Cases reported "Bone Diseases"

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1/44. Treatment of giant aortic aneurysm with tracheal compression and sternal erosion without circulatory arrest.

    Treatment of huge aneurysms involving the ascending aorta and the aortic arch with compression of the surrounding structures represents a surgical challenge. The case of a patient affected by respiratory insufficiency and sternal erosion caused by chronic giant aortic aneurysm is reported. The use of a stepwise approach and selective cerebral arterial perfusion ensured successful operative management, avoiding circulatory arrest and enabling an expeditious postoperative recovery.
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2/44. Intraosseous xanthoma without lipid disorders. Case-report and literature review.

    A case of intraosseous xanthoma in a patient with a normal lipid profile is reported. Hyperlipidemia is present in most patients with xanthomas. Intraosseous xanthomas are rare, particularly in normolipidemic patients, in whom the presenting symptom is pain without skin lesions. A lytic lesion with a rim of sclerosis is seen on radiographs. histology shows foam cells, giant cells, and fibrosis. Intraosseous xanthoma is a benign tumor, and other diagnoses must be ruled out (histiocytosis X, Erdheim Chester disease, clear cell carcinoma metastasis). Surgical excision of the lesion is the elective treatment.
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3/44. Giant intradiploic epidermoid cysts of the skull. A report of eight cases.

    Giant intradiploic epidermoid cyst of the skull is a rare tumour. Only a small number of cases have been reported. Over a 16-year period, we treated eight cases of giant intradiploic epidermoid cyst of the skull. All cases were between 19 and 45 years of age, except one aged of 61 years. Five cases presented with local swellings, three patients came with infection and one had an extradural abscess. All the patients were operated upon after adequate preoperative care. patients with infection required appropriate antibiotics. Total or near total excision was carried out in all. One patient developed postoperative infection. So for those have been no recurrences. This appears to be the largest series of giant intradiploic epidermoid cyst of the skull so far reported.
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4/44. erdheim-chester disease with extensive marrow necrosis: a case report and literature review.

    erdheim-chester disease is a rare systemic disorder characterized by a fibrosing xanthogranulomatous infiltration of multiple organs. We report a case of erdheim-chester disease with diffuse necrosis leading to difficulty in making a prompt diagnosis. Radiologically, osteosclerotic lesions with osteolytic element involved metadiaphyses of both proximal tibia, and retroperitoneal infiltrations encasing both kidneys, both adrenals, and aorta were found. A biopsy of the tibia showed diffuse infiltration of foamy histiocytes, Touton-type giant cells, and fibroblastic cells associated with extensive coagulative necrosis. Immunohistochemically, foamy histiocytes were positive for CD68 and peanut agglutinin and negative for S-100 protein. A few Langerhans' cells, which were difficult to identify in hematoxylin-eosin stain, were highlighted by immunostain for S-100 protein. The patient received supportive therapy and was alive 1 1/2 years after diagnosis, with newly developed bilateral retrobulbar lesions and worsened heart failure.
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5/44. Intraosseous cholesterol granuloma of radius showing a lamellar structure on MR images: a case report.

    We report a case of an intraosseous cholesterol granuloma in the radius showing a lamellar structure on MRI. T1-weighted imaging revealed a central area of mildly increased signal intensity with a surrounding low-intensity layer. On T2-weighted images, the central lesion demonstrated low-intensity signals, and the surrounding area exhibited a lamellar structure with three layers of high, low, and high intensity from the inside. On histological examination, the central area was found to consist of cholesterine crystals embedded in erythrocytes, and in the surrounding lamellar structure collagenous tissue may have provided the low signals, with vessel- and giant cell-enriched structures present at both the inner and outer margins, presumably evoking the high-intensity signals. The present lesion may have been formed by chronic hemorrhage into a preexisting simple bone cyst.
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6/44. Giant cell reparative granuloma of the temporal bone: neuroradiological and immunohistochemical findings.

    A 38-year-old man presented with a giant cell reparative granuloma (GCRG) of the left temporal bone. Computed tomography showed a osteolytic middle cranial mass lesion. Magnetic resonance (MR) imaging showed the lesion as low intensity with heterogeneous enhancement by gadolinium on the T1-weighted images, and extremely low intensity on the T2-weighted images. angiography showed the lesion as highly vascular and fed by branches of the left external carotid artery. After preoperative embolization, gross total removal of the tumor was performed. The postoperative course was uneventful and no evidence of recurrence has been found for more than 4 years. Histological examination revealed GCRG with multinucleated giant cells in the fibrous background, abundant collagen bundles, hemosiderin deposits, and trabeculae of reactive bone. Some of the mononuclear stromal cells and almost all of the giant cells were positive for CD68, suggesting histiocytic differentiation. These histological features reflect the marked decrease in signal intensity on T2-weighted MR images.
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7/44. Classic adamantinoma with osteofibrous dysplasia-like foci and secondary aneurysmal bone cyst.

    adamantinoma, a rare bone lesion of the tibia and fibula, has two distinct variants, classic adamantinoma and osteofibrous dysplasia-like adamantinoma. Composite lesions have not been described. Aneurysmal bone cyst is a benign cystic lesion which may also occur in the tibia and fibula. We report an unusual case of classic adamantinoma with osteofibrous dysplasia-like areas and foci of secondary aneurysmal bone cyst with prominent giant cells. A lesion was diagnosed in a 17-year-old girl with a 14-year history of a slowly enlarging left tibial mass and increasing deformity. Pathologically, the predominant pattern was classic adamantinoma, with minor foci of osteofibrous dysplasia-like adamantinoma and areas of secondary aneurysmal bone cyst with abundant multinucleated giant cells. We report the clinical, radiologic, and pathologic features of this case, and summarize lesions associated with secondary aneurysmal bone cyst. To our knowledge, the association of adamantinoma with secondary aneurysmal bone cyst has not been previously reported.
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8/44. Giant cell reparative granuloma of the occipital bone.

    Giant cell reparative granuloma (GCRG) is a non-neoplastic fibrous lesion with unevenly distributed multinucleated giant cells, areas of osseous metaplasia and hemorrhage. The small bones of the hands and feet are the most common sites, followed by the vertebral bodies and craniofacial bones. In the craniofacial bones GCRG has been reported in the temporal bone, in the frontal bone and paranasal sinus. However, to the best of our knowledge no case has been reported in the occipital bone. We report on the imaging findings and pathological features of a GCRG of the occipital bone and discuss the differential diagnosis of this entity in this particular location, especially with giant cell tumor because of the therapeutic and prognostic implications.
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9/44. Multimodality imaging features of primary xanthoma of the calcaneus.

    Secondary xanthomatous features are histologically observed in various bone lesions, but primary xanthoma of bone is rare. We present a primary xanthoma of the right calcaneus in a 51-year-old woman who had no aberrant lipid metabolism. Roentgenograms showed a small osteolytic lesion in the calcaneal triangle, partially surrounded by bone sclerosis. Computed tomographic scans of the calcaneus showed multiple osteolytic areas, with an irregular trabecular pattern in the surrounding sclerotic bone. T1-weighted magnetic resonance images showed a lesion with central low signal intensity, surrounded by a peripheral ring with high signal intensity. The entire lesion showed high signal intensity on T2-weighted images, partially surrounded by areas with low signal intensity, concordant with reactive bone sclerosis. Histologically, the lesion consisted of numerous lipid-laden histiocytes arranged in sheets, scattered multinucleated giant cells and lymphocytes, and granulation tissues. There was no evidence of pre-existing lesions. Total excision of the tumor was curative.
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10/44. A case of giant expanding cephalhematoma: does the administration of blood coagulation factor XIII reverse symptoms?

    BACKGROUND: The mechanism of continuous massive hemorrhage into the subperiosteal space in children without coagulation defects is unknown. We report a case of giant expanding cephalhematoma reversed by the administration of blood coagulation factor XIII concentrate. METHOD: The patient was an 8-year-old boy with a history of minor head trauma who developed a giant expanding cephalhematoma with intraorbital extension. The laboratory data showed severe anemia, but a routine blood coagulation test showed no abnormalities except for a low factor XIII level. RESULT: The administration of factor XIII concentrate completely reversed the symptoms in 2 weeks. CONCLUSION: We speculate that one of the possible mechanisms of cephalhematoma expansion without blood coagulation defects might be acquired factor xiii deficiency from severe hemorrhage in a hematoma.
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