Radiological features of systemic mast-cell disease

Radiological studies were done on 23 patients with systemic mast-cell disease (SMCD). Significant changes occur most often in bones and less commonly in the gastrointestinal tract and other visceral organs. These changes may be related either to tissue infiltration by mast cells, or to the effect exerted on tissues by chemical mediators of the mast cells, although in some instances findings may be coincidental. Because the radiological changes are not unique to SMCD, their main value, in association with the clinical information, is in directing further studies for diagnostic confirmation and in estimating the extent of systemic involvement.     

Radiological assessment of aluminium-related bone disease

Two hundred and fifty-nine radiological skeletal surveys were reviewed in 67 cases of end-stage renal failure. Fractures were identified in 16 patients, of whom 12 (17.9% of total) had aluminium-related bone disease. Moderate or severe fracturing osteopathy with more than five fractures not explained by trauma was 100% specific for aluminium intoxication. It is sufficient to perform radiological skeletal surveys in the assessment of renal osteodystrophy annually. They should include radiographs of the fingers, a lateral view of the lumbar spine and oblique views of the ribs. The primary aim of reporting on such surveys should be to grade the severity of fracturing osteopathy and of subperiosteal erosions.     

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.     

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.     

Bilateral Paget disease of the calcaneus diagnosed by conventional bone scintigraphy

An 85-year-old woman who had an invasive ductal carcinoma of the breast and elevated alkaline phosphatases (420 U/L) was referred for Tc-99m MDP bone scintigraphy for initial evaluation of skeletal metastases. Bone symptoms were limited to chronic pain in both knees. A bone scan revealed pagetoid findings in both calcanei. Feet and toes on plain x-rays of the calcaneus were unremarkable. Findings were stable in the 2-year follow up, excluding other potential diagnosis as extensive metastases or stress fractures.     

Paget disease with contraction of long bones

The evolution of Paget disease characterized both by expansion and subsequent contraction of the long bones of the ipsilateral upper extremity over a 10-year period is described.     

Radiological and clinical features of aneurysmal bone cyst of the jaws

PURPOSE: To evaluate the clinical and radiological features of aneurysmal bone cyst (ABC) of the jaws. MATERIALS AND METHODS: A total of 64 cases (60 from the English-language literature and four new cases) were studied and critically evaluated with emphasis on the radiological features. RESULTS: Age at time of initial diagnosis ranged from 4-78 years, with a mean age of 21.5 years and median age of 17 years. There were no differences in gender distribution. The ratio between the mandible and maxilla was 2.4:1. Ninety-two per cent of the lesions were located in the posterior region of the jaws. Lesions were radiolucent in 87% of cases, radiopaque in 2% and mixed in 11%. Fifty-three per cent were multilocular, 43% unilocular and 3% not loculated. The border of the lesions was defined but not corticated in 39%, well defined in 33% and diffuse in 28%. CONCLUSIONS: ABC has a variable radiological appearance and should be considered in the differential diagnosis of any unilocular or multilocular radiolucent lesion of the jaws as well as any mixed radiolucent-radiopaque lesion.     

Radiological demonstration of healing in Paget's disease of bone treated with APD

Twenty-three patients with Paget's disease received the bisphosphonate APD and were examined radiologically every 6 months. Because routine roentgenographic procedures were followed, a number of the X rays were not fit for comparison. The radiographic technique, as well as the positioning of the patients, is critical since both could lead to artefacts. All patients reached normal biochemical levels, usually within 6 months. Out of the 23 patients 11 showed definite radiological improvement in one or more lesions; probable improvement was seen in another three. Of the 65 individual lesions, with films that were comparable, 30% definitely and 20% probably improved; 50% did not change but deterioration was never encountered. Osteolytic lesions in the long bones are most suitable for the evaluation of radiological changes and follow-up of these lesions during treatment should be most rewarding.     

Chondrosarcoma as a complicating factor in Paget’s disease of bone

 A 65-year-old male patient with an 8-year history of poliostotic Paget’s disease complained of shoulder pain that started 6 months prior to admission. An extensive lytic area was identified in the right proximal humerus along with Paget’s disease. There was cortical destruction and a soft tissue mass. Following an incisional biopsy, a diagnosis of grade 2 chondrosarcoma associated with Paget’s disease was made. The histologic identification of chondrosarcoma associated with Paget’s disease is rare. However, the presence of a calcified matrix in a destructive lesion associated with Paget’s disease should alert the radiologist and the pathologist to the possibility of a chondromatous differentiation taking place in the sarcoma associated with Paget’s disease. The histologic evaluation of the lesion will form the basis for the diagnosis.     

Critical illness myopathy associated with hyperthyroidism

A 51-year-old woman was admitted to the intensive care unit for exacerbation of chronic obstructive pulmonary disease. She received antibiotics, neuromuscular blocking agents, and steroids. After 8 days in the intensive care unit, she was noted to be severely weak, her serum creatine kinase had risen to 1,692 U/L (normal, 20-220 U/L), and a muscle biopsy was consistent with critical illness myopathy. As a result of evaluating for resting tachycardia, the patient was found to be hyperthyroid. Her weakness rapidly improved within 1 month after treatment of her hyperthyroidism with iodine-131 and methimazole. The metabolic alterations associated with hyperthyroidism may enhance the risk of developing critical illness myopathy after the administration of antibiotics, neuromuscular blocking agents, and steroids in the intensive care unit.     

Multiple giant cell tumors and Paget disease of bone: radiographic and clinical correlations

The clinical and radiographic findings of four patients with multicentric giant cell tumor (GCT) of bone and Paget disease were retrospectively reviewed. Three patients underwent magnetic resonance (MR) imaging evaluation; all patients underwent computed tomography (CT). The MR characteristics of the bone component in pagetic GCT appeared to reflect the pagetic phase; a sclerotic pattern was largely represented by hypointense marrow signal intensity on images obtained with both long and short repetition times (TRs) and echo times (TEs). Conversely, a tumor appearing in a mixed pagetic phase demonstrated more heterogeneous signal intensity with all pulse sequences. Extensive soft-tissue components, noted in all cases, showed largely intermediate signal intensity on short TR/TE images and foci of increased signal intensity on longer TR/TE images. In most cases, dramatic reduction in tumor bulk was noted with the use of steroids alone. An awareness of this entity is important because the appearance of lytic lesions with soft-tissue extension in patients with Paget disease does not necessarily imply a grave prognosis. Serial CT or MR imaging is helpful in monitoring the remissions and exacerbations that reflect response to therapy in Paget disease and GCT.