Cases reported "Skull Neoplasms"

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1/660. Cranio-orbital-temporal neurofibromatosis: are we treating the whole problem?

    Cranio-orbital-temporal neurofibromatosis is an uncommon subtype of neurofibromatosis 1 characterized by pulsatile exophthalmos, orbital neurofibromas, sphenoid wing dysplasia, expansion of the temporal fossa, and herniation of the temporal lobe into the orbit. The cause of the sphenoid wing dysplasia is uncertain. Reconstruction of the sphenoid defect, separating the orbit and cranial vault, has been problematic because of resorption of bone grafts. This reports illustrates one potential cause of the sphenoid defect and a possible cause of the bone graft resorption.
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2/660. hemangiopericytoma and temporal bone.

    hemangiopericytoma is a vascular tumor arising from the pericyts. The tumor involves usually the muskuloskeletal system and the skin. A hemangiopericytoma of the temporal bone is an extremely rare lesion. This paper describes a case of hemangiopericytoma located in temporal bone, its radiologic diagnosis and its therapeutic options as well.
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keywords = bone
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3/660. radium-induced malignant tumors of the mastoid and paranasal sinuses.

    In the records of 5,058 persons with therapeutic or occupational exposure to radium, 21 patients with carcinoma of the mastoid and 11 with malignant tumors of the paranasal sinuses were identified. Tumor induction times were 21-50 years for mastoid tumors (median, 33) and 19-52 years for paranasal sinus tumors (median, 34). Dosimetric data are given for the patients whose body burdens of radium have been measured. We found a high proportion of mucoepidermoid carcinoma, comprising 38% of the mastoid and 36% of the paranasal sinus tumors. Three patients had antecedent bone sarcoma at 20, 11, and 5 years, respectively, and a bone sarcoma was discovered at autopsy in a fourth patient. Radiographic changes in the mastoid and paranasal sinuses were similar to those seen in nonradium malignant tumors. More than 800 known persons exposed to radium before 1930 and another group of unknown size who received radium water or injections of radium from physicians are still alive and at risk of developing malignant tumors of the mastoid and paranasal sinuses.
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keywords = bone
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4/660. Angiomatous osteolysis of the skull vault.

    A case is reported of angiomatous osteolysis of the calvarium. This condition normally affects long bones and there is only one previous report in literature of massive osteolysis of the skull. The clinical, radiological and pathological implications of this condition are considered.
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keywords = bone
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5/660. Melanotic neuroectodermal tumour of infancy at the anterior fontanelle.

    We describe a 4-month-old girl presenting with a melanotic neuroectodermal tumour of infancy at the anterior fontanelle. According to the neuroimaging findings, this tumour was found to lie epidurally, adherent to the dura mater, with thickening of the adjacent frontal bone. The tumour was dense on CT, while MRI showed a major part of the tumour to be isointense with cerebral cortex on both T1- and T2-weighted images. The neuroimaging and clinical features are briefly discussed.
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keywords = bone
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6/660. giant cell tumors of the jugular foramen.

    PURPOSE: To review the diagnosis and treatment of giant cell tumors of the jugular foramen. MATERIALS AND methods: A typical case is reported. Symptoms, signs, and diagnostic studies are reviewed. Photomicrographs and angiographic studies showing the differences between these and glomus jugulare tumors are provided. A coherent approach to their management is presented. RESULTS: These hypervascular, traditionally radioresistant tumors may cause pulsatile tinnitus, conductive hearing loss, and lower cranial nerve paresis. Angiographic studies showed a hypervascular lesion supplied by numerous small branches of the external carotid artery, making embolization difficult. Complete resection was achieved by an infratemporal fossa approach with preoperative embolization. CONCLUSION: giant cell tumors of the temporal bone may mimic glomus jugulare tumors with respect to anatomic location, cranial nerve deficits, and vascularity.
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7/660. Giant-cell tumor of the skull base.

    giant cell tumors are uncommon primary bone tumors. They primarily occur in the long bones. giant cell tumors are extremely rare in the skull and head and neck. When it does occur, the maxilla and mandible are the common sites to be involved. We described two cases of giant cell tumor in the temporal bone. In the noncontrast enhanced CT, the lesion presents as a soft tissue density mass with expansion of the bone. The bony cortex is usually intact. The adjacent soft tissues and cerebral parenchyma show no infiltration or edema. The post contrast scan reveals homogenous enhancement of the mass.
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ranking = 2
keywords = bone
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8/660. Angiocentric lymphoma involving the temporal bone in a child.

    Involvement of the temporal bone in patients with malignant lymphomas is very rare. Most of the reported cases have been clinically asymptomatic and were diagnosed only by post-mortem examinations. We present a nasal, paranasal, nasopharyngeal lymphoma that occurred in a 12-year-old child and also involved the temporal bone. Clinical presentation began with bilateral chronic otitis media. Histopathologically, tumor was found to be an angiocentric lymphoma of B-cell origin. association with Epstein-Barr virus could not be demonstrated. Despite combination chemotherapy (with cyclophosphamide, vincristine, doksorubicine, prednisolone, L-asparaginase, cytosine arabinoside, metotraxate) and radiotherapy (to 40 Gy), disease progressed locally as well as to cervical lymph nodes and the lungs.
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keywords = bone
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9/660. temporal bone tumours in patients irradiated for nasopharyngeal neoplasm.

    radiation-associated tumours are rare complications of radiotherapy. This study seeks to highlight and discuss the clinically challenging problem of radiation-associated tumours (rats) in the temporal bones of seven patients previously irradiated for nasopharyngeal neoplasm. Seven patients (six males and one female) with radiation-associated temporal bone tumours are presented (five squamous cell carcinomas, one osteogenic sarcoma and one chondrosarcoma). The initial nasopharyngeal disease for which radiotherapy was indicated was nasopharyngeal carcinoma (six patients) and nasopharyngeal lymphoma (one patient). The latency period between radiotherapy and presentation of temporal bone tumours ranged from five years to 30 years with a mean of 12.9 years. All the patients underwent surgical tumour resection. Three patients had post-operative radiotherapy and one patient underwent pre- and post-operative chemotherapy. Two patients died from the disease within three months of treatment with one patient surviving 36 months at the time of writing. One patient died from an unrelated medical condition three months after surgery. With refinement in radiotherapy techniques and the resultant increase in patient survival, there may be more patients with radiation-associated tumours in the future. It remains imperative for clinicians to be vigilant when patients previously irradiated for nasopharyngeal carcinoma present with otological symptoms as the key to the successful management of this condition lies in the early detection and expedient treatment of this difficult disease.
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ranking = 3.5
keywords = bone
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10/660. Squamous cell carcinoma arising in an intradiploic epidermoid cyst.

    A 71-year-old woman presented with the symptoms of a posterior cranial fossa mass. CT and MRI revealed a lytic lesion in the occipital bone and a tumour infiltrating the dura mater, venous sinuses and cerebellum. Histopathology demonstrated a moderately differentiated squamous cell carcinoma arising from a primarily intradiploic epidermoid cyst. Despite surgery and radiotherapy, the tumour progressed and the patient died 1 year later.
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keywords = bone
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