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PubMedDate not supplied

Chest wall tumors: radiologic findings and pathologic correlation: part 1. Benign tumors.

Benign chest wall tumors are uncommon lesions that originate from blood vessels, nerves, bone, cartilage, or fat. Chest radiography is an important technique for evaluation of such tumors, especially those that originate from bone, because it can depict mineralization and thus indicate the diagnosis. Computed tomography (CT) and magnetic resonance (MR) imaging are helpful in further delineating the location and extent of the tumor and in identifying tumor tissues and types. Although the radiologic manifestations of benign and malignant chest wall tumors frequently overlap, differences in characteristic location and appearance occasionally allow a differential diagnosis to be made with confidence. Such features include the presence of mature fat tissue with little or no septation (lipoma), the presence of phleboliths and characteristic vascular enhancement (cavernous hemangioma), evidence of neural origin combined with a targetlike appearance on MR images (neurofibroma), well-defined continuity of cortical and medullary bone with the site of origin (osteochondroma), or fusiform expansion and ground-glass matrix (fibrous dysplasia). Both aneurysmal bone cysts and giant cell tumors typically manifest as expansile osteolytic lesions and occasionally show fluid-fluid levels suggestive of diagnosis.

Adult↗
PubMed2006

Long-term outcome of optic nerve encasement and optic nerve decompression in patients with fibrous dysplasia: risk factors for blindness and safety of observation.

OBJECTIVE: Fibrous dysplasia (FD) of bone may occur solely as a skeletal condition or it may occur in association with extraskeletal manifestations, including growth hormone (GH) excess. Uncertainty exists as to the management of FD involving the optic nerves. In an effort to clarify management, the authors studied a large population of patients. METHODS: One hundred four patients underwent an evaluation that included review of records, endocrine testing, cranial computed tomography, and neuro-ophthalmological examination. RESULTS: Ninety-one of 104 patients had craniofacial FD; complete records were available for 87 patients (174 nerves). Seventeen percent of the optic nerves were less than 50% encased, 22% were 50 to 99% encased, and 61% were 100% encased. Twelve percent of the nerves that were 100% encased showed evidence of optic neuropathy, but 88% did not. The group with optic neuropathy was not older than the group without. Patients with GH excess were significantly more likely to have nerves that were 100% encased (relative risk, 4.1; 95% confidence interval, 1.5-11.1; P = 0.0017) and to have optic neuropathy (relative risk, 3.8; 95% confidence interval, 2.0-7.1; P = 0.0019). Six prophylactic optic nerve decompressions were performed; in five patients, vision was stable after surgery, and one patient was blind after surgery. Thirteen interventional optic nerve decompression procedures were performed; six of the 13 patients showed some improvement and seven of the 13 showed no improvement or worsened vision. CONCLUSION: The vast majority of optic nerves encased with FD do not exhibit symptoms of optic neuropathy and seem to be stable over time. GH excess is associated with increased risk of nerve encasement and optic neuropathy. Patients with craniofacial FD should be screened for GH excess, and optic nerve decompression should be performed only when there is objective evidence of progressive optic neuropathy.

Adolescent↗
PubMed1989

[Fibro-osseous lesions of the jaw and facial bones: a clinico-histologic-radiologic study of 138 cases].

A clinical-histologic-radiologic study of 138 cases of fibro-osseous lesions of the jaw and facial bones is presented. In this series, all were monostotic lesions except 8 polyostotic fibrous dysplasia. In the 130 monostotic lesions, 59 occurred in maxilla, 66 in mandible, 4 in zygoma and one in ethmoid bone. Clinically, a painless enlargement of the involved bone was the main sign. Histologically, these lesions were composed of proliferating fibrous connective tissues and various amounts of calcified tissues, but different lesion has different feature. Radiologically, fibrous dysplasia was characterized by lesions with diffuse blending. The other three tumors including ossifying fibroma, cementifying fibroma and fibro-osteoma were all represented by lesions with well delineated borders. In this article, Diagnosis of the fibro-osseous lesions and some questions about fibro-osteoma of the jaw and facial bones and osteofibrous dysplasia of long bones are discussed.

Adolescent↗
PubMedDate not supplied

[Leontiasis ossea: a symptom...!].

The keyword "leontiasis ossea" has no diagnostic significance. It is only a description of a symptom of bone disease with a broad variability such as Paget's disease, fibrous dysplasia, hyperparathyroïdism and condensing osteopathies. (Pyle's disease, or cranio-diaphyseal dysplasia.) The first case presented shows advanced Paget's disease with radiographic signs of maxillary bone pathology. A tooth extraction in this patient was complicated by a severe healing problem. Sequestration occurred after two years. Healing was only obtained after removal of the sequestrum. The authors believe that the healing problem was directly related to Paget's disease of maxillary bone. The second case represents a patient with hyperparathyroïdism, showing an unusual facial swelling as only physical symptom of the disease. The problem of interpretation of biopsymaterial and the occurrence of osteosclerotic appearence on X-ray are discussed. The authors suggest that a complete examination (X-ray of the skeleton, blood and urine parameters, and functionel tests) should be performed to put forward a right diagnosis in the patient, presenting leontiasis ossea.

Adult↗
PubMed2001

[Helical CT and diagnostic evaluation of cranio-facial fibrous dysplasia].

PURPOSE: To evaluate the role of helical CT in the evaluation of skull abnormalities seen in fibrous dysplasia. MATERIALS AND METHODS: Prospective study on 6 cases with cranio-facial fibrous dysplasia (3 males and 3 females) aged 8 to 59 years old, evaluated with plain radiographs, helical CT and, in 1 case, MRI. Helical CT was performed in 3 cases on a CT Twin scan (Elscint), in 3 cases on a Somatom Plus scan (Siemens). Multiplanar reconstructions and 3D reconstructions were done in all cases. 4 cases had a histopathological confirmation of fibrous dysplasia lesions by bone-biopsy (3 cases) or surgical biopsy (1 case). RESULTS: The 6 cases included sclerotic form in 3 cases, cystic variety in 1 case, polyostotic variety in 1 case, mixed form in 1 case. Topographic localizations of fibrous dysplasia were skull base involvement in 1 case, multizonal skull involvement in 4 cases, maxillo-mandibulary involvement (cherubism) in 1 case. CONCLUSION: Helical CT is an optimal method to evaluate the skull lesions of fibrous dysplasia. The review of source images must always be done. 3-dimensional bone reconstructions are better to visualize the anterior cranial vault and skull base dysmorphy. Helical acquisition is a compromise in case by case between the dose of irradiation and the quality of source images.

Adolescent↗
PubMed2006

Prevalence, extension and characteristics of fluid-fluid levels in bone and soft tissue tumors.

The purpose of this study was to determine the prevalence, extension and signal characteristics of fluid-fluid levels in a large series of 700 bone and 700 soft tissue tumors. Out of a multi-institutional database, MRI of 700 consecutive patients with a bone tumor and MRI of 700 consecutive patients with a soft tissue neoplasm were retrospectively reviewed for the presence of fluid-fluid levels. Extension (single, multiple and proportion of the lesion occupied by fluid-fluid levels) and signal characteristics on magnetic resonance imaging of fluid-fluid levels were determined. In all patients, pathologic correlation was available. Of 700 patients with a bone tumor, 19 (10 male and 9 female; mean age, 29 years) presented with a fluid-fluid level (prevalence 2.7%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included aneurysmal bone cyst (ten cases), fibrous dysplasia (two cases), osteoblastoma (one case), simple bone cyst (one case), telangiectatic osteosarcoma (one case), "brown tumor" (one case), chondroblastoma (one case) and giant cell tumor (two cases). Of 700 patients with a soft tissue tumor, 20 (9 males and 11 females; mean age, 34 years) presented with a fluid-fluid level (prevalence 2.9%). Multiple fluid-fluid levels occupying at least one half of the total volume of the lesion were found in the majority of patients. Diagnoses included cavernous hemangioma (12 cases), synovial sarcoma (3 cases), angiosarcoma (1 case), aneurysmal bone cyst of soft tissue (1 case), myxofibrosarcoma (1 case) and high-grade sarcoma "not otherwise specified" (2 cases). In our series, the largest reported in the literature to the best of our knowledge, the presence of fluid-fluid levels is a rare finding with a prevalence of 2.7 and 2.9% in bone and soft tissue tumors, respectively. Fluid-fluid levels remain a non-specific finding and can occur in a wide range of bone and soft tissue tumors, both benign and malignant. Therefore, they cannot be considered diagnostic of any particular type of tumor, and the diagnosis should be made on the basis of other radiological and clinical findings.

Adolescent↗
PubMed2004

Optic nerve disorders: role of canal and nerve sheath decompression surgery.

Optic nerve sheath decompression (ONSD) maintains a role in the management of visual loss complicating papilloedema in selected patients primarily with idiopathic intracranial hypertension. The evidence base for this intervention is reviewed and audit data on visual outcomes for patients with acute, chronic, and atrophic forms of papilloedema are contrasted. Optic canal decompression has a role in the management of compressive optic neuropathies complicating mass lesions arising from paranasal sinuses and intracranially and can be achieved by transethmoidal, transbasal, and open craniotomy routes. The evidence base supporting this intervention in traumatic optic neuropathy and in primary bone disease causing canal stenosis (in particular fibrous dysplasia) is reviewed where the indications are more controversial.

Decompression, Surgical↗
PubMedDate not supplied

[Fibrous dysplasia. The clinico-therapeutic picture and new data on its etiology. A review of the literature].

In this paper the authors examine current findings on the etiology of fibrous dysplasia. Particular emphasized is the role of the biochemical pathways and the genetic mutations occurring in the disease. In fact it is demonstrated that the McCune-Albright syndrome, a variant of fibrous dysplasia, is caused by the mutations of the GNAS 1 gene that codify for the alfa-subunit of the stimulatory guanine-nucleotide binding protein (G-protein). This mutation activates adenylate cyclase and consequently increases intracellular concentrations of cAMP. The increased signaling through the cAMP is believed to be responsible for the clinical characteristic of the McCune-Albright syndrome. The cap is translocated to the nucleus where a family of transcription factors is phosphorylated. This group of factors regulates the expression of CAp responsive genes: one of them, the c-fos proto-oncogene, produces a nuclear protein that binds with other proteins encoded by c-jun proto-oncogene, to form a transcription factor, AP-1. Several studies have shown an increase of c-fos mRNA in the bone lesions of patients with fibrous dysplasia. It suggests that the overexpression of c-fos may represent the first step in the carcinogenesis of bone sarcomas. Finally, attention is focused on the intravenous use of pamidronate as medical management in the treatment of the lesions that are not susceptible to surgical treatment.

Facial Bones↗
PubMedDate not supplied

[Differential diagnosis of fibrous osteodysplasia of the bones of the skull].

The work deals with the results of clinical and X-ray examination of 96 patients with fibrous dysplasia of the skull bones; 60 (62.5%) of these patients were sent to the clinic with an erroneous diagnosis. Sarcoma, osteoma, and osteoblastoclastoma were the most frequently recorded erroneous diagnosis. The condition of the end plates and the structure of the pathological focus are of leading importance in the differential diagnosis of the disease discussed. The pain syndrome, the soft-tissue component and the periosteal spicules and peaks are not encountered in fibrous osteodysplasia. The zone of the anomaly progresses only in the period of body formation. In adults osteodysplasia follows a stable course.

Adolescent↗
PubMedDate not supplied

[Bone tumors. Their prevalence].

1,200 cases of bone tumors were reviewed from the Orthopedic Hospital "Magdalena de las Salinas" between 1982 and 1989. 66.7% of them were benign tumors, 14.3% pseudotumoral lesions, 10.1% metastatic tumors, 8.6% primary malignant tumors, and 0.3% were malignant invasive tumors to bone. The most frequently found benign tumors were: osteochondroma, enchondroma and giant cell tumor; the most frequent pseudotumoral lesions were metaphyseal fibrous defects, solitary bone cysts and fibrous dysplasia; the most frequent primary malignant tumor was osteosarcoma. Prevalence, frequency, distribution, sex, most frequent ages, affected bones, multicentricity, and aggregated fractures, as well as the site of the primary lesion in metastatic and invading tumors, were similar to those reported in the classical series.

Age Factors↗
PubMed2005

[Craniofacial fibrous dysplasia: a case report].

Fibrous dysplasia is classified among benign bone tumors. It is a bone development anomaly characterized by hamartoma proliferation of fibrous tissue within the medullary bone, with secondary bony metaplasia, producing immature, newly formed and weakly calcified bone, without maturation of the osteoblast. It can be monostotic or polyostotic. The craniofacial localization occurs in 10%-25% of cases in monostotic forms and in 50% of cases in polyostotic forms. Fibrous dysplasia essentially affects children and young adults, with no sex preference. Its diagnosis is not always straightforward because the functional symptomology is often absent or not specific. Medical imagery is necessary and in difficult cases a bone biopsy. The major complication of the illness remains sarcomatous degeneration. Only symptomatic forms are treated. We report an observation of craniofacial fibrous dysplasia affecting a child and discovery of proptosis, with no other ocular or general functional signs.

Child↗