Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

81 patients with expansive lesion in the optic chiasm region were reviewed. Pituitary adenomas were diagnosed in 37 patients: 5 with acromegaly, 2 with prolactinoma and the remaining 30 with hormone inactive adenomas. Meningiomas were diagnosed in 39, craniopharyngiomas in 4 patients and chordoma in one patient. The most common symptom was visual disturbance in 86%, but headache, hormonal disturbance occurred as well. All patients with adenomas were endocrinologically evaluated after detailed ophthalmologic and neurologic examinations. 28 patients were transcranially operated, 5 transsfenoidally and transcranially and 4 only transfenoidally. After endocrinologic reevaluation all patients with adenomas had undergone postoperative radiotherapy. Tumor recurrence was diagnosed in 14 patients, while 6 patients underwent reoperation. The approach to surgical regimen depended on the size and localization of meningioma. Unilateral subfrontal approach was most frequent (35). Subtotal tumor ablation was performed in 5 (13%) patients. Craniopharyngiomas were diagnosed in 4 patients (2 adults and 2 children). Total tumor ablation was performed only in one case.
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PMID:[Expansive processes in the optic chiasm region]. 892 47

The authors report a rare case of ecchordosis physaliphora (EP) in the prepontine region. A 51-year-old woman was admitted with a small cystic mass in the prepontine region, complaining of headache and an episode of transient double vision. Plain X-ray and lateral tomography films showed a protrusive hyperostosis at the middle clival region. The CT scan showed no abnormal densities in the retroclival region, and CT cisternography showed an isolated small round mass in the prepontine cistern. A small cystic mass with no enhancement with Gd-DTPA was revealed on MRI, mildly compressing the basilar artery and the rostral surface of the pons. The totally excised mass was pedunculated and was contiguous with the dorsal wall of the clivus via a small dural defect. The histologic diagnosis was EP, consisting of scattered physaliphorous cell nests, which were not positive for MIB-1 staining. The pedicle consisted of mature cartilaginous cells. The authors briefly reviewed the few previously reported cases of symptomatic EP and intradural chordoma, and discussed the differences between them. The histological features, especially the proliferation potential, may be pathognomonic. The histogenesis and the clinical features of symptomatic EP are also provided.
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PMID:[A surgical case of ecchordosis physaliphora]. 893 71

A 59-year-old man presented with a rare cholesterol granuloma of the petrous apex manifesting as headache, left facial dysesthesia, diplopia, left hearing impairment, and left tinnitus. Neurological examination revealed dysesthesia of territory in all divisions of the left trigeminal nerve, left incomplete abducens nerve palsy, left mixed hearing impairment, and left tinnitus. Plain CT scan showed a smoothly marginated mass involving the left petrous apex. The mass was isodense with the brain parenchyma and not enhanced by contrast medium. The mass appeared heterogeneously slightly hyperintense on the T1-weighted MR image and homogeneously hyperintense on the T2-weighted MR image except for the peripheral portion. The mass was not enhanced after intravenous gadolinium DTPA administration. Surgery via a petrosal approach totally removed the mass in the intracranial, extradural space. Histological examination showed typical features of cholesterol granuloma, with cholesterin clefts, hemosiderin deposits, and erythrocytes in non-specific granulation tissue. Cholesterol granuloma most commonly occurs in the middle ear cavity, and rarely in the petrous apex. The characteristic hyperintense appearance of cholesterol granuloma on T1- and T2-weighted MR images is very useful for differentiation from other lesions of the petrous apex and the cerebellopontine angle such as cholesteatoma, mucocele, chordoma, and meningioma. Solid cholesterol granuloma of the petrous apex should be treated by total removal via craniotomy, not by drainage which is commonly performed by otorhinologists.
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PMID:[An operative case of cholesterol granuloma of the petrous apex]. 893 74

Three cases of chondromyxoid fibroma arising in the skull base are reported. The tumors arose in females 34, 65, and 66 (median 55) years of age. Two women presented with headaches, and one with nasal obstruction. Radiographic studies revealed that all three lesions were expansile soft tissue masses centered in the clivus, at least 4 cm in greatest diameter. One lesion involved primarily the clivus, the others extended from the clivus into the sphenoid and ethmoid sinuses. Two of the three cases were initially misdiagnosed as chordoma or chondrosarcoma. The initial treatment was curettage of gross disease in all three cases. One patient also received radiation therapy. One patient had local progression of disease, which was treated with surgery and radiation therapy. All patients are clinically free of disease 11 to 26 months following the most recent treatment. Chondromyxoid fibroma can and should be distinguished from chondrosarcoma and chordoma, two tumors which more commonly arise in the skull base and which have the potential to metastasize.
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PMID:Chondromyxoid fibroma of the skull base: a tumor which may be confused with chordoma and chondrosarcoma. A report of three cases and review of the literature. 915 83

Chordomas are slow growing, locally invasive tumors that most commonly present as midline masses in the sacrococcygeal or clival regions. The case presented in this paper demonstrates the typical MRI appearance of a clival chordoma in a patient presenting with classic symptoms of cranial nerve compression and headache. While the exact signal characteristics of these lesions seen on MRI may vary with pathologic subtypes, MRI has proven essential in the diagnosis, surgical planning and post-treatment evaluation of patients with these lesions.
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PMID:Clivus chordoma. 932 79

Chordoid meningioma is a rare variant of meningioma that bears a striking histological resemblance to chordoma and has greater likelihood of recurrence. Although most meningiomas occur in the intracranial, orbital and intravertebral cavities, rare meningiomas have been reported in extracranial organs; thus, it is important to be able to distinguish them from other neoplasms that have similar histology but different biological behavior and therapies. A case of chordoid meningioma in a 48-year-old woman who did not have Castleman's syndrome is described in the present report. The patient presented with a mass in her left frontoparietal region, and had been suffering from headaches for many years. Magnetic resonance imaging of the brain demonstrated an expansive lytic lesion in the squamous portion of the left temporal bone. The lesion extended in both directions. Histological examination of the surgical specimen revealed a tumor composed of cords and nests of eosinophilic vacuolated cells embedded in a myxoid matrix. A typical meningiomatous pattern was observed focally, and positive staining of the tumor cells for vimentin and epithelial membrane antigen confirmed the diagnosis of chordoid meningioma.
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PMID:Chordoid meningioma: rare variant of meningioma. 1548 3

Chordoid meningioma is a rare meningothelial tumor characterized by chordoma-like histological features with lymphoplasmacellular infiltration. This tumor is often seen in children, but not in adults, with a systemic inflammatory syndrome (iron-resistant microcytic anemia and/or dysgammaglobulinemia) and very rarely with a persistent moderate hyperthermia. In the present report the authors describe a temporal chordoid meningioma in a 30-year-old woman who presented with fever, headache, and a serological inflammatory syndrome. The clinical symptomatology, chiefly the fever, disappeared immediately after removal of the tumor. To the authors' knowledge, only one similar patient with such clinical presentation and response to surgery has been mentioned in the literature. Interestingly, at immunohistochemical examination, the neoplasm showed focal positivity for the pyrogenic cytokine interleukin-6. The capacity of the tumor to produce this pyrogenic cytokine could explain both the patient's clinical presentation and her response to the surgical management.
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PMID:Pyrogenic cytokine interleukin-6 expression by a chordoid meningioma in an adult with a systemic inflammatory syndrome. Case report and review of the literature. 1623 90

Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment.
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PMID:Craniospinal dissemination of clival chondroid chordoma. 1633 98

Chordomas are rare tumors of the central nervous system and primarily occur in the extradural space. We report two patients with intracranial chordomas located in the region of the clivus that invaded the prepontine cisterns. The patients, 45 and 62 years old, had histories of cranial neuropathies and headache, respectively. Petrosal approaches were performed in both with radical resection of the tumors. Anatomopathological studies confirmed the diagnosis of chordoma. The symptoms of both patients resolved, and they have had no recurrence after 18 months of follow-up. Intradural chordomas or extradural chordomas that invade the intradural space are difficult to differentiate from ecchordoses physaliphorae, a non-neoplastic entity with similar radiological features. MBI-1 studies were therefore performed to confirm the diagnosis of chordoma. The differential diagnosis for these two entities, the different modalities of treatment, and prognosis are reviewed.
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PMID:Intradural invasion of chordoma: two case reports. 1716 71

Twelve patients with histologically confirmed clivus chordoma were treated at the Johns Hopkins Hospital between 1971 and 1989. Eight of the patients were men and four were women. The mean age at first operation was 51 years (range, 10 to 80). The most common presenting symptoms were headache, diplopia, dysphagia and dysarthria, and facial sensory changes. Computed tomography, with and without contrast enhancement, proved adequate for tumor identification and localization. Magnetic resonance imaging and angiography were occasionally employed to localize the tumors further and to define tumor vascular supply and proximity to vascular structures. Twenty-two resections were performed in 11 patients, and another patient underwent biopsy only. Seven were also treated with radiation therapy. Tumors recurred in eight patients, six of whom underwent further operations. The mean time to first recurrence was 22 months (range 8 to 36 months). Six of the patients are still alive, with a mean follow-up of 31 months (range, 3 to 89 months) from first surgical resection. The mean survival time from first treatment was 31 months (range, 4 to 62 months) among those patients who died. There was no operative mortality. The 5-year cumulative survival rate was 20%. Six patients with long follow-up have had fair to good results, being free of recurrences for at least a year. However, none of the patients returned to their premorbid baseline of activities. Five of the patients had tumors with the histologic diagnosis of chondroid chordoma. Three of these patients are still alive. The mean age at first treatment was 44 (compared with 62 for typical chordomas). The mean time from symptoms to diagnosis was 29 months (typical chordomas, 18 months). The mean length of survival and time to tumor recurrence were not significantly different between chondroid and typical chordomas.
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PMID:Clivus chordoma: a report of 12 recent cases and review of the literature. 1717 Aug 37


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