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PubMed2005

Bone scintigraphy in common tumors with osteolytic components.

Osteolytic lesions are frequently encountered in clinical practice. Radionuclide bone scans with technetium-99m-labeled diphosphonates are often performed in the evaluation of both solitary and multiple osteolytic lesions. In this pictorial review, we critically evaluate the current role of bone scan in common osteolytic tumors including aneurysmal bone cyst, simple bone cyst, fibrous dysplasia, nonossifying fibroma, giant cell tumor, eosinophilic granuloma, enchondroma, chondrosarcoma, osteosarcoma, Ewing sarcoma, myeloma, and metastases. The merits and limitations of bone scanning are emphasized.

Aged↗
PubMed1998

Craniofacial fibrous dysplasia with rapidly increasing proptosis due to a mucocele behind the globe.

A 16-year-old male with fibrous dysplasia, in which rapidly increasing proptosis of the left eye was induced by a mucocele behind the globe, is reported. The mucocele was removed and the expanded bone was completely recontoured back to normal dimensions with the aid of an acrylic skull replica fabricated preoperatively by laser lithography on the basis of computed tomographic data. The result was satisfactory and no recurrence of bony swelling or proptosis has been detected for 2 years and 9 months.

Adolescent↗
PubMed2006

Fibrous dysplasia of the clivus with a second T8 bone lesion: case report.

BACKGROUND: We present a rare case of a probably poliostotic fibrous dysplasia of the clivus in a young woman with a clival and vertebral involvement. We also compare our case with data reported in tables that include all cases of clival fibrous dysplasia present in literature. CASE DESCRIPTION: Thirty-year-old women presented with headache. We reviewed radiological and clinical literature before surgery. It was possible to perform a preoperative diagnosis of fibrous dysplasia of the clivus so it was possible to adopt a minimally invasive surgical approach on the clivus. The diagnosis of fibrous dysplasia of the clivus was confirmed after the operation. As the patient was asymptomatic, we did not operate on the vertebral lesion which was stable after 1 year. CONCLUSION: We draw the conclusion that it is possible to obtain a correct preoperative diagnosis of fibrous dysplasia based on magnetic resonance imaging and computed tomographic scans; this makes it possible to avoid major surgical operations. The reported case is particularly interesting because apart from the clival fibrous dysplasia, a vertebral involvement of the same type can be assumed; this association has never been reported in literature.

Adult↗
PubMed1997

[Value of a synthetic osseous model obtained by stereo-lithography for preoperative planning. Correction of a complex femoral deformity caused by fibrous dysplasia].

PURPOSE OF THE STUDY: The authors investigated the application of life-sized bone models obtained by stereolithography in orthopaedics. MATERIALS: The method was applied to planify correction of a severe femoral bone deformity secondary to fibrous dysplasia in a 27 years old man. This deformity was responsible for abnormal hip range of motion 70 degrees/-10 degrees (flexion-extension), -20 degrees/60 degrees (abduction-adduction), -30 degrees/60 degrees (external-internal rotation), and restricted walking ability because of lack of abduction and external rotation. A "shepherd-cross" deformity was identified on X-rays. A correction osteotomy was considered but we were unable to planify the angle of osteotomy on plain X-rays. CT scan identified 100 degrees of varus cervical deformity and 90 degrees of cervical antetorsion, but CT scan was helpless to choose the position for the osteosynthesis device. METHODS: 2D pictures obtained by CT scan were introduced and treated on a Silicon Graphics Indigo2 hardware. Mimics software authorized 2D and 3D views of bone which were separated of soft tissue by color separation process. CTM software authorized the 3D bone surface reconstruction (3D files). The 3D files were used to obtain life-sized bone model in 6 hours by stereolithographic process (scale 1/1). RESULTS: We planified a 70 degrees valgus and 40 degrees derotation and chose the best location for osteosynthesis device considering the fibrous dysplasia (best location was the posterior and superior aspect of the femoral neck). The planified osteotomy was performed and we obtained the stability of a nail-plate in the femoral neck. During surgery, we observed the bone model and the deformed femur had the same shape. Likewise, the model strongly indicated the inside bone structure (ie distribution of fibrous dysplasia tissue). Bone healing was obtained after 5 months with improvement of range of motion [(70/0) (20/30) (30/20)]. Histologic examination diagnosed fibrous dysplasia without malignancy features. CONCLUSION: Computer-generated life-sized bone models are available from computer tomographic data by means of stereolithographic process. This technic was helpful to improve planification of this complex proximal femoral osteotomy. Obtaining life-sized bone models could improve preoperative planning in case of multidirectional deformity, unusual site for osteotomy, or severe deformity impairing the choice for fixation device or its position into bone extremities. The indications for this method should be restricted to unusual and severe bone deformities, with inadequate preoperative assessment by standard X-rays or CT scans. Likewise, this method could be indicated for preoperative planning of technically demanding osteotomies such as oblique plane.

Adult↗
PubMed1999

Quantitative analysis of the plain radiographic appearance of unicameral bone cysts.

RATIONALE AND OBJECTIVES: The authors determine the features that distinguish the radiographic appearance of unicameral bone cysts (UBC) from other solitary lesions of bone and to develop an ordered differential of similar-appearing lesions. MATERIALS AND METHODS: Seven hundred nine cases of solitary bone lesions, including 40 UBCs, were analyzed according to demographic, anatomic, and plain radiographic features. Vector analysis of both classic and new groups of features was performed to determine the sensitivity and specificity of radiographs for UBC and an ordered differential based on radiographic features. RESULTS: The features of the UBCs in this study compared favorably with those in the literature. The male:female ratio was 3.3:1, with an average age of 15 +/- 13 years. Age ranges and lesion diameters were similar for both sexes. Thirty-three of the 40 (83%) UBCs were in long bones, and seven were in the pelvis or calcaneus. All 40 UBCs were geographic, medullary, and lytic. None had an associated soft-tissue mass. Fifty-five percent had pathologic fractures, and 10% had fallen fragment signs. Ninety-eight percent had no cortical break, and 88% had well-defined margins. CONCLUSION: Classic descriptions of UBCs can be refined to improve sensitivity without sacrificing specificity. The best vector in this study had a sensitivity of 80% and a specificity of 93% and included these radiographic features: metaphyseal, diaphyseal, or flat bone location, geographic, lytic, medullary-based, no matrix, no satellite lesions, no subarticular extension, no soft-tissue mass, no cortical break, and central location in long bones. An ordered differential diagnosis, starting with the most likely lesion, includes enchondroma, aneurysmal bone cyst, fibrous dysplasia, nonossifying fibroma, Brodie abscess, and chondrosarcoma.

Adolescent↗
PubMedDate not supplied

Wide resection of primary tumors of bone and replacement with massive bone grafts: an improved technique for transplanting allogeneic bone grafts.

The treatment by wide resection of 104 local aggressive and low grade malignant bone tumors is presented with results after follow-up for 5.0 years on the average. The series comprises 29 giant cell tumors, most of them malignant or premalignant, 25 cartilaginous tumors (12 verified chondrosarcomas and 13 chondromas suspect of malignancy), 14 cases of sarcoma, 6 of fibrosarcoma, 4 parosseal sarcomas, 3 osteosarcomas and one chordoma. The remaining 36 cases include: aneurysmal bone cysts, fibrous dysplasia, chondroblastomas, osteoblastomas, giant chondromyxoid fibroma, and hemangiomas. After resection of the affected area, the resulting defect was replaced with massive autologous or allogeneic bone or osteoarticular graft in altogether 68 cases (autologous bone transplant in 61 cases and allograft in 7). A metallic endoprosthesis was used in replacement in 8 cases. No replacement of the defect was needed in 28 cases. The technique of the allogeneic bone transplantations consisted of massive bone or bone cartilage grafts which had been stored at a low temperature, and firm osteosynthesis. In every case, allogeneic (stored) bone was supplemented with autologous cancellous bone around the juncture of graft and host bone. This supplement proved to be important for success of the operation. The overall results were good in 81% and fair in 11%. Five cases had to be amputated and 3 patients were lost to follow-up, a possible total of 8% poor results. Extensive resection with massive bone grafting is a worthwhile approach in the treatment of selected cases of aggressive and low grade malignant bone tumors.

Adolescent↗
PubMed2006

Leontiasis ossea and post traumatic cervical cord contusion in polyostotic fibrous dysplasia.

Leontiasis ossea (leonine facies) or cervical canal stenosis are rare complications of polyostotic fibrous dysplasia (PFD). This case report documents dramatic leontiasis ossea in PFD as well as post traumatic cervical cord contusion due to hyperextension injury in a patient with generalized PFD involving the cranio-facial bones, axial skeleton and entire spine with secondary cervical canal stenosis. Cervical cord contusion has not been reported earlier in PFD.

Accidental Falls↗
PubMed1976

Alopecia with fibrous dysplasia and osteomas of skin. A sign of polyostotic fibrous dysplasia.

Localized alopecia of the scalp proved to be the cutaneous sign of underlying polyostotic fibrous dysplasia (Albright disease). Histologically, the hair follicles had been replaced by a strange coil of fibrous tissue. Some of these follicular sites showed ossification so prominent as to be evident in soft tissue reoentgenograms. In this patient, the skin itself showed a fibrous dysplasia analogous to that which has always been identified as occurring within the bones in Albright disease. In her skin, it is the hair follicle that is the site of this dysplasia. The appearance of ossification in the skin and fibrous dysplasia in the bone would seem to demonstrate a delicate equilibrial balance between fibrous and osteal tissue in organs of a common mesenchymal origin. Localized connective tissue stromal dysplasia is believed to play a role in the pathogenesis of not only the bony, but aslo the pigmentary and endocrine abnormalities of the disease.

Alopecia↗
PubMed2001

Fibrous dysplasia involving the skull base and temporal bone.

OBJECTIVE: To gain a broader appreciation of the clinical presentation, operative treatment, and outcome of patients with fibrous dysplasia involving the skull base. DESIGN: Retrospective review of a clinical case series. SETTING: A single tertiary academic medical center. PATIENTS: Twenty-one patients with histopathologically confirmed fibrous dysplasia involving the skull base cared for over a 15-year-period (1983-1998). MAIN OUTCOME MEASURES: Clinical and radiographic location of the fibrous dysplasia lesions within the skull base, clinical presentation, surgical intervention, and clinical outcome were tabulated for each patient. RESULTS: The ethmoids were most commonly involved (71%), followed by the sphenoid (43%), frontal (33%), maxilla (29%), temporal (24%), parietal (14%), and occipital (5%) bones. The most common presenting features included atypical facial pain and headache, complaints referable to the sinuses, proptosis and diplopia, hearing loss, and facial numbness. Surgical treatment, guided by clinical presentation, ranged from simple biopsy with conservative follow-up to craniofacial resection. CONCLUSIONS: Fibrous dysplasia can present in myriad ways within the skull base. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward. Surgery, particularly in such a challenging region as the skull base, should be reserved for patients with functional impairment or a cosmetic deformity. Because of the benign nature of the condition, the surgery itself should be relatively conservative, with the primary goal being preservation of existing function.

Adolescent↗
PubMed2003

Expression of VEGF and MMP-9 in giant cell tumor of bone and other osteolytic lesions.

This study aims to investigate the expression of vascular endothelial growth factor (VEGF) and matrix metalloproteinase-9 (MMP-9) in giant cell tumor of bone (GCT) and other osteolytic lesions in bone. By using semi-quantitative RT-PCR, we showed that three major isoforms of VEGF (121, 165 and 189) were expressed in GCTs, with isoform 121 being the most abundant. The expression levels of VEGF and MMP-9 mRNA were significantly higher in advanced GCTs (stage II/III) than in stage I GCTs. We further elucidated the cellular localization of VEGF and MMP-9 gene transcripts in GCT and other osteolytic lesions using an in situ hybridization assay. The results showed that stromal tumor cells and osteoclast-like giant cells of GCT, fibrous stromal cells in anuerysmal bone cysts and fibrous dysplasia, and Langerhans-type giant cells as well as histocytes in eosinophillic granuloma, were all strongly positive for VEGF and MMP-9 mRNA expression. In a prospective study, we performed VEGF and MMP-9 immuno-staining on paraffin sections of pathological tissues harvested from 48 patients (14 GCT, 10 anuerysmal bone cysts, 10 eosinophillic granuloma, 4 fibrous dysplasia, 2 simple bone cyst, 2 osteomyelitis and 6 patients with fractured femoral head as control). The results showed that the differences in VEGF and MMP-9 expression between Stage I and other advanced Stages (II, III and recurrent) were highly significant (p<0.001), with advanced stages showing a higher mean expression. The difference between recurrent and Stage II and III lesions, was also statistically significant (p=0.03 for VEGF, and p=0.01 for MMP-9 expression), with recurrent lesions showing a higher mean expression of both VEGF and MMP-9. In conclusion, VEGF and MMP-9 expression in osteolytic lesions of bone co-relates well with the extent of bone destruction and local recurrence. Their expression may therefore provide some prognostic indication of the possible aggressive behavior of the underlying pathology.

Bone Cysts↗
PubMed1977

Fibrous dysplasia.

An overview of the spectrum and natural history of fibrous dysplasia is an essential prerequisite of management. Current medical interest has focused upon the intriguing pathogenesis of the bone lesions, the endocrinopathies, the cutaneous pigmentation, and malignant transformation. The mundane aspects of diagnosis and the complications of a soft bone disorder reside with the clinical orthopedist. Diagnosis is straightforward with the aid of biopsy. Conservatism in the surgical treatment of monostotic lesions and experience in the care of fractures and deformity in polyostotic fibrous dysplasia constitute the lessons learned in a series of over 50 patients with fibrous dysplasia.

Adolescent↗
PubMed1999

Osteosarcoma in a patient with McCune-Albright syndrome and Mazabraud's syndrome.

Sarcomas infrequently develop in osseous sites of fibrous dysplasia. We report a patient with Mazabraud's syndrome (polyostotic fibrous dysplasia and soft tissue myxomas) complicated by the development of osteogenic sarcoma in a bone affected by fibrous dysplasia. This is the third case of osteosarcoma within the small population of reported patients with Mazabraud's syndrome. There may be an increased incidence of malignant transformation in these individuals' dysplastic bones above that associated with patients suffering from fibrous dysplasia alone.

Adult↗
PubMed2001

[Adamantinoma of the long bones: an anatomo-clinical review and its relationship with osteofibrous dysplasia].

Adamantinoma of long bones is a perplexing tumor for its histology as well as for its histogenesis. Recent progress has been made with ultrastructural and immunohistochemical works. The possible relationship with osteofibrous dysplasia is the subject of conflicting discussions and the potential link has implications for the diagnosis, prognosis, and treatment. Clinical aspects of adamantinomas are described: location, age, gender, symptoms, imaging, diagnosis, treatment, clinical course, prognosis. Histopathology is extensively covered for usual and peculiar forms. Immunohistochemical results are described and discussed. Data of electron microscopy, cytological findings, and DNA flow cytometry are also reported. The putative association with osteofibrous dysplasia is the subject of an extensive development with the report of the personal experience and works of the authors, and arguments for viewing osteofibrous dysplasia and "osteofibrous dysplasia-like" adamantinomas as precursor lesions of classic adamantinomas are favoured.

Bone Neoplasms↗