瘤坏死百分比并与血管造影表现对照。结果 治疗后大部分患者临床指标明显改善,肢体肿瘤患者９２％行保肢手术     

Diagnostic imaging of giant cell tumors

Although classified as benign neoplasms, giant cell tumors are locally aggressive with a high recurrence rate of 30–50%. The histological appearance include osteoclast-like, multinucleated giant cells and round to spindle-shaped mono-nuclear stromal cells. These neoplasms predominatly affect adults in the third and forth decades of life (70–80%). Giant cell tumors predominatly arise in long tubular bones (75–95%) with the majority occurring around the knee (50%). The next most common site being the distal radius (10%). The epicenter of giant cell tumors is in the epiphysis. The radiographic signs of giant cell tumors are a geographical radiolucentcy with no internal mineralisationa, a thinning of the cortex, eccentric in the epiphysis of long bones. MRI signs of giant cell tumors are high signal intensity in T2-weighted images, high contrast media enhancement, fluid levels, signs according to haemorrhage and haemosiderin deposition. CT shows the expanded and thinned cortex. Plain radiographs remain the mainstay of diagnosis of giant cell tumors. MRI and CT are important for staging and therefore for surgical planning.