Search

Goal: Inpatient health care data are often used as a source of information for health reporting in Germany, despite the fact that a lot of diseases are predominantly treated in the outpatient sector. This study provides a first overview of the outpatient care situation in relation to Paget's disease., Method: Outpatient care data from the Association of Statutory Health Insurance Physicians constituted the database for a descriptive analysis, capturing the state of medical care for the rare rheumatic illness Paget's disease (Osteodystrophia deformans) in the region of East Westphalia-Lippe in 2005., Results: While the health report of North Rhine-Westphalia documents a total of 56 cases of M. Paget discharged from hospital for the year 2003, 166 patients suffering from Paget's disease consulted an practice-based physician in 2005 in the district of Detmold alone. The latter figure corresponds to 8.0 treated patients per 100.000 inhabitants. The treatment rates of men and women are comparable. The probability of treatment increases with advancing age. On average, patients with Paget's disease are 65.6 years old (SD=15.4 years). Almost 90% of the diagnoses of Paget's disease are classified as M88.9 according to ICD 10; more exact localisations are provided only for relatively few cases. Nearly a quarter of the cases (24.1%) are treated by general practitioners and internists. Anaesthetists treated 17.5% of the patients and orthopaedists 16.9%. In addition, ophthalmologists treat a considerable proportion of cases (12.0%)., Conclusion: Consistent with the rareness of Paget's disease, the treatment data are as low as expected. However, the results show that routinely collected health care data allow insights into morbidity structures within the outpatient sector. It follows that for statutory health insurants (approximately 90% of the population) there should be an extension of health reporting to diseases that are mainly treated in outpatient settings.

Juvenile Pagets disease (JPD), a rare genetic disorder characterized by markedly accelerated bone turnover, presents in early childhood. We report a child with typical features of JPD who remained undiagnosed till 15 years of age. Rarity of this disease in Indian literature and need for early diagnosis to prevent progression of disease prompted us to report this case.

This is the second of a two-part unit on Paget's disease. Part 1 outlined the epidemiology, pathophysiology, causes and clinical features of the condition. This part outlines advice on diagnosis, drug therapy, surgery, other management options and emotional support.

Paget disease of the cervical spine is a common disease in an uncommon location. The authors present an unusual case of a 58-year-old woman with a pathological fracture through the base of the odontoid vertebra secondary to Paget disease. The purpose of this case report is to describe the radiological features of Paget disease and their important application to treatment management.

Despite significant advances in management, Paget disease remains an enigmatic disorder. There are no animal models, and while its end result --a focal disorder of accelerated bone turnover--is easily recognized, the causes and evolution of the disorder remain uncertain. Recent evidence strongly implicates both genetic and environmental factors in its etiology. The authors consider some of the unresolved questions surrounding Paget disease, including the attenuating prevalence and severity of the disease; how these observations might be reconciled with an apparently highly penetrant genetic susceptibility; what the putative environmental triggers of Paget disease might be; and what relapse after treatment tells us. Most observations seem to fit best with the idea that Paget disease behaves as a multifocal benign neoplasm.

Consensus guidelines for the treatment of Paget's disease of bone have been published, but it is not known how closely these reflect clinical practice. We conducted a multi-centre, stratified, retrospective review of case notes of 531 subjects treated for Paget's disease of bone between 2000 and 2005 in 29 Australian centres. The subjects received 1072 courses of bisphosphonate treatment (pamidronate 363, alendronate 324, risedronate 208, tiludronate 103, zoledronic acid 69, and etidronate 5). The most recent treatment received was oral therapy in 57% of patients (alendronate 29%, risedronate 24%, and tiludronate 4%) and intravenous in 43% (pamidronate 33%, and zoledronic acid 10%). For oral bisphosphonates, the percentages of courses which were at the recommended dosage and duration were: alendronate 33%, risedronate 60% and tiludronate 29%. Pamidronate was administered in a wide range of dosing schedules, most commonly 60 mg every 3 months (18%), 6 months (17%) or annually (12%), whereas zoledronic acid was mainly given as a 4 mg infusion (98%) as a single dose (52%) or annually (19%). Most clinicians reported taking into account symptoms, plasma alkaline phosphatase activity and anatomical location of disease in determining the need for treatment. Patient preference, intolerance of oral therapy and compliance were ranked highest in determining the choice between oral and intravenous therapy. We conclude that oral and intravenous bisphosphonate dosing regimens are both commonly used to treat Paget's disease of bone in Australia. Only a minority of courses of oral bisphosphonate treatment are at the recommended dosage and duration, and there is a lack of consensus on regimens for intravenous treatment.

Objective: To describe the magnetic resonance imaging (MRI) findings of thoracic and lumbar epidural lipomatosis associated with Paget disease of the spine., Summary of Background Data: A 60-year-old male presented with progressive weakness of both limbs and dorsalgia and subsequently developed paraparesis., Methods: MRI of the thoracic and lumbar spine were undertaken and urgent decompression laminectomy followed., Results: MRI of the thoracic spine disclosed abnormal tissue extending posteriorly in the epidural space displacing the spinal cord. The lesion had intermediate to high signal intensity on T1 weighted images and intermediate signal intensity on T2 weighted images. MRI of the lumbar spine revealed abnormal tissue in the extradural space with the same MRI characteristics as seen in the thoracic spine. There was also abnormal signal intensity of the thoracic and lumbar vertebrae at the same levels where the abnormal epidural tissue existed. Abundant infiltrated epidural adipose tissue was removed during urgent decompression laminectomy of the thoracic spine and pathology diagnosed epidural lipomatosis. Histology of the bony elements of the specimen was consistent with Paget disease. Postoperative MRI showed resolution of the epidural mass of the thoracic spine and the patient was discharged 6 weeks after the surgery. Six months later the patient experienced progressively aggravated lumbar pain and sciatica. A follow-up MRI of the lumbar spine showed an increase in the amount of the epidural adipose tissue at this level and more pronounced fatty deposition of the involved lumbar vertebrae. Findings were consistent with progression of Paget disease and lumbar epidural lipomatosis and patient was treated with zoledronic acid. His neurologic function returned to normal within 3 weeks. He is free from symptoms on 5 months follow-up., Conclusion: Paget disease of the spine may rarely be complicated by spinal epidural lipomatosis and may be considered in patients with paraparesis and symptoms of spinal stenosis.

Paget's disease of bone (PDB) is a chronic disorder of bone metabolism characterized by abnormalities of osteoclast function possibly based on the genetic background and slow viral infection. The symptoms of PDB are usually pain and deformity in involved bones and a subsequent increasing risk of pathological fractures, neurological complications and secondary malignant tumor development. The diagnosis of PDB is based on the clinical manifestations carefully examined, laboratory data such as measurement of serum alkaline phosphatase and characteristic findings of radiology including bone scintigraphy. The pathological examination also utilized for diagnosis of PDB in order to exclude bone metastases. Recently, the guidelines for diagnosis and management of PDB in Japan is proposed. According to these guidelines, etidronate and calcitonin are approved by government for treating PDB and surgical treatment are suggested to apply for orthopaedic problems in PDB. Also, rehabilitation is important for preventing the onset and worsening of de-conditioning secondary due to the complications of PDB.

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.

Context: Sarcomatous transformation is a rare complication of Paget disease of bone. Prognosis in patients with other types of sarcomas arising in bone has improved in the last several decades because of therapeutic advances. However, because of the rarity of Paget sarcoma, outcome studies in these patients are limited., Objective: To determine whether prognosis for Paget sarcoma has improved., Design: Seventy cases of sarcomas arising in the setting of Paget disease were collected, and the histologic and clinical findings were reviewed. Clinical follow-up was obtained in 67 cases., Results: Sarcoma arising in Paget disease tended to arise in older men (46 men, 24 women; age range, 31-88 years; mean age, 66 years) and predominated in the axial skeleton (n = 37), especially in the pelvis. Thirty-three patients had a clinical history of Paget disease ranging in duration from 16 months to 30 years (mean, 15 years). No significant difference in incidence between monostotic (n = 33) and polyostotic (n = 36) disease was noted. Most tumors were osteosarcomas (88%). All tumors were high grade. Follow-up information was obtained in 67 of 70 cases (range of follow-up, 1-252 months). Survival ranged from 1 month to 20 years, with a 5-year survival rate of 10%., Conclusions: Prognosis remains poor in patients with Paget sarcoma. There is no significant correlation between the number of bones involved with Paget disease or the duration of disease and development of Paget sarcoma. Poor prognosis in Paget sarcoma is unrelated to site or stage at presentation.

Paget's disease of bone is a chronic focal abnormality of bone turnover that remains totally asymptomatic over a very long period of time but that eventually ensue in bone pain and skeletal deformities. Although, in the last decade new insights have been obtained on its etiology, this remains largely obscure. Effective medical treatment (based on the use of bisphosphonates) has become available and the diagnostic procedures are now well defined. However, there remains considerable controversy regarding the hierarchy of diagnostic procedures and the medical treatment threshold. In the last few years different institution have published national guidelines, reflecting local national health systems and the available medical treatment. In this review, a working group derived from members of the SIOMMMS has examined the information available regarding the diagnosis and treatment of Paget's disease in order to develop guidelines to assist in the management of this condition. The first draft was then extensively reviewed by experts derived from the most representative scientific societies of rheumatology, internal medicine, and orthopaedic surgery. The document provides the most updated recommendations based primarily on the "evidence-based- medicine" but also on the Italian regulation for the diagnostic procedures and on the available medical treatments.

Objectives: To evaluate the epidemiology, quality-of-life (QoL) impact, management, and cost of Paget's disease of bone., Methods: Retrospective and prospective data were collected in a representative cross-section of office-based and hospital-based rheumatologists in France, in early 2005. Each rheumatologist included consecutive outpatients with Paget's disease seen over a 2-month period. For each patient, a medical questionnaire and the 36-item Short-Form self-questionnaire (SF-36) evaluating health-related quality of life were completed. A descriptive analysis of the patient population was conducted. Medical costs over the last 12 months were estimated., Results: Four hundred and forty-six medical questionnaires and 387 SF-36 questionnaires were available. Mean age was 74 years, and 58.3% of patients were male. The diagnosis was fortuitous (usually radiological) in 39.2% of cases. Complications occurred in 40% of cases; 97.8% of complications involved the joints. Bisphosphonate therapy was used in nearly 84% of patients. The SF-36 scores were significantly decreased compared to the general population, in both males and females. Total cost of management was less than one-third the cost of managing type 2 diabetes mellitus., Conclusion: The results confirm the well-established characteristics of Paget's disease. Bisphosphonate therapy was widely used. Quality of life was significantly altered. The cost of management was moderate.

Paget's disease of bone is a focal disorder of bone remodeling accompanied initially by an increase in bone resorption, followed by a disorganized and excessive formation of bone, leading to pain, fractures and deformities. It exhibits a marked geographical variation in its prevalence. In Brazil it predominantly affects persons of European descent. The majority of the reported cases of the disease in Brazil are from Recife, owing to its peculiar mixed European colonization over approximately four centuries. The etiology is complex and involves both genetic and environmental factors. The disease is often asymptomatic and diagnosis is usually based on biochemical markers of bone turnover, radionuclide bone scan and radiological examination. Bisphosphonates, in particular zoledronic acid, are regarded as the treatment of choice for Paget's disease of bone.

Study Design: Retrospective case study of 13 cases of Paget sarcoma of the spine accrued from a prospectively collected Tumor Registry database., Objectives: To analyze the clinical, radiologic, and histologic features of Paget sarcoma of the spine and to determine the factors influencing the prognosis., Summary of Background Data: Paget disease of bone is a common disorder with the spine being involved in over 50% of patients. However, sarcomatous degeneration in the vertebral column is an extremely rare complication. There is very little in the literature with regard to clinical presentation and prognosis of patients with Paget sarcoma affecting the vertebral column., Methods: Between January 1944 and December 2003, 89 patients were registered with a diagnosis of Paget sarcoma in the Scottish Bone Tumor Registry. Thirteen patients with Paget sarcoma of the spine were analyzed with regard to their clinical, radiologic, and histopathologic features along with the prognostic predictors., Results: The mean age was 66.9 years (range: 56-79 years). There were 10 males and three females. There were seven cases involving the sacral spine (63.6%), three cases involving lumbar vertebrae, two affecting the dorsal spine, and one with diffuse dorsolumbar involvement (D11-L3). The mode of presentation was progressively increasing low back pain (in all 13), unilateral sciatica (six; left-sided, five; right-sided, one), bilateral sciatica (two), lower limb weakness (eight), and autonomic dysfunction (four). Ten of 13 cases (76.9%) were osteosarcoma. The rest were chondrosarcoma (n = 1), fibrosarcoma (n = 1), and malignant fibrous histiocytoma (n = 1). Decompression laminectomy was performed in three patients with progressive neurologic deficit. Eight patients had received radiotherapy. The mean survival was 4.22 months., Conclusions: This series confirmed that Paget sarcoma of the spine has a very poor prognosis. We found a constellation of symptomatology in patients with sarcomatous Paget spine resulting from radiculomedullary compression, primarily lumbosacral involvement and predominantly osteosarcomatous histology. There was no significant difference observed on the overall prognosis of the patients with Paget sarcoma of the spine in the last 6 decades.

Introduction: Medical care costs are difficult to calculate in diseases such as Paget's disease because they have low detection rates and a wide range of clinical manifestations that commonly occur in aging patient populations., Materials and Methods: Using 2001-2002 MarketScan Research databases, this study linked medical claims, prescription records, and encounter data on 2.8 million active and retired employees to create a longitudinal panel with 24 months of observation. Patients with Paget's disease were identified by ICD-9 code 731.0. Matched controls (MC) were identified through an exact match procedure using gender, age, and predicted Medicare costs estimated with a risk adjuster. Diagnostic and expenditure records were extracted for the sample and prevalence rates calculated for 20 conditions with well-documented associations to Paget's disease. Comorbidities and health care costs of Paget's disease patients were compared to those of the MCs, and the differences tested using Chi-square and t tests., Results: Our study identified 244 matched pairs. The average age was 72.7 years; 50.8% were female. Significantly higher comorbidities (P < 0.05) were detected in Paget's disease patients relative to MCs for: pathological fractures (4.9% vs. 0.4%), heart murmurs (3.3% vs. 0.4%), low back pain (19.7% vs. 8.6%), spinal stenosis (16.4% vs. 9.8%), and hearing loss (13.5% vs. 5.7%), respectively. Biannual per patient outpatient costs were significantly higher in Paget's disease patients (Paget's disease $9301 vs. MC $6339, P < 0.05), especially for services associated with physician visits and diagnostic tests. Prescription costs for antiresportive agents and analgesics were also higher (Paget's disease $1115 vs. MC $507, P < 0.05). Inpatient costs (Paget's disease $16,144 vs. MC $21,480) were comparable., Conclusion: This study is the first to describe the excessive costs of Paget's disease, based on known patterns of disease expression, evaluation, and treatment.

We here propose guidelines for the diagnosis and management of Paget's disease of bone (PDB) in Japan. These guidelines provide basic information on the epidemiology, pathophysiology, clinical signs and symptoms, diagnosis, indications for treatment, and available therapy, including orthopedic surgery. PDB is a chronic disorder characterized by focal abnormalities of bone turnover. The characteristic feature of PDB is excessive osteoclastic bone resorption coupled to increased and disorganized bone formation. The most common symptom of PDB is pain in involved bones. The most serious complication of PDB is malignant bone or soft-tissue tumor. PDB is uncommon in Japan; our survey in 2003 found 169 patients with PDB. The prevalence of PDB in Japan is 0.15/100 000; in patients aged 55 years or more, the proportion reaches 0.41/100 000. A careful medical history and physical examination are essential for the diagnosis. The diagnosis of PDB is based on finding the typical features on radiographs. Bone scintigraphy and measurement of serum alkaline phosphatase are sensitive means of screening for PDB. Since PDB is a rare disease in Japan, bone biopsy is quite often used to exclude bone metastases. The only evidence-based indication for treatment of PDB is pain in involved bones. In Japan, etidronate and calcitonin are approved by the Ministry of Health, Labour and Welfare for treating PDB, but currently risedronate is also under development for treating PDB in Japan. Indications for surgical intervention in PDB include unstable fractures, osteoarthritis, malignant soft-tissue tumor, osteosarcoma, and bone deformity.

Unlabelled: Paget's sarcoma is a rare entity that principally occurs in elderly people with extensive Paget's disease. The primary sites of occurrence are the pelvis, proximal femur, proximal humerus, and calvarium. The lesions are highly malignant and the patients frequently are found to have metastases at the time of discovery. The survival rate reported in the literature generally is very poor. We were able to locate 16 patients with Paget's sarcoma in the Dr. Henry L. Jaffe Pathology Collection treated from 1942 to 1967. These were added to 27 patients treated for the disease by the Harvard Orthopaedic Oncology Group from 1972 to 2001. Three were giant cell tumors, which are occasionally found in patients with florid Paget's disease but are not malignant. Of the remaining 40 patients, outcome data were available on 35 patients. The rate of Musculoskeletal Tumor Society Stage III tumors was very high (35%) and the rate of survival was 14% at approximately 2.5 years, without differences between the two series. In view of the remarkable concordance in the poor survival figures for patients treated between 1946 and 1967 and those treated between 1972 and 2001 it appears that little progress has occurred in the treatment of Paget's sarcoma over the years, which is in sharp contrast with the current statistics for standard osteosarcoma., Level of Evidence: Prognostic study, Level IV (case series). See the Guidelines for Authors for a complete description of levels of evidence.

This study aimed to analyse the clinical, radiological and histological features of 16 scapulo-humeral Paget's sarcoma cases accrued from Scottish Bone Tumour Registry between January 1950 and December 2000. The mean age was 61.5 (range, 44-77) years with 12 men and 4 women. There were three scapular cases and 13 humeral (1 whole length, 5 upper humeral, 5 lower humeral and 2 mid-humeral). These patients presented with progressively increasing pain in the shoulder, arm or elbow (n = 5), a painful mass associated with a pathological fracture (n = 4), a pathological fracture with progressively worsening pain (n = 3), a painful mass (n = 3) and a painless mass associated with wrist drop (n = 1). Histology showed predominantly osteosarcoma (n = 12), followed by pleomorphic sarcoma (n = 2), malignant fibrous histiocytoma (n = 1) and fibrosarcoma (n = 1). Overall, the median survival period was 4.5 months. In summary, Paget's sarcoma of the scapulo-humeral area presents with progressively worsening pain, a painful mass with or without a pathological fracture, predominantly lytic in humeral and purely sclerotic in scapular lesions, osteosarcoma-dominant histology, primarily treated with an early limb ablative surgery and associated with a poor 1-year survival (12.5%) and 5-year survival (6.2%) rate.

When confronted with a radiographic osteolytic lesion where the diagnosis of Paget disease is considered, preservation of fatty marrow signal intensity on MRI excludes more ominous diagnostic considerations.

Paget's disease (PD) is the second most common bone disease in older adults, yet many clinicians are unfamiliar with it and may not consider it a diagnostic possibility. This article uses a case study to introduce the topic of Paget's disease. The definition, incidence, and etiology of PD is addressed. Normal bone physiology is defined in contrast to the pathophysiology of PD. Patient history, laboratory tests, and imaging studies are summarized with regard to the diagnosis of PD. Treatment options are discussed including pharmacologic management and prognosis. The article concludes with implications for nursing care of the patient with PD.

Background: Paget disease was first described in 1877 by Sir James Paget. It is a focal disorder of bone remodelling, involving increased bone resorption and formation. The aetiology is uncertain but both environmental and genetic factors are thought to be involved in pathogenesis., Objective: This article outlines the clinical presentation, diagnosis and treatment of Paget disease., Discussion: Paget disease is associated with musculoskeletal pain, significant disability and impaired quality of life. Complications include pathological fracture, arthritis in adjacent joints, hearing loss, other neurological complications, heart failure and, rarely, osteosarcoma. Recent clinical trial data has demonstrated histological and radiological improvements in bone of patients treated with bisphosphonates. There is little data evaluating the long term effect of therapy on the risk of complications, however, restoration of normal bony architecture offers the prospect that complications related to deformity and increased bone fragility might be reduced by effective therapy.

Paget's disease of bone is characterized by an anarchic bone remodelling, associated with morphological and functional abnormalities of osteoclasts. Its prevalence and incidence rates decreased gradually over the past two decades; the reason for this remains unclear. The aetiology of the disease is still obscure, the paramyxoviral theory being very controversial. Recent advances in understanding of the disease come from genetic studies, with the identification of specific mutations in the p62-sequestosome gene, which could be involved in pathogenetic mechanisms leading to increased osteoclast activity. The disease affects one or several bone pieces, leading to bone pain, deformities, characteristic imaging features, and increased markers of bone remodelling. The long-lasting disease activity leads to complications, including arthropathies, neurological compressions, fissures or fractures and, rarely, osteosarcomatous transformation of a pagetic lesion. Potent bisphosphonates have proven their efficacy in reducing symptoms and disease activity. They are currently used as the first-line treatment with the goal of normalizing bone remodelling and, hopefully, preventing late complications.

Osteoporosis is the most common contributing factor of spinal fractures, which characteristically are not generally known to produce spinal cord compression symptoms. Recently, an increasing number of medical reports have implicated osteoporotic fractures as a cause of serious neurological deficit and painful disabling spinal deformities. This has been corroborated by the present authors as well. These complications are only amenable to surgical management, requiring instrumentation. Instrumenting an osteoporotic spine, although a challenging task, can be accomplished if certain guidelines for surgical techniques are respected. Neurological deficits respond equally well to an anterior or posterior decompression, provided this is coupled with multisegmental fixation of the construct. With the steady increase in the elderly population, it is anticipated that the spine surgeon will face serious complications of osteoporotic spines more frequently. With regard to surgery, however, excellent correction of deformities can be achieved, by combining anterior and posterior approaches. Paget's disease of bone (PD) is a non-hormonal osteometabolic disorder and the spine is the second most commonly affected site. About one-third of patients with spinal involvement exhibit symptoms of clinical stenosis. In only 12-24% of patients with PD of the spine is back pain attributed solely to PD, while in the majority of patients, back pain is either arthritic in nature or a combination of a pagetic process and coexisting arthritis. In this context, one must be certain before attributing low back pain to PD exclusively, and antipagetic medical treatment alone may be ineffective. Neural element dysfunction may be attributed to compressive myelopathy by pagetic bone overgrowth, pagetic intraspinal soft tissue overgrowth, ossification of epidural fat, platybasia, spontaneous bleeding, sarcomatous degeneration and vertebral fracture or subluxation. Neural dysfunction can also result from spinal ischemia when blood is diverted by the so-called "arterial steal syndrome". Because the effectiveness of pharmacologic treatment for pagetic spinal stenosis has been clearly demonstrated, surgical decompression should only be instituted after failure of antipagetic medical treatment. Surgery is indicated as a primary treatment when neural compression is secondary to pathologic fractures, dislocations, spontaneous epidural hematoma, syringomyelia, platybasia, or sarcomatous transformation. Five classes of drugs are available for the treatment of PD. Bisphosphonates are the most popular antipagetic drug and several forms have been investigated.

Paget's disease and fibrous dysplasia are benign disorders that can involve the temporal bone and skull base. They commonly lead to otolaryngologic symptoms such as impingement of cranial nerves or the orbit or blockage of the external auditory canal or paranasal sinuses, although they can often be a challenge to diagnose because of their insidious presentation. Their benign nature and common presentation within the difficult-to-access confines of the skull base should lead the clinician to exercise caution in their treatment, reserving surgical intervention for either diagnosis or the relief of symptoms. As a better understanding of the etiology of these conditions develops and new pharmacotherapeutic agents are tested, it is likely that physicians will be able to turn to medical rather than surgical techniques to treat these disorders.

Paget's disease is a metabolic bone disease characterized by abnormalities of bone turnover, structure and architecture. The disease is of unknown aetiology, although both genetic and environmental factors have been implicated. Treatment is indicated for patients with active disease and currently bisphosphonates are the first-choice management option.

The European Musculo Skeletal Oncology Society (EMSOS) has carried out a retrospective review of patients over the age of 40 years with osteosarcoma. 481 patients from 12 centres or multicentric groups were included. 42 patients had osteosarcoma arising in Paget's disease, median survival was 9 months. Patients with axial or metastatic tumours also did badly whilst 41 patients with radiation-induced osteosarcoma had a prognosis paralleling conventional osteosarcoma matched for patient age and site of the tumour. 238 patients had high grade non-metastatic osteosarcoma and had a survival of 46% at 5 years. Older patients had less chemotherapy and fared worse. Osteosarcoma in the elderly is a curable condition and warrants intensive treatment with chemotherapy and surgical resection.

Paget disease of bone is a common disorder affecting approximately 3%-4% of the population over 40 years of age. The pathologic abnormality in Paget disease is excessive and abnormal remodeling of bone. Three pathologic phases have been described: the lytic phase (incipient-active), in which osteoclasts predominate; the mixed phase (active), in which osteoblasts cause repair superimposed on the resorption; and the blastic phase (late-inactive) in which osteoblasts predominate. Radiographic appearance of Paget disease reflects these pathologic changes and is often characteristic. Initially, there is osteolysis, particularly affecting the skull (osteoporosis circumscripta) and subchondral long bones, with subsequent development of trabecular and cortical thickening and enlargement of bone in the mixed phase of the disease. Finally, areas of sclerosis may develop in the blastic phase. Frequent sites of involvement include the skull (25%-65% of cases), spine (30%-75%), pelvis (30%-75%), and proximal long bones (25%-30%). Bone scintigraphy typically demonstrates marked increased uptake of radionuclide in all phases of Paget disease. Computed tomography and magnetic resonance imaging often show changes similar to those seen radiographically in noncomplicated Paget disease with maintenance of yellow marrow. Complications of Paget disease include the effects of osseous weakening (deformity and fracture), arthritis, neurologic symptoms, and neoplastic involvement. Sarcomatous transformation is the most feared complication, occurring in approximately 1% of cases, and is seen on images as focal bone destruction extending through the cortex with an associated soft-tissue mass. Recognition of the radiologic spectrum of the appearances of Paget disease usually allows prospective diagnosis and differentiation of its associated complications, which helps guide therapy and improve patient management.

Paget's disease of bone (also known as osteitis deformans) is a nonmalignant disease involving accelerated bone resorption followed by deposition of dense, chaotic, and ineffectively mineralized bone matrix. The origin of the disease is unknown, and it is frequently asymptomatic; however, the patient may present with symptoms depending on the bones involved. The most common symptom is pain in the affected bone; neurologic, hearing, vision, cardiac, and oncologic complications are possible. Diagnosis is primarily made by radiographs. Bisphosphonates are the most common treatment.

The first report of a patient with gross Paget's disease and progressive hearing loss who successfully underwent hearing rehabilitation with an osseo-integrated hearing aid is presented. The otological manifestations of Paget's disease and the principles of osseo-integration are discussed. The use of a bone-anchored hearing aid (BAHA) in selected patients with Paget's disease can provide useful amplification and hearing rehabilitation.

A review of the literature was conducted to study the pathomechanics by which Paget's Disease of bone (PD) alters the spinal structures that result in distinct spinal pathologic entities such as pagetic spinal arthritis, spinal stenosis, and other pathologies, and to assess the best treatment options and available drugs. The spine is the second most commonly affected site with PD. About one-third of patients with spinal involvement exhibit symptoms of clinical stenosis. In only 12-24% of patients with PD of the spine is back pain attributed solely to PD, while in the majority of patients back pain is either arthritic in nature or a combination of a pagetic process and coexisting arthritis. Neural element dysfunction may be attributed to compressive myelopathy by pagetic bone overgrowth, pagetic intraspinal soft tissue overgrowth, ossification of epidural fat, platybasia, spontaneous bleeding, sarcomatous degeneration and vertebral fracture or subluxation. Neural dysfunction can also result from spinal ischemia, when blood is diverted by the so-called "arterial steal syndrome". Because the effectiveness of pharmacologic treatment for pagetic spinal stenosis has been clearly demonstrated, surgical decompression should only be instituted after failure of antipagetic medical treatment. Surgery is indicated as a primary treatment when neural compression is secondary to pathologic fractures, dislocations, spontaneous epidural hematoma, syringomyelia, platybasia, or sarcomatous transformation. Since, in the majority of cases with pagetic spinal involvement, there are also coexisting osteoarthritic changes, antipagetic medical treatment alone may be disappointing. Therefore, one must be careful before attributing low back pain to PD alone. Five classes of drugs are available for the treatment of PD: bisphosphonates, calcitonins, mithramycin (plicamycin), gallium nitrate, and ipriflavone. Bisphosphonates are the most popular, and several forms have been investigated, but only the following forms have been approved for clinical use: disodium etidronate, clodronate, aledronate, risedronate, neridronate, pamidronate, tiludronate, ibadronate, aminohydroxylbutylidene bisphosphonate, olpadronate, and zoledronate. Several of these forms are still under investigation.

Paget's disease of bone affects 10% of those surviving to the eighth decade of life. Relatively easy to diagnose, treatment decisions are more complex. Surgery and immobilization present unique challenges often requiring prophylaxis to prevent activation/exacerbation of the disease.

Objective: To report the clinical and pathologic features of sarcomatous transformation of the skull with involvement of the orbit, in Paget's disease., Design: Interventional case report., Participant: An 83-year-old woman with Paget's disease who experienced progressive proptosis of the left eye., Intervention: Fine-needle aspiration biopsy of the orbital mass., Results: Cytologic examination revealed round to oval malignant cells with wispy cytoplasm, consistent with the diagnosis of sarcoma. The patient died shortly after institution of orbital radiation therapy and systemic steroid therapy., Conclusions: Proptosis related to Paget's disease is uncommon, and only rarely is it a result of sarcomatous transformation. Sarcomatous transformation of the skull involving the orbit should be included in the differential diagnosis of progressive proptosis in patients with Paget's disease.