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PubMed1952

Fibrous dysplasia.

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Bone Diseases, Developmental↗
PubMed2006

Malignant change secondary to fibrous dysplasia.

BACKGROUND: Malignant change in fibrous dysplasia (FD) is very rare. This study was carried out to establish some characteristic clinical information about this disorder. METHODS: Four cases with a malignant change in FD out of 128 cases with FD were surgically treated and followed up for a median period of 61.3 months. The mean age of the patients was 39.8 years. Clinical features, radiological findings, and the outcome were analyzed for each of the four cases. RESULTS AND CONCLUSION: The sites of the lesions were tibia (2 cases), femur (1 case), and rib (1 case). The forms of FD were monostotic in one case and polyostotic in three cases. Radiologically, plain films and computed tomography (CT) showed osteolytic lesions with poorly delineated margins within and/or near areas having a ground-glass appearance. In the osteolytic lesions, simple cystic changes associated with old FD could be excluded by enhanced magnetic resonance imaging (MRI). Histopathologically, two cases were osteosarcoma, one case was malignant fibrous histiocytoma (MFH), and one case was fibrosarcoma. The management of this disease should be decided according to the type of primary high-grade bone sarcoma. One patient, with MFH, was dead of lung metastasis 13 months after surgery. The others are alive without disease.

Adult↗
PubMed1999

[Osteofibrous dysplasia and its differential diagnosis].

We present a case of a 13 year old girl with swelling and pain of the right lower leg persisting for four months. The radiological and histological diagnosis is osteofibrous dysplasia. Osteofibrous dysplasia is a rare bone lesion predominantly affecting the cortex of the tibia. Almost all patients are younger than ten years at presentation. Swelling is a typical clinical manifestation. Radiologically and even histologically the distinction between the osteofibrous dysplasia and the more aggressive adamantinoma can be difficult. Other bone lesions potentially mimicking osteofibrous dysplasia include: fibrous dysplasia, osteoid osteoma, osteosarcoma, osteoblastoma and intracortical Brodie's abcess. The natural course of the disease is unpredictable. Lesions with typical radiological appearance are considered to be "no touch lesions". In equivocal cases block resection (with both a diagnostic and therapeutic purpose) should be performed.

Adolescent↗
PubMed2006

McCune-Albright syndrome and acromegaly: effects of hypothalamopituitary radiotherapy and/or pegvisomant in somatostatin analog-resistant patients.

BACKGROUND: Acromegaly, which may be present in patients with McCune-Albright syndrome (MCAS), in association with café-au-lait spots, precocious puberty, and fibrous dysplasia, is often difficult to treat surgically because skull base bone dysplasia prevents the removal of the pituitary adenoma. Somatostatin analogs (SAs) generally give only partial responses. The use of radiotherapy (RT) is controversial because of a possible risk of bone sarcomatous transformation. AIM: This study was a retrospective analysis of the efficacy and adverse effects of different treatment modalities in six patients with both MCAS and acromegaly. PATIENTS AND METHODS: Because surgery was impossible and SA failed to normalize GH/IGF-I hypersecretion, five of the six patients received fractionated RT (45-55 Grays). Three patients (two with previous RT) were also prescribed pegvisomant. We analyzed the clinical features of acromegaly, GH, and IGF-I concentrations and bone radiological features. RESULTS: GH and IGF-I concentrations fell after RT (median follow-up, 5 yr; range, 0.5-9 yr). Symptoms of acromegaly improved in parallel. Bone sarcomatous transformation was only noted in one patient in a region (the mandible) outside the radiation field. RT alone and/or combined with SA failed to normalize GH/IGF-I levels in the five patients concerned. In contrast, IGF-I levels normalized very rapidly (5-9 months) in the three patients receiving pegvisomant (10-20 mg/d). CONCLUSION: RT may be an option for the treatment of acromegaly in patients with MCAS when surgery is impossible and SA therapy is ineffective. However, although no bone sarcomatous transformation was observed within the radiation field in this series, this risk cannot be ruled out. As shown in this small series of severely affected patients, pegvisomant therapy may thus be useful to normalize IGF-I levels rapidly.

Acromegaly↗
PubMed1991

[Roentgenographic diagnosis of bone tumors--its basic knowledge and clinical application].

Roentgenographic diagnosis of bone tumors is considered to be extremely important to decide whether a new patient should be biopsied immediately to make the proper diagnosis or the patient could be followed up without operative treatment. The basis knowledge important in making the proper decision on the roentgenogram of the bone tumor, are presented and four cases of bone tumors or tumor-like conditions are presented to show how such knowledge becomes useful. First of all, the basic knowledge based on the anatomical peculiarities are described. Chordoma deriving from the remnant tissue, notochord, should be placed in the midline of the body. Metaphyseal region of the growing child showed a local immune deficient state because of the special anatomic arrangement of the vascular structure for the sake of the open epiphyseal line. This is thought to be related with the high rate of primary malignant bone tumors in this area and in this age group, for example, osteosarcoma. Next, roentgenographic changes are described separately: intracortical changes, cortical changes, periosteal reaction and soft tissue extension. These changes are summarized to differentiate the benign lesion from the malignant one. Thirdly, the peculiar location of the tumor is described. Examples are giant cell tumor, chondroblastoma and clear cell chondrosarcoma in the epiphyseal region, osteoid osteoma, intracortical abscess and the osteofibrous dysplasia in the intracortical region.

Animals↗
PubMed1995

Criteria and quantification of fibrous dysplasia on MDP scanning.

Eleven patients with histologically proven fibrous dysplasia (biopsy) were imaged with 3-phase bone scanning, using 99mTc-MDP. Ten had monostotic and one had polyostotic fibrous dysplasia. The perfusion (phase I) and uptake (phase III) of monostotic lesions was compared to the normal contralateral side and the rest of the skeleton was inspected for other abnormalities. Lesions presented with slightly increased perfusion (1.65 normal +/- 0.907) and markedly increased uptake of tracer (6.0 normal +/- 2.58). Other features that were noted are high spinal and scapula and poor renal tracer uptake.

Adolescent↗
PubMed2005

Skeletal changes in epidermal nevus syndrome: does focal bone disease harbor clues concerning pathogenesis?

Epidermal nevus syndrome (ENS) is a rare, sporadic, congenital disorder of unknown etiology featuring a complex and highly variable phenotype that can include focal or generalized skeletal disease. We describe a young man with ENS manifesting right-sided linear skin lesions, generalized weakness, diffuse osteopenia associated with hypophosphatemic rickets, and distinctive focal bone lesions ipsilateral to the skin findings. Review of the literature concerning ENS-associated skeletal disease suggested such focal bone defects are fibrous dysplasia, but our patient did not have the typical radiographic or histopathologic findings of fibrous dysplasia. Nevertheless, his circulating fibroblast growth factor 23 (FGF-23) level was elevated, likely functioning as a "phosphatonin," yet no activating mutations in GNAS previously reported in fibrous dysplasia or McCune-Albright syndrome were detected in his leukocytes or affected skin. We postulate that the focal skeletal disease, although different than fibrous dysplasia, may be a source of FGF-23 in ENS.

Adolescent↗
PubMed1993

Magnetic resonance imaging findings of craniofacial fibrous dysplasia.

There have been few reports in the literature concerning the magnetic resonance imaging (MRI) appearances of fibrous dysplasia. We reviewed MRIs of 4 patients with craniofacial lesions. Using a 1.5-T Signa Unit (General Electric, Milwaukee, WI), both T1- and T2-weighted MRI scans were performed. In our 4 patients, the fibrous dysplastic lesions were characterized by a decreased signal on both T1- and T2-weighted images. And all lesions had sharply demarcated borders. The fine image of fibrous dysplasia by MRI is extremely useful, not only as a diagnostic aid, but also as an invaluable guide to surgery.

Adolescent↗
PubMed2006

Metabolic bone disease in children : etiology and treatment options.

Metabolic bone disease in children includes many hereditary and acquired conditions of diverse etiology that lead to disturbed metabolism of the bone tissue. Some of these processes primarily affect bone; others are secondary to nutritional deficiencies, a variety of chronic disorders, and/or treatment with some drugs. Some of these disorders are rare, but some present public health concerns (for instance, rickets) that have been well known for many years but still persist. The most important clinical consequences of bone metabolic diseases in the pediatric population include reduced linear growth, bone deformations, and non-traumatic fractures leading to bone pain, deterioration of motor development and disability. In this article, we analyze primary and secondary osteoporosis, rickets, osteomalacia (nutritional and hereditary vitamin D-dependent, hypophosphatemic and that due to renal tubular abnormalities), renal osteodystrophy, sclerosing bony disorders, and some genetic bone diseases (hypophosphatasia, fibrous dysplasia, skeletal dysplasia, juvenile Paget disease, familial expansile osteolysis, and osteoporosis pseudoglioma syndrome). Early identification and treatment of potential risk factors is essential for skeletal health in adulthood. In most conditions it is necessary to ensure an appropriate diet, with calcium and vitamin D, and an adequate amount of physical activity as a means of prevention. In secondary bone diseases, treatment of the primary disorder is crucial. Most genetic disorders await prospective gene therapies, while bone marrow transplantation has been attempted in other disorders. At present, affected patients are treated symptomatically, frequently by interdisciplinary teams. The role of exercise and pharmacologic therapy with calcium, vitamin D, phosphate, bisphosphonates, calcitonin, sex hormones, growth hormone, and thiazides is discussed. The perspectives on future therapy with insulin-like growth factor-1, new analogs of vitamin D, strontium, osteoprotegerin, and calcimimetics are presented.

Bone Development↗