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PubMed1976

Lipomembranous polycystic osteodysplasia.

A case of lipomembranous polycystic osteodysplasia is presented. The clinical features were characterized by multiple cystic changes of the bones and progressive psychomotor retardation. Although the diagnosis of polyostotic fibrous dysplasia of bone was made first because of expanded tumorlike lesions in bones, pathology of bone and bone marrow confirmed the diagnosis of lipomembranous polycystic osteodysplasia. Light microscopy showed a great number of peculiar undulating membranous structures in the adipose tissue. With the electron microscope the membrane seemed to be composed of an accumulation of endoplasmic reticulum-like tubular profiles. They appear first, in close connection with fat droplets, within the mesenchymal cell.

Adult↗
PubMed1987

Total hip arthroplasty and polyostotic fibrous dysplasia: a case report.

Polyostotic fibrous dysplasia with Albright's syndrome causes progressive skeletal lesions which require orthopaedic surgical management. A case is presented of a 43-year-old woman with a history of multiple proximal femoral osteotomies with iliac bone grafting who developed a degenerative hip joint. A total hip arthroplasty was performed and a specially designed Charnley-Mueller type of prosthesis implanted. The patient is asymptomatic eight years postoperatively.

Adult↗
PubMed1987

[Value and limitations of computerized tomography in the study of benign tumors of the bone].

In order to stress the role of CT in the evaluation of benign tumors of the bone the authors studied by means of a high-resolution CT scanner 74 patients that later underwent surgery (70 cases) and histological examination (all cases). CT findings were compared with the surgical and histological data. The results prove that the diagnostic contributions of CT are less important in the evaluation of benign than in malignant bone tumors. The major contributions of CT were:--the capability of detecting the presence of osteoid osteoma not found by means of conventional radiographic techniques;--differentiation between benign osteochondroma and peripheral chondrosarcoma by measurement of the peripheral cartilaginous cap: this criterion, on the other hand, does not seem to be always reliable and useful;--differential diagnosis of different types of cystic and fibrocystic diseases (simple and aneurysmal bone cyst, fibrous dysplasia);--evaluation of soft-tissue tumoral masses and of their extra-osseus extent in giant cell tumor and chondroblastoma; this allows us to establish the real extension of the tumor and to plan surgery more accurately.

Adolescent↗
PubMed1986

Benign fibrous histiocytoma of bone.

The cases of seven patients who had a lytic lesion that was histologically similar to a metaphyseal fibrous defect (non-ossifying fibroma) of bone were studied. The patients all were adults and had pain without a fracture. These features were considered distinctive for the lesion, which has the same histological appearance as benign fibrous histiocytoma of soft tissue. The lesion is a benign tumor with fibroblastic and histiocytic differentiation. This picture may be seen in foci in other lesions of bone (aneurysmal bone cyst, fibrous dysplasia, and giant-cell tumor). Ten cases of giant-cell tumor of bone that had a large component of the same foci were also reviewed. It should be emphasized that these areas are secondary reactive tissue rather than the true neoplastic tissue of benign fibrous histiocytoma.

Adult↗
PubMed2003

Benign fibrous histiocytoma of the posterior arch of C1 in a 6-year-old boy: a case report.

STUDY DESIGN: Presented is a unique case report of a rare bone tumor: a benign fibrous histiocytoma (BFH) located in the posterior arch of C1 in a 6-year-old child. OBJECTIVE: To describe a benign fibrous histiocytoma of bone and the differential diagnostic considerations based on the authors' case report. SUMMARY OF BACKGROUND DATA: A BFH is a rare tumor composed of varying degree of fibroblast-like spindle cells, foam cells, and multinucleated giant cells. Approximately 86 cases have been reported in literature. Its exact nature remains somewhat controversial. A lesion may be designated a benign fibrous histiocytoma based on clinical, radiographic, and microscopic criteria. MATERIALS AND METHODS: The clinical symptoms, plain radiographs, computerized tomography (CT), magnetic resonance images (MRI), bone scintigraphy, and histologic section of the lesion are discussed, evaluated, and compared with other benign bone lesions. RESULTS: This case is, to the best of the authors' knowledge, the first benign fibrous histiocytoma to be reported in the cervical spine of a child. Various benign lesions such as nonossifying fibroma, giant-cell tumor, fibrous dysplasia, aneurysmal bone cyst, osteoblastoma, and eosinophilic granuloma are included in the differential diagnosis. CONCLUSION: Benign fibrous histiocytoma is a rare skeletal tumor. Because of this and its nonpathognomonic microscopic features, the diagnosis can be somewhat troublesome. However, by systematically reviewing patient's symptoms, tumor location, and radiographic and microscopic characteristics, other benign lesions can be eliminated. The diagnosis of a BFH is one of exclusion.

Cervical Atlas↗
PubMed1984

Monostotic fibrous dysplasia in the femoral neck. A clinicopathologic study.

In eight patients with monostotic fibrous dysplasia in the femoral neck, the lesion was virtually limited to the neck region without any extension into the shaft. In one patient with symptoms of 40 years' duration and another with a recurrent lesion, there was a deformity in the neck of the femur. A pathologic fracture, including a minor one, was identified in two patients. Roentgenographically, the lesion should be distinguished from various entities producing a localized central region of rarefaction in the proximal femur. The histologic features of fibrous dysplasia are characteristically diagnostic. Six of the eight patients were successfully treated by curettage and bone graft. There was no evidence of malignant transformation.

Adolescent↗
PubMed1985

Craniofacial fibrous dysplasia.

This article describes a 20-year-old man with widespread polyostotic fibrous dysplasia that resulted in massive craniofacial deformity with unilateral exorbitism and oronasal obstruction. The patient illustrates the rare occurrence of fibrous dysplasia with major malformation of the face and skull, requiring multiple stages of surgical "sculpture" to improve function and appearance. The disease entity, differential diagnosis, and treatment are discussed.

Adolescent↗
PubMed1984

Mammosomatotroph hyperplasia associated with acromegaly and hyperprolactinemia in a patient with the McCune-Albright syndrome. A histologic, immunocytologic and ultrastructural study of the surgically-removed adenohypophysis.

An 11-year-old girl, with the McCune-Albright syndrome, exhibited fibrous dysplasia of several bones, skin pigmentation, precocious puberty, growth hormone hypersecretion, acromegaly and hyper-prolactinemia. Histologic, immunocytologic and ultrastructural investigation of the surgically-removed pituitary showed massive mammosomatotroph hyperplasia. Since no adenoma was found, the abundance of these bihormonal cells, capable of producing both growth hormone and prolactin, was implicated in the causation of growth hormone and prolactin excess. Somatoliberin overproduction and/or somatostatin and dopamine deficiency could not account for the hypophysial abnormality, since changes in secretory rates of these hypothalamic hormones would lead to proliferation of mature somatotrophs and lactotrophs, rather than mammosomatotrophs. In our patient, a congenital hypothalamic malfunction might have been accompanied by hypersecretion of an unidentified releasing factor, resulting in pathologic differentiation of the pituitary and mammosomatotroph hyperplasia. Alternatively, mammosomatotroph hyperplasia may have been due to an inherent genetic or embryonic defect affecting primarily the pituitary. According to this interpretation, the pituitary lesion represented yet another developmental error in the setting of the McCune-Albright syndrome.

Acromegaly↗
PubMed2006

Fibrous dysplasia protuberans in a patient with McCune-Albright syndrome.

Fibrous dysplasia protuberans refers to a benign fibro-osseous exophytic mass originating in the intra-medullary cavity of an adjacent bone. A case of fibrous dysplasia protuberans is hereby presented, manifesting as an exudative pleural effusion in a 49 years old male with history of McCune-Albright syndrome. This case represents the first reported case of fibrous dysplasia protuberans in a patient with McCune-Albright syndrome. Without a high index of suspicion, detailed radiographic imaging and meticulous histological examination, this benign bony excrescence is likely to be misdiagnosed, especially in a patient with no previous history of fibrous dysplasia.

Fibrous Dysplasia of Bone↗
PubMed1984

Light- and electron-microscopic studies in congenital pseudarthrosis.

This study presents the results of light- and electron-microscopic and enzyme histochemical investigations in ten cases of congenital pseudarthrosis of the lower limb. At the time of surgery, six of the ten patients had not been operated on previously. The characteristic histological feature of the "sclerotic type" of congenital pseudarthrosis was a marked fibromatous reaction consisting of cellular connective tissue. The constituent cells were arranged in bundles and had elongated nuclei. The number of nuclei per visual field was considerably higher in pathological specimens than in specimens from the uninvolved leg. In places, the histological appearance resembled somewhat that of palmar fibromatosis (Dupuytren's disease). Destruction and absorption of bone were always found. Electron-microscopic analysis showed that a large number of the cells represented myofibroblasts. These findings were supported by the positive reaction of the cells for the enzyme diaminopeptidase IV, a marker enzyme for myofibroblasts [30]. As yet it is not possible to decide whether the constriction of the pseudarthritic bone is caused by a thickened myofibroblast-containing periosteum [40] or by the aggressive osteolytic component of the fibromatosis [12, 19, 41]. Furthermore, the relationship of congenital pseudarthrosis to fibrous dysplasia of bone is still unknown. Obviously, there are histological similarities between the two diseases, including the presence of osteolytic fibrous tissue in the medullary cavity and C-shaped bone trabeculae. However, the pattern of bone involvement and prognosis are different. Irrespective of the type of congenital pseudarthrosis, focal angiomatous hyperplasia was noted in some cases. This proliferation of blood vessels is most likely a reactive change.(ABSTRACT TRUNCATED AT 250 WORDS)

Bone and Bones↗
PubMed1997

Primary hyperparathyroidism-associated polyostotic fibrous dysplasia: absence of McCune-Albright syndrome mutations.

Several cases of sporadic primary hyperparathyroidism in association with fibrous dysplasia of the bone have been reported in the English literature. Since fibrous dysplasia is a major feature and hyperparathyroidism is occasionally found in the McCune-Albright syndrome, we hypothesized that such cases may represent a variant of this syndrome. A 28-year-old male had primary hyperparathyroidism associated with polyostotic fibrous dysplasia but no other manifestations of the McCune-Albright syndrome. Genomic DNA samples from his parathyroid adenoma, dysplastic bone sample, and peripheral leukocytes were analyzed for the presence of activating mutations of the stimulating G protein alpha subunit gene (gsp). Allele-specific hybridization revealed the presence of normal sequences only, coding for arginine and glutamine at codons 201 (exon 8) and 227 (exon 9), respectively. Further, single strand conformational analysis of a 224 base pair fragment of exon 8 revealed no conformational aberrations. Furthermore, the sequences of a 164 base pair fragment of exon 8 and a 170 base pair fragment of exon 9 were normal. The results strongly suggest that gsp mutation is absent in affected and normal tissues in this patient and that the association of hyperparathyroidism and fibrous dysplasia may not represent a variant of the McCune-Albright syndrome.

Adenoma↗
PubMed1996

Extradural optic nerve decompression for fibrous dysplasia with a favorable visual outcome.

A 10-year-old boy with progressive left visual disturbance associated with craniobasal fibrous dysplasia underwent left frontotemporal craniotomy. Dysplastic lesions of the sphenoid ridge, orbital roof, anterior clinoid, and ethmoid sinus were removed through an extradural pterional approach and the optic nerve was completely decompressed. His vision was markedly improved postoperatively. Consecutive follow-up studies for 3 years have shown no deterioration of his visual acuity. Early optic nerve decompression is highly recommended to preserve visual function in patients with craniofacial fibrous dysplasia causing visual disturbance.

Child↗
PubMed1990

[Fibrous metaphyseal defect--a stage- and age-dependent differential diagnosis].

Based on 57 fibrous metaphyseal defects the conventional radiologic signs are described as diagnostically reliable including asymptomatic patients as well as patients with a history of pain and lesions with positive scintigraphy. The radiologic morphology of the fibrous endosteal and medullary defects differs with the age of the patient. Beyond the age of 18 years there is a sclerosis of the lesion and a less marked arrosion of the corticalis. The differential diagnosis depends on the cortical, endosteal of medullary site of the fibrous defect as well as on the age of patient and includes the cortical metastasis, the juxta-articular osteoid-osteoma, the periosteal chondroma, the cortical irregularity, the solitary bone cyst, the fibrous dysplasia, the chronic osteomyelitis and the osteosclerotic osteosarcoma.

Aging↗