Cases reported "Sclerosis"

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1/79. Reconstructive treatment of sclerosing osteomyelitis of the entire femur of 30 years' duration with avoidance of segmental resection.

    We describe the successful operative treatment of a patient with chronic sclerosing osteomyelitis of the femur in which en bloc resection was avoided. Therapy consisted of combined endoscopic, computed tomography and bone scan-guided fenestration and intramedullary reaming, with removal of all sclerotic zones and normalization of the cortical thickness. An adequate supply of oxygen to the area was ensured by improved vascularisation and the application of hydrogen peroxide. At the medium term follow-up no recurrence was seen, and hip and knee function was normal.
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ranking = 1
keywords = bone
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2/79. Chronic sclerosing sialadenitis of the submandibular gland associated with idiopathic retroperitoneal fibrosis.

    We report a case of a 57-year-old man who developed a fibrosclerosing lesion in the submandibular gland and idiopathic retroperitoneal fibrosis (IRF) involving the unilateral periureteral region within a year. Both lesions were resected surgically because of the suspicion of neoplasm. Pathologic examination revealed similar histologic and immunohistochemical features for both lesions, namely, fibrosclerosis with prominent hyalinizing collagen bundles and proliferation of myofibroblastic cells, and a non-neoplastic reactive nature. There was infiltration by lymphocytes with prominent lymph follicles, plasma cells and macrophages. The histologic and immunohistochemical findings suggest that the two lesions were of a similar pathogenesis, which was possibly mediated by macrophages. We think that the present case may be an unusual form of multifocal fibrosclerosis. Although sialolithiasis is thought to be a major pathogenic factor for chronic sclerosing sialadenitis of the submandibular gland, the present case suggests that certain cases might have an etiology similar to IRF.
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ranking = 16.997561011928
keywords = macrophage
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3/79. Sclerotic bone metastases from sarcomatoid renal cell carcinoma.

    We present a case of sarcomatoid renal cell carcinoma with multiple sclerotic skeletal metastatic lesions. Renal cell carcinoma is frequently metastatic at presentation, with a high incidence of skeletal involvement, classically described as osteolytic. However, sclerotic or osteoblastic metastatic skeletal lesions from renal cell carcinoma are rare, with only two previous reports identified in the literature, neither of which involved the sarcomatoid variant of renal cell carcinoma. In our case the sclerotic metastases were characterized by bone scan, computed tomography (CT), magnetic resonance imaging (MRI), and histologic analysis.
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ranking = 5
keywords = bone
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4/79. In situ demonstration of parathyroid hormone-related protein mRNA in sclerosing hepatic carcinoma.

    A 69-year-old man had a hepatic tumour occupying the left and half of the right lobe, with portal vein thrombus. There were hypercalcaemia and hypophosphataemia with increased nephrogenous cyclic adenosine monophosphate; bone metastases were excluded. serum parathyroid hormone-related protein (PTHrP) was elevated, but no increase in intact parathyroid hormone (PTH) or vitamin D3 metabolites was found. At autopsy the histological features were typical of sclerosing hepatic carcinoma. By immunohistochemistry PTHrP was detected in cancer cell nests but not in the fibrous stroma. PTHrP transcripts were demonstrated by in situ hybridization using a polymerase chain reaction (PCR)-derived single-stranded dna probe. Tumour cells expressed AE1 and CA19-9 (markers for cholangioepithelium) and CEA (for bile canaliculi). Electron microscopy revealed microvilli on the apical surface, and secretory granules 100 nm in diameter were observed. These findings indicate that this case is one of cholangiocellular sclerosing hepatic carcinoma. The interaction between cancer and stromal cells may be the cause of PTHrP overexpression.
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ranking = 1
keywords = bone
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5/79. [11C](R)-PK11195 positron emission tomography imaging of activated microglia in vivo in Rasmussen's encephalitis.

    This study was designed to explore the feasibility of PET using [11C](R)-PK11195 as an in vivo marker of activated microglia/brain macrophages for the assessment of neuroinflammation in Rasmussen's encephalitis (RE). [11C](R)-PK11195 PET was carried out in four normal subjects, two patients with histologically confirmed RE, and three patients with clinically stable hippocampal sclerosis and low seizure frequency. Binding potential maps showing specific binding of [11C](R)-PK11195 were generated for each subject. Regional binding potential values were calculated for anatomically defined regions of interest after coregistration to and spatial transformation into the subjects' own MRI. In one patient with RE who underwent hemispherectomy, the resected, paraffin-embedded brain tissue was stained with an antibody (CR3/43) that labels activated human microglia. Whereas specific binding of [11C](R)-PK11195 in clinically stable hippocampal sclerosis was similar to that in normal brain, patients with RE showed a focal and diffuse increase in binding throughout the affected hemisphere. In RE, [11C](R)-PK11195 PET can reveal in vivo the characteristic, unilateral pattern known from postmortem neuropathologic study. PET imaging of activated microglia/brain macrophages offers a tool for investigation of a range of brain diseases where neuroinflammation is a component and in which conventional MRI does not unequivocally indicate an inflammatory tissue reaction. [11C](R)-PK11195 PET may help in the choice of appropriate biopsy sites and, further, may allow assessment of the efficacy of antiinflammatory disease-modifying treatment.
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ranking = 16.997561011928
keywords = macrophage
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6/79. Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

    rhabdomyosarcoma in adults represents a rare soft tissue neoplasm which is seen most frequently in its pleomorphic subtype in this age group. Very rarely, clear cell and spindle-cell variants have been reported. In this study we describe three cases of rhabdomyosarcoma in adult patients, characterised by prominent hyaline sclerosis and a pseudovascular growth pattern. All cases were identified in the consultation files of one of the authors and routinely processed. Immunohistochemical studies were performed on paraffin sections with the alkaline phosphatase-antialkaline phosphatase method. The patients, two women and one man, were 40, 41, and 56 years old. One developed a deep-seated soft tissue mass in the left lower leg, and one, a tumour of the left upper jaw. In one patient a bone tumour in the proximal body of the sacrum without extension into soft tissues was seen. The patients were treated by wide excision, piecemeal excision and incomplete excision in one case each; additional radiotherapy was performed in all three cases, and chemotherapy in two patients. In one patient multiple pulmonary metastases were noted, which showed progression despite systemic chemotherapy. Histologically, the neoplasms were composed of round/polygonal and spindle-shaped tumour cells including typical rhabdomyoblasts. In all cases a pseudovascular pattern and prominent hyaline sclerosis of the intercellular matrix was seen. Immunohistochemically, tumour cells stained positively for desmin and muscle actin (HHF35) and also for markers of striated muscle differentiation (myogenin, MyoD1, fast myosin). In this paper an unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.
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ranking = 1
keywords = bone
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7/79. tooth eruption in a patient with craniometaphyseal dysplasia: case report.

    Craniometaphyseal dysplasia (CMD) is a very rare genetic disorder of bone remodeling caused by osteoclast dysfunction. The clinical and radiographical features of oral findings are presented in a sporadic case of CMD in a child (age 10 years, 7 months). An intraoral examination showed severe malocclusions, including anterior crossbite and deep bite. Furthermore, a radiographic examination showed increased radiopacity of the maxilla and mandibular bones due to hyperostosis and sclerosis of the jaw. There was no root resorption of the canines or molars in the primary dentition, although root formation of the permanent teeth was proceeding. Dental age was calculated to be approximately 1 year, 4 months younger than his chronological age. The eruption speed of the permanent lateral incisors after the gingival emergence was shown to be within normal values, and we discuss whether the canines and premolars in the permanent dentition could erupt or not.
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ranking = 2
keywords = bone
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8/79. Bone dysplasia sclerosteosis results from loss of the SOST gene product, a novel cystine knot-containing protein.

    Sclerosteosis is an autosomal recessive sclerosing bone dysplasia characterized by progressive skeletal overgrowth. The majority of affected individuals have been reported in the Afrikaner population of south africa, where a high incidence of the disorder occurs as a result of a founder effect. Homozygosity mapping in Afrikaner families along with analysis of historical recombinants localized sclerosteosis to an interval of approximately 2 cM between the loci D17S1787 and D17S930 on chromosome 17q12-q21. Here we report two independent mutations in a novel gene, termed "SOST." Affected Afrikaners carry a nonsense mutation near the amino terminus of the encoded protein, whereas an unrelated affected person of Senegalese origin carries a splicing mutation within the single intron of the gene. The SOST gene encodes a protein that shares similarity with a class of cystine knot-containing factors including dan, cerberus, gremlin, prdc, and caronte. The specific and progressive effect on bone formation observed in individuals affected with sclerosteosis, along with the data presented in this study, together suggest that the SOST gene encodes an important new regulator of bone homeostasis.
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ranking = 3
keywords = bone
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9/79. Aneurysmal bone cyst of the sphenoid sinus.

    Aneurysmal bone cysts of the sphenoid sinus are very rare, with only six cases described in the literature. We present a case of an aneurysmal bone cyst of the sphenoid sinus with associated fibrous dysplasia in which the radiological findings had some features of a mucocoele. We discuss the differences in pathogenesis, clinical presentation and radiological appearances between these two lesions, and propose a simple drainage procedure as an effective modality of treatment.
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ranking = 6
keywords = bone
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10/79. Therapeutic possibilities of long-term roxithromycin treatment for chronic diffuse sclerosing osteomyelitis of the mandible.

    The clinical efficacy of long-term roxithromycin treatment was examined objectively in nine patients with chronic diffuse sclerosing osteomyelitis of the mandible. roxithromycin was administered orally at a dose of 300 mg/day for between 68 days and 66 months. In seven of the nine cases (77.8%), the symptoms disappeared 1-12 months after the start of therapy. radiography showed that osteolytic changes (evident from 'moth-eaten' appearance of bone) had improved but that osteosclerosis had persisted or become more predominant by the end of therapy. Therefore, the optimum duration of treatment should be decided according to the amelioration of symptoms along with the disappearance of osteolytic findings in radiographs. Diarrhoea and stomach discomfort occurred in one case, and liver dysfunction in another, but these adverse reactions were relatively mild. The mechanism of action of roxithromycin in this study is not yet fully understood, but our results indicate that long-term roxithromycin treatment may be useful for diffuse sclerosing osteomyelitis of the mandible and should be attempted before surgical treatment is considered.
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ranking = 1
keywords = bone
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