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[Intraosseous osteolytic lesions. Diagnostic, differential diagnosis and therapy (author's transl)].

Any pathological damage occurring in a bone will produce either an osteolytic or osteosclerotic lesion which can be seen in the macroscopic specimen as well as in the roentgenogram. Various bone lesions may lead to local destructions of the bone. An osteoma or osteoplastic osteosarcoma produces an osteosclerotic lesion showing a dense mass in the roentgenogram; a chondroblastoma or an osteoclastoma, on the other had, induces an osteolytic focal lesion. This paper presents examples of different osteolytic lesions of the humerus. An osteolytic lesion seen in the roentgenogram may be either produced by an underlying non-ossifying fibroma of the bone, by fibrous dysplasia, osteomyelitis or Ewing's sarcoma. Differential diagnostic considerations based on the radiological picture include eosinophilic bone granuloma, juvenile or aneurysmal bone cyst, multiple myeloma or bone metastases. Serious differential diagnostic problems may be involved in case of osteolytic lesions occurring in the humerus. Cases of this type involving complications have been reported and include the presence of an teleangiectatic osteosarcoma as well as that of a hemangiosarcoma of the bone.

Bone Cysts↗

Osteofibrous dysplasia.

Osteofibrous dysplasia is a benign, slowly progressive lesion which generally involves one tibia of an infant or young child, and presents as anterior bowing associated with a painless mass. Surgical intervention is usually not indicated but, when carried out, recurrences are common. Osteofibrous dysplasia is readily distinguished histologically from fibrous dysplasia by fibrous stroma, containing bone trabeculae which are rimmed by osteoblasts, and by a "zonal architecture." The radiographic appearance is that of a benign, lucent, intracortical lesion which is expansile and produces bowing of the tibia. Four patients with this condition are presented.

Age Factors↗

Monostotic fibrous dysplasia of the left parietal bone--case report.

A 14-year-old female presented with a hard, painless mass, 5 x 5 cm, in the left parietal region. Skull x-rays showed a radiolucent skull tumor with a sclerotic margin in the parietal region. Computed tomography revealed an intradiploic multilocular mass separated by bony trabeculae. The outer table had thinned and protruded outward. The inner table was also thin and protruded inward slightly. External carotid angiography revealed a faint tumor stain and feeding from the middle meningeal artery. Bone scintigraphy revealed abnormal uptake in the lesion. Total removal of the skull tumor and cranioplasty were performed. The histological diagnosis was fibrous dysplasia. Fibrous dysplasia within the cranial vault is often expressed as painless bulging without neurological symptoms. Surgery is recommended when neurological symptoms and/or cosmetic problems are present. Histological confirmation of the diagnosis is also important.

Adolescent↗

Monostotic fibrous dysplasia of the foot.

Fibrous dysplasia is a benign, nonfamilial disorder of the skeleton, characterized by expanding fibroosseous lesions occupying single or multiple bones with possible extraskeletal anomalies. Fibrous dysplasia begins in childhood and is not usually recognized until adolescence or adulthood. When fibrous dysplasia affects the foot, it is often the expression of the polystotic form of the disease. This paper will review the pathology of the disease and monostotic involvement of the first metatarsal.

Fibrous Dysplasia of Bone↗

[Malignant transformation of fibrous dysplasia. About a case review of the literature].

A case of chondrosarcoma arrising from polyostotic form of fibrous dysplasia coming after 35 years is reported. About this new occurence, a review of the 61 cases of the literature is effectuated. Sarcomatous transformation is a rare complication of fibrous dysplasia. Its frequency change with the authors, because of lack of proof of the preexistence of fibrous dysplasia. She occur specially on polyostotic fibrous dysplasia. This complication is severe, death following before two years. In the literature several etiological factors are discussed: repeated X-rays, radioscopic examinations; cartilaginous nodules. In our case we means that the sarcoma arrise on a chondroblastic bone healing.

Bone Neoplasms↗

[Identification of serum alkaline phosphatase from human bone].

Polyacrylamide gel electrophoresis utilizing sodium dodecyl sulfate followed by specific staining for alkaline phosphatase was accomplished using sera from patients with osteosarcoma, polyostotic fibrous dysplasia, metastatic bone tumor, and idiopathic hyper-alkalinephosphatasemia. Alkaline phosphatase activity of the sera was uniformly demonstrated at a molecular weight of 60,000. L-homoarginine more strongly inhibited the alkaline phosphatase activity than did L-phenylalanine. Alkaline phosphatase activity was markedly inactivated by heating. Regarding substrate specificity, the hydrolysis of p-nitro-phenylphosphate occurred at a lower rate than did that of phenylphosphate. By contrast, the hydrolysis of alpha- and beta-glycerophosphate occurred at a higher rate than did that of phenylphosphate. As seen from the data presented here, the serum alkaline phosphatase samples obtained from these patients with skeletal disorders have several common characteristics.

Alkaline Phosphatase↗

Treatment of benign lesions of the femoral head and neck.

Benign lesions of the proximal femur usually weaken the supporting framework in the femoral neck. Of seven patients aged 11 through 26 years with the diagnosis of fibrous dysplasia (four), aneurysmal bone cyst (two), and simple bone cyst (one), six were treated with curretage and an autogenous fibula strut graft in conjunction with a sliding hip screw. One patient was treated with only the curretage and strut graft. The functional results were excellent (five), good (one), and fair (one), with no local recurrence. In treating fibrous dysplasia, dysplastic bone should be supplemented with grafts of cortical bone. This construct provides increased strength and prevents deformity and fracture, but it does not eradicate the disease. Internal fixation promotes union of the cortical graft to the host cancellous bone. The sliding screw stabilizes the bone and eliminates the need for plaster casts.

Adolescent↗

[Fibrous dysplasia of the skull: neurologic manifestations].

The neurological manifestations of eight patients with osseous fibrous dysplasia of skull were retrospectively studied. In this series, the frontal bone was involved in 75% of the patients, the sphenoid or temporal bones in 37.5%, and the parietal in 25%. It was observed that cranial deformities occurred in all but one case, headache was present in 87.5% of the patients, visual disturbances in 50%, proptosis in 25%, and auditive symptoms in 12.5%. Six out of the eight patients were operated on: three of the four who presented progressive visual loss had postoperative improvement. In their follow up, none presented, until now, evidence of recurrence or worsening of symptoms, confirming the benign nature of fibrous dysplasia.

Adolescent↗

Optic neuropathy in McCune-Albright syndrome: an indication for aggressive treatment.

McCune-Albright syndrome consists of the triad polyostotic fibrous dysplasia, café-au-lait spots and autonomous hyperfunction of one or more endocrine systems. The most frequent neurological complication of craniofacial fibrous dysplasia is visual loss. We describe a 17-y-old boy with McCune-Albright syndrome and acute loss of vision in the left eye caused by encroachment of the left optic nerve by fibrous dysplastic lesions. Neurosurgical intervention improved left eye vision. Aggressive intervention is indicated in cases of acute visual loss in patients with craniofacial fibrous dysplasia. This is supported by a review of other reported cases.

Adolescent↗