Cases reported "Skull Neoplasms"

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1/68. Intrasellar chordomas mimicking pituitary adenoma.

    OBJECT: Whereas chordomas involving the sellar region are uncommon, largely or entirely intrasellar examples are rare. The goal in this study was to present examples of these rare tumors as a guide to their proper diagnosis and treatment. methods: The authors report three cases in which the chordomas filled the pituitary fossa and presented as nonfunctioning pituitary adenomas. All lesions exhibited the typical histological patterns and immunophenotype of chordoma. One tumor, studied ultrastructurally and subjected to dna analysis, was shown to have a diploid histogram. The authors present a clinicopathological study of these three cases and review the literature on intrasellar chordomas. CONCLUSIONS: Although these tumors are easily misdiagnosed and therefore may not receive optimal treatment, aggressive surgical resection can yield a favorable prognosis in lesions with a limited extent.
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ranking = 1
keywords = chordoma
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2/68. Management issues in chordoma: a case series.

    This study presents a combined case series of chordomas from two Canadian institutes. Twenty-seven patients were identified for the period 1954-1998. Management issues with regard to diagnostic pitfalls, selection of charged particle treatment and retreatment of recurrences are discussed. The diagnosis of early stage chordoma requires a high index of suspicion. One patient in the series presented with hoarseness and is described in detail. The diagnosis was made coincidentally by a computed tomographic scan of the head, performed after a motor vehicle accident. The planning of both surgery and radiotherapy was optimized by using magnetic resonance imaging. A review of the literature supports the use of a combined surgical and radiotherapeutic approach.
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ranking = 0.75
keywords = chordoma
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3/68. Fibromyxoma of the petrous apex.

    The common petroclival bony tumors are chordoma and chondrosarcoma. myxoma of the bone occurs only in the facial bones, and it has not been reported in skull bones. The computed tomographic scanning, magnetic resonance imaging and intraoperative characteristics of a case of fibromyxoma of the petrous bone in a 14-year-old girl are described.
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ranking = 0.125
keywords = chordoma
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4/68. Craniocervical chordomas.

    In a 20 year period seven cases of craniocervical chordoma were seen and treated. Since it is not possible to completely resect these lesions, the aim of treatment has been to debulk the tumor as much as feasible and employ postoperative irridation. Palliation is the most that can be hoped for, and minimal success in delaying the natural history of the tumor has been achieved. Surgery for relief of symptoms secondary to bulky recurrence is indicated for palliation, as is a repeat course of x-ray therapy. Isolated cases of long-term useful survival have followed doses in the range of 10,000 rads or more [19,20]. This therapy is usually given in smaller increments of an initial dose when there is evidence of recurrent tumor or pain [2]. However, whenever the dosage to the spinal cord or the base of the brain exceeds 4,000 rads, there is a concern about possible permanent radiation damage. These patients are difficult to care for late in the course of their disease when multiple cranial nerve paralysis, long tract signs, and intractable pain become progressively severe. Chordomas usually remain localized; however, about 10% metastasize distally. One case is presented of a large retropharyngeal mass with destruction of the second cervical vertebra that recurred locally in 3 months despite usual treatment; distal bony metastases were found 3 years after initial treatment. The histology of the lesion, its recurrence, and distal bony metastases were essentially indistinguishable from the other lesions that remained localized. Short periods of palliation from severe local pain and exquisite tenderness have been achieved by local resection of the involved bones.
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ranking = 0.625
keywords = chordoma
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5/68. Chondroid chordoma presenting with hypopituitarism.

    A 28-year-old man with chondroid chordoma, an uncommon variant of chordoma, is reported. The patient presented with visual disturbance and hypopituitarism. The latter is a rare complication of intracranial chordoma. The preoperative diagnosis of chondroid chordoma of the skull base was based on unique findings on computed tomography and magnetic resonance imaging studies. The development of the tumor over six years suggests that the prognosis of chondroid chordoma may be poor in younger patients, as recently reported. Although chondroid chordoma is very rare, it should be included in the differential diagnosis of hypopituitarism.
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ranking = 1.25
keywords = chordoma
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6/68. Cranial chordoma in the first decade.

    Cranial chordomas are extremely rare in childhood with only 25 cases having been reported in the first decade of life. A 6-year-old female child with cranial chordoma is reported. literature on the subject is reviewed, with special reference to the management, histopathological features and prognosis in childhood chordomas as compared to the adult variety.
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ranking = 0.875
keywords = chordoma
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7/68. Transoral approach to tumors of the clivus: report of two cases.

    A transoral approach was used in 2 patients with tumors of the clivus, chordoma, and fibrochondro-chordosarcoma. Choice of the approach was based on data provided by craniofacial examination, radiographic examinations, magnetic resonance imaging, computed tomographic scans, and vertebral angiography, all of which suggested tumor involving the oropharynx and nasopharynx, with destruction of the clivus bone structure. This report demonstrates the feasibility of the transoral approach.
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ranking = 0.125
keywords = chordoma
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8/68. The subtemporal, transcavernous, anterior transpetrosal approach to the upper brain stem and clivus.

    The temporal lobe, posterolateral cavernous sinus, tentorium, and petrous apex restrict anterolateral surgical access to lesions of the upper brain stem and clivus. The authors describe a modified transpetrosal approach that enhances the exposure of clival chordomas and aneurysms of the basilar artery bifurcation. An intradural and extradural subtemporal approach is combined with division of the tentorium and superior petrosal sinus, posterolateral dissection of the cavernous sinus, and intradural removal of the petrous bone from its apex to the cochlea. The indications, advantages, and disadvantages of this subtemporal, transcavernous, anterior transpetrosal approach are described in detail, along with its use in six patients.
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ranking = 0.125
keywords = chordoma
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9/68. chordoma mimicking the trigeminal schwannoma: a case report.

    Intracranial chordomas are rare tumor arising from the remnants of embryologic notochord. Bony destruction and tumor calcification are their characters. Now we represent an extra-axial tumor with an unusual dumbbell shape in the right Meckel's cave and the appearance mimics the trigeminal schwannomas. However, the histopathological findings reveal a chordoma. Bony destruction in the right petrous bone and clivus provides a hint to make appropriate diagnosis.
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ranking = 0.25
keywords = chordoma
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10/68. cytogenetic analysis of three variants of clival chordoma.

    chordoma is an uncommon malignant neoplasm derived from remnants of the embryonal notochord. The tumor arises in the sacrococcygeal region in most cases. Cytogenetic information on clival chordomas is scarce due to the low incidence of these tumors. In this study, we applied the G-banding and spectral karyotyping techniques to compare the karyotypes of three variants of clival chordoma: conventional, chondroid, and dedifferentiated. We describe a normal karyotype of a chondroid chordoma and a complex karyotype of a conventional chordoma involving chromosomes 1, 2, 3, 5, 8, 9, 11, 15, 19, 20, and X. The cytogenetic analysis of the dedifferentiated chordoma showed a polyploid complex karyotype of 71-123 chromosomes with double minutes that originated from chromosome 17.
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ranking = 1.125
keywords = chordoma
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