Wednesday, June 1, 2011

Fibroma ossificans

Osteofibroasa dysplasia or ossified fibroma is a benign fibrous tumor with local aggressive behavior. Location is most common in adults mandible. For children the most common location is the tibia, followed by other long bones. Fibroma ossificans appears in the first decade of life and presents clinically as a mass progressive painless.
Fibroma ossificans shows a distinctive radiological appearance.This lesion occurs in the cortex above the lithic or metaphysical tibia shaft and cause the anterior-posterior bulge. This well-circumscribed tumor looks fine multiloculat cortex and cause distortion.
Treatment includes fibroma ossificans in patients older conservative curettage. It requires a full pathological investigation for possible adamantinoame. Patients who still has open growth plates are placed under medical observation. If the tibia is recommended tijarea bulging occurs. No tumor metastasized.
Due to the high rate of recurrence of the lesion nonchirurgical recommend treatment until reaching skeletal maturity, at which point marginal resection and bone grafting can be performed without increased risk of recurrence. For patients of any age, surgical correction of associated deformities may be necessary.Surgery is indicated if the lesion is aggressive or if the patient suffers multiple pathological fractures. Resection of large portions of the lesion is not necessary because it increases succeptibilitatea recurrent fractures.
The natural ossified fibroma is unpredictable. The rate of progression varies from slow to fast and spontaneous resolution is possible. The continued growth of the lesion often occurs prior to skeletal maturity. In most cases moderate progression is followed by gradual improvement once they have reached skeletal maturity.
Pathogenesis and causes
Fibroma ossificans etiology and origin of tumor cells is unknown. It originated out of a fault ipotetizeaza fibrovasculara. He proposed a cause-effect relationship between fibroids and adamantinom ossificans based on a common causative factor, fibrovascular defect. According to this theory fibroma ossificans occurs through a fault in haversiene channels, while adamantinomul develop a defect secondary intramedullary vasculature. Etiologic factor has been suggested that blood flow deficit in the periosteum.
Signs and symptoms
Fibroids and adamantinomul ossificans shows similar clinical pictures, as well as radiological and pathological features.Although it may sometimes look adamantinomul osteogenic sarcoma, fibroma ossificans show no histological features of malignancy. There histological grade between fibroma ossificans, adamantinomul benign and malignant adamantinomului aggressive appearance that is found in adults. The latter osteoid production with cellular mitosis can make it part of osteogenic sarcoma and thus can progress to malignancy.
Adamantinomul is a malignant bone tumor, locally aggressive, histogenetica unknown origin. It is considered to be of epithelial origin. Localization is predominantly in tibial shaft but it may occur in the fibula and other long tubular bones. They are slow-growing tumors with limited propensity to metastasis and local recurrence, are controlled by local resection.Initial symptoms are indolent and nonspecific, depending on the location and extent of the disease. Insidious onset, slowly progressive nature. Patients tolerate symptoms for several years before the doctor. The lesions may be discovered incidentally on X-rays after the patient has suffered a trauma to the affected extremity. The accused may present with mild bone pain for a few months or few years. Most patients present with local swelling with or without pain.
Diagnosis
Imaging Studies:Radiographic fibroma ossificans appears as an intramedullary lesion of the bone with crushed glass appearance. Fibroma ossificans in children is the eccentric lesions, osteolytic and intracorticale. It is this expansion through the cortical surface area internal cortical sclerosis. Frequently observed the appearance of soap bubbles by multilocular lesion. extension into soft tissue is absent and periosteala reaction is rare, if not associated with a pathological fracture. Lesion size is variable. Usually affects the shaft, although damage was reported and metaphysics.Biopsy: clinicr because evolution and appearance of the fibroid osficicant radiological, diagnostic for children, biopsy is rarely indicated and should be avoided.Histological examination: Microscopic fibroma ossificans consists of trabecular bone spiculi irregular bordered by osteoblasts. These osteoblasts produce a ring around the center bone lamellar bone bulged. The tumor was positive for citokeratina. Adamantinomul and represent stages in the evolution of dysplasia osteofibroasa fibroma ossificans.The differential diagnosis is made with the following diseases: fibrous dysplasia, fibroids nonosificant, adamantinomul.
Treatment
Therapy fibroma ossificans:Noninterventional treatment is recommended until skeletal maturity.Recurrent pathological fractures in children can be a problem active. Use tijari pseudoartrozele tibial defects similar to those of the tibia to minimize pathological fractures. Fractures can be treated with immobilization and ghipsaj.The recommended treatment of the injury until reaching nonchirurgical skeletal maturity, at which point marginal resection and bone grafting can be performed without increased risk of recurrence. For patients of any age, surgical correction of associated deformities may be necessary. Surgery is indicated if the lesion is aggressive or if the patient suffers multiple pathological fractures. Resection of large portions of the lesion is not necessary because it increases succeptibilitatea recurrent fractures.Malignant transformation is very rare lesion.
Adamantinomului therapy:The tumor is resistant to radiation and chemotherapy has not been shown effective. Interventional treatment is standard and includes amputation or en bloc resection with wide margins and limb salvage. Wide margins of excision are associated with decreased risk of local recurrence. The survival rate is 82% at 10 years. Limb reconstruction can be done by osteogenesis, allograft, vascularized fibular autografts, autografts and osteoplasty nonvascularizate metal.
Prognosis:The natural ossified fibroma is unpredictable. The rate of progression varies from slow to fast and spontaneous resolution is possible. The disease may follow three paths: moderate progression, especially during the first 5-10 years of life, aggressive growth with marked bone deformation and spontaneous resolution. The continued growth of the lesion often occurs prior to skeletal maturity. In most cases moderate progression is followed by gradual improvement once they have reached skeletal maturity.

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