Polyostotic Fibrous Dysplasia with Epiphyseal Involvement in Long Bones: A Case Report
An ossifying fibroma is a poorly defined proliferative, fibro-osseous, tumor-like, Osteofibrous dysplasia is almost always seen only in the tibia and fibula and .. until the ends of the incision meet on the midline caudal to the central incisors. Fibrous dysplasia is a common benign skeletal lesion that may involve one bone (monostotic) or multiple . into immunocompromised mice, and induced dysplastic bone production. This in vivo totic disease, met the inclusion criteria of the study. Seventy-one the tibia, in eight; the pelvis, in four; the humerus, in two; and. Skipping in MET Cause Osteofibrous Dysplasia. Mary J. Gray,1,23 cortex, almost exclusively of the tibia and fibula. (Figure 1A), although.
This histologic progression corresponds with the clinical and radiographic resolution of the lesions summary by Gray et al. Hunter and Jarvis noted that there may be a relationship between osteofibrous dysplasia and adamantinoma of long bonesalthough the latter condition usually presents at a later age.
These examinations demonstrated the syndrome of congenital bowing of the tibia, fibular hypoplasia, and pectus excavatum transmitted as an autosomal dominant trait.
One individual also had apparently healed congenital pseudarthrosis of the ulna. Affected members were observed in 3 generations and 5 sibships with 2 instances of male-to-male transmission. The first was a girl noted to have nontender bowing of her left tibia at birth.
Radiographs revealed metaphyseal-diaphyseal lucent defects in the proximal tibia bilaterally, with anterior bowing and periosteal reaction of the left tibia. At 8 months of age, she presented with a pathologic fracture of the left tibia; tissue removed from the lesion was consistent with osteofibrous dysplasia.
Osteofibrous dysplasia of the tibia and fibula.
The second patient was a boy who was noted to have right anterior tibial swelling at age 2 years, at which time x-rays showed cortex-based lucencies in both tibias.
By age 4 years, the lesions in both tibias had grown; biopsy of the larger lesion on the left, through which he subsequently developed a fracture, showed osteofibrous dysplasia. Hunter and Jarvis could find only 2 reported cases of osteofibrous dysplasia in which tibial involvement was reported as bilateral.
They described 2 brothers, 1 with bilateral and 1 with probable bilateral tibial involvement, and a girl from an unrelated family with bilateral tibial involvement. In 1 of the brothers, onset was at the age of 6 years, when swelling over the right leg was noted; in the other brother, a 'small bump' over the right anterior tibia was noted at the age of 4 years.
The girl had presentation at the age of 8 years with bilateral shin pain and a bumpy prominence over the anterior aspect of the midshaft of both tibias. All 6 had pathologic fractures of the tibia or fibula in childhood, but none had femoral lesions or pathologic fractures in the upper extremities. X-rays showed well-demarcated lytic lesions, not limited to the cortex of the bone, with surrounding sclerosis.
Histologic analysis of lesional tissue from one of the patients revealed a fibrous stroma with spicules of osseous trabeculae, rimmed with osteoblasts. In the 3 patients for whom x-rays obtained in adulthood were available, irregularities in the medullary canal of the fibula and thickening of the fibular diaphyseal cortex were observed, but there was no evidence of persistent disease.
Radiographs of 10 clinically unaffected relatives did not reveal pathologic lesions in the tibia or femur, and there was no evidence of a previous fracture. Histologic staining of the cells rimming the lesions revealed markers of osteoblastic differentiation such as osterix SP7; and alkaline phosphatase see Osteoclasts staining for tartrate-resistant acid phosphatase ACP5; were also prominent at the periphery of the lesions. In the present study, we describe a rare case of polyostotic FD with unilateral multiple epiphyseal lesions arising in long bones with the growth plates.
Case Presentation A year-old girl was referred to our department with dull pain in the right knee while in motion, which she had noticed approximately one month before her initial visit. There was no pain at rest, swelling, or tenderness in the lower right limb.
Osteofibrous dysplasia of the tibia and fibula.
The range of motion was not limited in the right hip, the right knee, or the right ankle. There was no history of endocrine dysfunction including precocious puberty, and no cutaneous abnormality was found. Radiographs of the right lower limb revealed multiple radiolucent lesions with sclerotic contours in the diaphyses of the femur the tibia, the patella, the pelvis, and the tarsal bones.
Characteristic lesions were also found in the distal epiphysis of the femur, and the proximal and distal epiphyses of the tibia. The growth plates in both the femora and the tibia had not been closed.
The lesions in the diaphyses of the right femur and the right tibia were distended causing the surrounding cortex to thin Figure 1. Technetium bone scintigram showed increased uptake in the diaphyses of the right femur and the right tibia and the right pelvis and around the bilateral knees and ankles Figure 2.
On magnetic resonance imaging MRIthe epiphyseal lesions of the distal femur and the proximal tibia showed a low signal intensity on T1-weighted images and a high signal intensity on fat-suppressed T2-weighted images, and the lesions were markedly enhanced by gadolinium Figure 3.
OMIM Entry - # - OSTEOFIBROUS DYSPLASIA, SUSCEPTIBILITY TO; OSFD
Plain radiographs of the right leg. Radiolucent lesions with sclerotic contours in the diaphyses of the right femur a and the right tibia b and the epiphyses of the right knee c. Abnormal uptakes are shown in the diaphyses of the right femur and the right tibia and around the bilateral knees and ankles.
Magnetic resonance imaging MRI of the lesions in the distal epiphysis of the right femur and the proximal epiphysis of the right tibia. All lesions show a low signal intensity on a T1-weighted image a and a high signal intensity on a fat-suppressed T2-weighted image b.
The lesions were strongly enhanced by gadolinium c T1-weighted images enhanced by gadolinium. On the basis of radiological findings, benign bone tumors, such as polyostotic FD or nonossifying fibroma, were suspected.
Two months after the first surgery, surgeries for the lesions in the diaphysis of the right tibia and in the epiphysis of the right femur were performed. As FD does not usually arise in the epiphysis, an open biopsy alone was performed for the distal epiphyseal lesion of the right femur in order to make a definitive diagnosis. The lesion in the epiphysis of the distal femur was histologically composed of haphazard bony trabeculae and fibrous tissue stroma containing benign spindle cells.
A histological diagnosis of FD was made. Three months after the second surgery, the patient fell and sustained a fracture in the proximal diaphysis of the right femur and underwent osteosynthesis using an intramedullary nail. There was no recurrence in the lesions in the diaphyses of the right femur and the right tibia, and the epiphyseal lesions of the distal femur and the proximal tibia showed no increase in size radiographically at the final follow-up examination Figure 5.
Microscopic findings of the excised lesion in the distal epiphysis of the right femur.Knee pain Taping: Fibular head subluxation = Antero/postero-Superior
Spindle cells grow in storiform pattern around haphazard bony trabeculae and fibrous tissue stroma. There is lack of osteoblast rimming surrounding the trabeculae. Plain radiographs of the right knee five years after the first surgery. Discussion FD is an uncommon, but well-known benign skeletal disorder, which is considered to be a pathological condition as a result of developmental failure in the remodeling of immature bone to mature lamellar bone and of inappropriate bone realignment in response to a mechanical stress.
The disorder of bone maturation leaves a mass of immature isolated trabeculae woven bone in dysplastic fibrous tissue [ 7 ]. The true incidence of FD is unclear.
FD can occur as monostotic or polyostotic lesions. The latter form of FD is rarely accompanied by endocrine dysfunction, including precocious puberty and cafe-au-lait spots such as characteristic cutaneous abnormalities, known as McCune-Albright syndrome.