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A simple bone cyst containing secretory cells in its lining membrane in a patient with polyostotic fibrous dysplasia.

An 11-year-old boy, severely affected with polyostotic fibrous dysplasia, showed radiographically rapid expansion of a cystic lesion in his right humerus. At biopsy, there was an extraordinarily thin shell of bone and a cavity encapsulated by a hypertrophic fibrous membrane and filled with yellow serous fluid. Histologically, in addition to typical features of fibrous dysplasia, the fibrous capsule membrane was composed of proliferated mesenchymal cells characteristic of the affected bone. Ultrastructurally, many secretory granules were observed in numerous cytoplasmic vacuoles in the capsular cells as well as in the cultured cells isolated from the evacuated fluid.

Biopsy↗

Lectin histochemistry of pathological bones.

Our present knowledge of the structure and function of glycoproteins in bone tissue is very limited. The introduction of lectins into histology offered principally a new approach for studying the presence and chemical structure of glycoproteins in tissue sections. In this paper these highly specific carbohydrate binding molecules have been used to characterize glycoproteins in the cellular elements of normal bone and benign bone lesions. We retrospectively examined 35 benign bone lesions (7 fibrous dysplasias, 5 foreign body granulomas, 5 epulis, 8 osteoid osteomas, 10 giant cell tumors) together with 25 normal bone samples. In normal bone samples and all cases of benign bone lesions, two characteristic types of PNA binding were found after neuraminidase digestion in osteoclasts. In osteoclasts which did not adhere to bone surface, diffuse intracytoplasmic PNA binding was seen, and following the adherence to the bone surface, it disappeared, and the resorption zone became stainable. We assume that this PNA binding glycoprotein is formed in the cytoplasm of osteoclasts, then, after the activation of osteoclasts, the glycoprotein gets accumulated at the resorption zone of the cytomembrane where it plays a significant role in the bone resorption.

Bone Diseases↗

Craniofacial surgery and optic canal decompression in adult fibrous dysplasia.

A 29-year-old female had a 3-year history of bony swelling over the right frontal area. For 3 months she noticed proptosis of her right eye. Investigations revealed fibrous dysplasia involving the right half of the frontal bone and the right greater and lesser wings of the sphenoid bone. Visual evoked potentials (VEP) showed delayed latencies on the involved side. A craniofacial surgery with optic canal decompression was performed. Follow-up after 2 years revealed normalization of VEP.

Adult↗

[Bone dystrophies].

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Fibrous Dysplasia of Bone↗

[Two cases of coexisting dysplasia fibrosa and mesenchymoma].

Two patients with dysplasia fibrosa who also had a tumor of mesenchymal origin are presented. One of them underwent one time resection of dysplasia fibrosa focus and the tumor, the other had the tumor resected 17 years after surgical resection of focus in the femur. The authors suggest an etiologic relationship between these two conditions based on mesenchymal changes--the occurrence never described previously. They also indicate efficacy of surgical treatment.

Adult↗

[Classic adamantinoma, differentiated adamantinoma and osteofibrous dysplasia of long bones].

BACKGROUND: Just for over 100 years, adamantinoma has been recognized as a primary enigmatic bone tumour with epithelial characteristics and predominantly involving the tibia. Several similarities between osteofibrous dysplasia has been recognised, differentiated adamantinoma and classic adamantinoma. The purpose of this study was to compare these lesions and to define their histogenesis and mutual relations. METHODS AND RESULTS: Comparison of the clinical, radiologic, histological, immunohistochemical and electron microscopic findings was carried out on 6 cases of classic adamantinoma, 2 cases of differentiated adamantinoma and 2 cases of osteofibrous dysplasia. This study confirmed the epithelial nature of long bone classical and differentiated adamantinomas irrespective of their wide-ranging morphologic pattern that can mimic tumours of various origins. Both types of adamantinoma were positive for cytokeratins in coexpression with vimentin. The epithelial component of the differentiated adamantinoma was much smaller than in classic adamantinoma and was present in scattered islands or single cells distributed within fibrovascular stoma. In one case the scattered epithelial cells had abundant eosinophilic cytoplasm and they resembled rhabdoid elements. On the basis of distinct histological pattern, a new variant of differentiated adamantinoma was described--a rhabdoid variant. In our cases of osteofibrous dysplasia occurring in two children with deformity of the tibia no epithelial cells were identified by the immunohistochemical methods. The lesions were composed of variably shaped spicules of woven and lamellar bone separated by a fibrovascular stroma. The woven bone spicules were surrounded by a uniform rim of plump osteoblasts. The lesion exhibited a zonal phenomenon with maturation of woven bone to bone with a lamellar configuration at the periphery of the lesion. The similar zonal phenomenon was also observed in the cases of differentiated adamantinoma. CONCLUSIONS: On the basis of the clinical, histological, immunohistochemical and electron-microscopical studies, a common histogenesis for classic adamantinoma, differentiated adamantinoma and osteofibrous dysplasia seems likely. The epithelial cell components of these lesions exhibit similar cytokeratin immunoprofiles and ultrastructural features. However, the prognosis for classic adamantinoma is much less favorable than that for cases of osteofibrous dysplasia and differentiated adamantinoma.

Adolescent↗

Rare benign bone tumors and dystrophy in the hand. Review of literature and report of four cases.

In 1986, the Groupe d'Etude des Tumeurs osseuses (Bone Tumors Studying Group), organised a multicentric study of benign bone tumors and dystrophies localised in the hand. The result was very poor, because only five cases were found. Adding these cases to those already published in the literature, we report: 58 aneurysmal cyst, 12 chondromyxoid fibroma, 9 chondroblastoma, 6 bone cyst, 5 monostotic fibrous dysplasia. The total of 90 cases seems to confirm that the hand is a very unusual localisation for these tumors. These concerned equally metacarpal and phalanx for 37 each, but the carpus was concerned in only 7. Diagnosis was regularly missed before operation, and chondroma was usually suggested; the reason is probably that these tumors lost their usual characters in this particular localisation, except in two cases of fibrous dysplasia. In most cases curettage and cancellous graft seems sufficient to ascertain histology and cure the lesion.

Adult↗

Case report 587: Adamantinoma of the tibia mimicking osteofibrous dysplasia.

This case emphasized that the differential diagnosis of osteofibrous dysplasia and adamantinoma of the tibia can be difficult on roentgengrams as well as on histological studies. A radiological and histological diagnosis of osteofibrous dysplasia of the tibia in a young adult should always raise the consideration of the possibility of an adamantinoma, and in the correlation of the radiological features with the biopsy findings it is very important to gain representative tissue material for establishing the correct diagnosis of adamantinoma.

Adolescent↗

Ophthalmic manifestations of fibrous dysplasia: a disease of children and adults.

OBJECTIVE: To determine whether adults constitute a significant proportion of patients presenting with ophthalmic complications of fibrous dysplasia. DESIGN: A retrospective case series. PARTICIPANTS: Twenty patients with biopsy-proven fibrous dysplasia participated. MAIN OUTCOME MEASURES: Patient demographics, major signs and symptoms, occurrence of visual loss, pattern of bone involvement, and radiographic appearance of fibrous dysplasia were studied. RESULTS: In contrast to observations made in the past, the authors found that adults constitute a significant proportion of patients suffering from the ophthalmic complications of fibrous dysplasia. Of 20 patients, 9 were younger than 18 years of age (children) and 11 were 18 years of age or older (adults) at the time of presentation. Changes in facial contour and symmetry were the most common presenting signs and symptoms. Five patients, four of whom were adults, presented because of acute visual loss. Most patients had monostotic lesions that crossed suture lines to involve multiple craniofacial bones. On computed tomographic scan, most fibrous dysplasia lesions had a characteristic, pagetoid appearance, with alternating areas of radiolucency and radiodensity. CONCLUSIONS: Because fibrous dysplasia of the orbital bones can be a cause of significant dysfunction and disfigurement, as well as a treatable cause of blindness in both children and adults, the diagnosis of fibrous dysplasia should not be ruled out based solely on the age of the patient. The characteristic radiologic appearance of this disease allows one to differentiate fibrous dysplasia from other tumors associated with bony expansion or density changes, specifically meningioma.

Adolescent↗