Cases reported "Craniopharyngioma"

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11/208. Long-term magnetic resonance imaging follow-up of asymptomatic sellar tumors. -- their natural history and surgical indications.

    Serial magnetic resonance (MR) images and clinical symptoms were analyzed in 23 patients with sellar lesions, who were followed up without initial therapy for mass reduction to evaluate their natural history and surgical indication for these lesions. The patients were aged 17 to 78 years (mean 47.3 years) and the follow-up period was 1.5 to 11.6 years (mean 5.1 years). Lesions were divided into two types based on the MR imaging findings, regardless of their histological types. Type C was cystic with or without enhancement of the smooth and thin wall. Type S had enhanced solid components. Ten patients had Type C tumors. Three patients presented with sudden onset of headache. The tumor size spontaneously decreased with intensity change, indicating pituitary apoplexy as the trigger of the onset and intensity change. Four patients presented with the visual disturbance which improved with the reduction of tumor size, but three patients deteriorated and required surgery. The operation revealed Rathke's cleft cyst. The remaining three patients were found incidentally and have been asymptomatic without MR imaging changes. Thirteen patients had Type S tumors. Six patients of nine with 14 mm or larger tumors developed symptomatic tumor enlargement over the follow-up period of 1.2 to 8.6 years (mean 4.9 years) and required treatment. The remainder showed no change. Type C tumors frequently shrink or even disappear spontaneously. We can justify conservative follow-up of Type C tumors in patients with no or only transient symptoms. Type S tumors, larger than 14 mm in size, need closer observation or treatment because they often enlarge and become symptomatic.
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12/208. Fatal toxic effect of bleomycin on brain tissue after intracystic chemotherapy for a craniopharyngioma: case report.

    OBJECTIVE AND IMPORTANCE: Craniopharyngiomas are benign neoplasms of epithelial origin that arise from the remnants of Rathke's pouch and are located in the sellar, parasellar, and third ventricular regions. Despite major advances in microsurgical techniques, total removal of these tumors is associated with a high risk of death, long-term endocrinological dependence, cognitive dysfunction, and behavioral disorders. For patients with monocystic craniopharyngiomas, encouraging postoperative survival rates and high rates of cyst regression after intracavitary administration of bleomycin have been reported. Moreover, only a few side effects have been reported for this treatment method. We report a patient with a cystic craniopharyngioma who was treated using intracavitary bleomycin administration and died as a result of the direct toxic effects of bleomycin on deep brain structures and the brainstem. CLINICAL PRESENTATION AND INTERVENTION: A 47-year-old woman with a cystic craniopharyngioma underwent stereotactic insertion of a catheter attached to a subcutaneous reservoir. Five months after the procedure, positive-contrast computed tomographic cystography was performed to confirm the absence of fluid leakage. Daily intracavitary injections of bleomycin were administered through the reservoir into the cyst, until a total dose of 56 mg had been administered in 8 days. After the treatment, the cystic cavity regressed but the patient exhibited neurological deterioration; magnetic resonance imaging scans revealed diffuse edema in the diencephalon and brainstem. The patient died 45 days after completion of the treatment. CONCLUSION: Intracavitary administration of bleomycin is not a treatment protocol without risks or side effects, even if there is no fluid leakage into the cerebrospinal fluid. Although this is known to be an effective treatment for cystic craniopharyngiomas, previous reports cannot be used to establish a standard treatment method, and more research is needed to yield a safer effective protocol.
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13/208. Severe hypoglycemia and reduction of insulin requirement in a girl with insulin-dependent diabetes mellitus: first sign of a craniopharyngioma.

    A girl with a history of insulin-dependent diabetes mellitus since 5.5 years, and Hashimoto's thyroiditis since 12 years, developed episodes of severe hypoglycemia from the age of 12 years. This was associated with falling insulin requirements, from 0.78 U/kg/day at 11 years to 0.34 U/kg/day at 16 years. At 16 years she was found to have GH, gonadotropin, ACTH, and probably also TSH deficiency with hyperprolactinemia. MRI scan revealed a cystic intrasellar craniopharyngioma with moderate suprasellar extension. In spite of cortisol replacement at 17 years, insulin requirement fell further to 0.25 U/kg/day at 18 years. In this girl, decreasing insulin requirements represented an early manifestation of combined growth hormone and cortisol deficiency.
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14/208. Neonatal craniopharyngioma. Case report.

    The authors report a case of craniopharyngioma in a newborn infant. Marked hydrocephalus of the fetal head was recognized in the pelvimetric roentgenograms of the mother and massive suprasellar calcification was seen in the plain skull films.
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15/208. Giant cystic craniopharyngiomas with extension into the posterior fossa.

    Between 1991 and 1998, 24 patients underwent surgery for a craniopharyngioma in our department. This group included two patients who had tumors with extensive growth along the midline, and along the posterior fossa in particular. In both cases suprasellar calcifications were typical features on magnetic resonance imaging (MRI) and computed tomography (CT). A 7-year-old boy presented with a 6-month history of headache, nausea, and progressive unilateral hearing loss. With a suboccipital approach it was possible to remove the main part of the tumor. In a 13-year-old boy headache and visual deterioration led to the diagnosis of a craniopharyngioma, which was removed with a pterional approach. For the neuroimaging work-up in such cases of atypically growing craniopharyngiomas MRI is the method of choice. Additional CT scanning is recommended, which provides valuable information about bony changes at the skull base due to space-occupying growth. CT substantiates the differential diagnosis if typical calcifications are seen.
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16/208. Resection of suprasellar tumors by using a modified transsphenoidal approach. Report of four cases.

    Generally accepted contraindications to using a transsphenoidal approach for resection of tumors that arise in or extend into the suprasellar region include a normal-sized sella turcica, normal pituitary function, and adherence of tumor to vital intracranial structures. Thus, the transsphenoidal approach has traditionally been restricted to the removal of tumors involving the pituitary fossa and, occasionally, to suprasellar extensions of such tumors if the sella is enlarged. However, conventional transcranial approaches to the suprasellar region require significant brain retraction and offer limited visualization of contralateral tumor extension and the interface between the tumor and adjacent structures, such as the hypothalamus, third ventricle, optic apparatus, and major arteries. In this paper the authors describe successful removal of suprasellar tumors by using a modified transsphenoidal approach that circumvents some of the traditional contraindications to transsphenoidal surgery, while avoiding some of the disadvantages of transcranial surgery. Four patients harbored tumors (two craniopharyngiomas and two hemangioblastomas) that arose in the suprasellar region and were located either entirely (three patients) or primarily (one patient) within the suprasellar space. All patients had a normal-sized sella turcica. Preoperatively, three of the four patients had significant endocrinological deficits signifying involvement of the hypothalamus, pituitary stalk, or pituitary gland. Two patients exhibited preoperative visual field defects. For tumor excision, a recently described modification of the traditional transsphenoidal approach was used. Using this modification, one removes the posterior portion of the planum sphenoidale, allowing access to the suprasellar region. Total resection of tumor was achieved (including absence of residual tumor on follow-up imaging) in three of the four patients. In the remaining patient, total removal was not possible because of adherence of tumor to the hypothalamus and midbrain. One postoperative cerebrospinal fluid leak occurred. Postoperative endocrinological function was worse than preoperative function in one patient. No other new postoperative endocrinological or neurological deficits were encountered. This study demonstrates the feasibility of using a modified transsphenoidal approach for resection of certain suprasellar, nonpituitary tumors.
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ranking = 8.7321770769388
keywords = sella turcica, turcica, sella
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17/208. Global improvement in intellectual and neuropsychological functioning after removal of a suprasellar cystic craniopharyngioma.

    This is a case report of a patient who presented with cognitive and behavioral decline and underwent surgery for removal of a suprasellar craniopharyngioma. Previous studies have reported memory impairments in the presence of craniopharyngioma, but information is lacking regarding the impact of craniopharyngioma on other brain functions as well as on intellectual abilities. In this study, comprehensive neuropsychological testing, including intellectual testing, was conducted 9 to 14 days before surgery and 16 to 22 days after surgery. Results before surgery demonstrated average intellectual abilities, which were decreased from premorbid estimates, and diffuse neuropsychological impairments. Postoperatively, significant improvement in both intellectual abilities and neuropsychological test scores was observed. These results suggest that craniopharyngiomas can be associated with impairments in intellectual abilities and in brain functions aside from memory. The results are contrasted with those of previous reports, and implications for future research are discussed.
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18/208. recurrence of sellar and suprasellar tumors in children treated with hGH--relation to immunohistochemical study on GH receptor.

    PURPOSE: GH replacement therapy is required in the majority of children with GH deficiency after treatment of sellar and suprasellar tumors. Owing to the high cell proliferative ability of human GH (hGH), its influence on tumor recurrence has been debated. We retrospectively studied the immunohistochemical expression of the GH receptor in various tumor tissues, in order to investigate the relation between tumor recurrence and hGH replacement. methods: GH replacement therapy was performed in 25 patients (8 boys and 17 girls) after the treatment. Tumor recurrence was noted in 4 patients (craniopharyngioma: 2 patients, pilocytic astrocytoma and germinoma: 1 each). Immunohistochemical study of GH receptor in tumor tissue was carried out in those recurrent and recurrence-free cases, by using MAb 263 as a primary antibody. RESULTS: Two patients with recurrent craniopharyngioma were positive for MAb 263, but 1 recurrence-free patient was negative. patients with pilocytic astrocytoma (recurrent and recurrence-free: 1 each) were all positive. Five patients with germinoma (1 with recurrence and 4 without recurrence) were all negative. CONCLUSION: In the patients with craniopharyngioma treated with GH, a positive immunohistochemical expression of GH receptor in tumor tissue may indicate a high probability of recurrence. In our cases, GH receptor was positive in astrocytomas and negative in germinomas, with or without recurrence. It is therefore speculated that each brain tumor may have its specificity in GH receptor expression.
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keywords = sella
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19/208. Visual loss in a 42-year-old man.

    A 42-year-old man developed blurred vision and was found to have a right, incongruous, inferior homonymous quadrantanopia. neuroimaging disclosed a suprasellar mass. The mass was thought to be a pituitary adenoma that was compressing the left optic tract, and transsphenoidal surgery was planned; however, because the defect was primarily inferior, indicating damage to the superior aspect of the optic tract, it was recommended that a craniotomy be performed. The mass was found at surgery to be a craniopharyngioma.
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20/208. Spontaneous intraventricular rupture of craniopharyngioma cyst.

    BACKGROUND: rupture of a cystic craniopharyngioma is a rare phenomenon. The rupture of the cyst causes decompression of the adjacent neural structures resulting in spontaneous improvement of the visual symptoms or level of sensorium. The leakage of its contents into the subarachnoid space gives rise to meningismus. We report an extremely rare phenomenon of an intraventricular rupture of a cystic craniopharyngioma, which resulted in acute neurological deterioration and chemical ventriculitis. CASE DESCRIPTION: A 38-year-old lady presented with a 1-year history of frontal lobe dysfunction and bilateral primary optic atrophy. The CT scan showed a multi-loculated, hyperdense lesion in the region of the third ventricle and suprasellar cistern. She suffered acute deterioration of neurological status; computed tomography (CT) scan showed a hypodense lesion in the suprasellar cistern with persistent hydrocephalus. She was treated with ventricular drainage, steroids and anticonvulsants. Ventricular fluid showed high cholesterol and LDH levels. The diagnosis of craniopharyngioma was subsequently verified histologically. CONCLUSIONS The intraventricular rupture of a cystic craniopharyngioma can result in acute clinical deterioration and morbidity because of chemical ventriculitis. This is unlike the rupture in the subarachnoid space or sphenoid sinus which usually results in symptomatic improvement, although chemical meningitis may occur. This rare phenomenon should be recognized, and prompt ventricular drainage is advised. The literature is reviewed, and management of this condition is discussed.
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