Cases reported "Craniopharyngioma"

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1/20. Suprasellar cystic germinoma.

    We report on a germinoma in the suprasellar region, which had multiple large cystic components. A 13-year-old girl with disturbed visual acuity and growth retardation was admitted to our hospital for treatment of an intracranial tumor. The lesion was difficult to diagnose as a germinoma preoperatively, because of its radiographic characteristics. Histopathological examination revealed that the tumor was a germinoma. Surgery, chemotherapy with carboplatin and etoposide, and radiotherapy (30 Gy) were successful in inducing complete remission of the tumor. The patient's endocrine status remained normal, except for a low GH concentration and diabetes insipidus.
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keywords = diabetes insipidus, insipidus, diabetes
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2/20. 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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keywords = diabetes
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3/20. Suprasellar malignant mixed germ cell tumour presenting as craniopharyngioma.

    A 15 year old boy presented with diminution in vision of both eyes, diabetes insipidus and hypopituitarism. MRI was suggestive of a large suprasellar and retrosellar craniopharyngioma with stretching of the optic chiasma. Histopathological findings on the first surgical specimen were interpreted as a craniopharyngioma. He was reoperated on account of clinical deterioration and increase in tumour size. Histological examination this time revealed derivatives of all three germ cell lineages along with areas of embryonal carcinoma, and yolk sac tumour besides squamous cysts, establishing the diagnosis of malignant mixed germ cell tumour. serum and CSF were strongly positive for alpha foetoproteins.
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keywords = diabetes insipidus, insipidus, diabetes
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4/20. Radical surgery in a neonate with craniopharyngioma. report of a case.

    ultrasonography revealed a suprasellar tumor in a fetus at 28 weeks of gestation. The male newborn, delivered 10 weeks later, was operated at the age of 17 days, and a craniopharyngioma was completely removed. Intraoperatively, inappropriate secretion of antidiuretic hormone occurred and was followed by diabetes insipidus causing imbalance of fluid and electrolytes. The tumor recurred and was totally removed 1 year later. Further development was uneventful and, at the age of 8 years, the boy is in generally good mental and physical condition except for a left-sided hemiparesis. In contrast to the poor outcome of neonatal craniopharyngioma reviewed in the literature, this case may encourage radical surgery even in the very young.
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keywords = diabetes insipidus, insipidus, diabetes
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5/20. Perioperative management of central diabetes insipidus in kidney transplantation.

    Central diabetes insipidus is clinically masked in dialysis patients. We report a 12-year-old girl receiving a living-related donor graft for renal failure from Alport syndrome, in whom a craniopharyngioma had been resected 6 months before transplantation. Pretransplant evaluation had documented central hypothyroidism, growth hormone deficiency, and presumptive hypogonadotropic hypogonadism. The corticotropin-releasing factor test had been normal. Four hours after transplantation, urine output exceeded 1,000 ml/h without diuretic therapy. serum sodium concentration was 155 mmol/l, serum osmolality 333 mmol/kg, and plasma antidiuretic hormone 4.9 ng/l, while urine osmolality was 233 mmol/kg. Desmopressin acetate was started by continuous intravenous infusion at 1 microgram/day. serum electrolytes rapidly normalized, urine output stabilized at 2 l/day. The patient was discharged 4 weeks after transplantation with good allograft function, receiving intranasal desmopressin acetate 10 micrograms twice daily. Pre-existing central diabetes insipidus is unmasked after successful kidney transplantation, leading to rapid dehydration and hypernatremia, which can be prevented by prompt institution of desmopressin therapy.
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keywords = diabetes insipidus, insipidus, diabetes
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6/20. Intraventricular craniopharyngioma.

    An unusual case of an adult with a craniopharyngioma within the third ventricle is reported. The patient complained of headaches, had a history suggestive of diabetes insipidus, and presented with a severe dementia. A brain scan revealed the suprasellar midline lesion, and a pneumoencephalogram confirmed its location within the third ventricle. Therapy included partial surgical excision, followed by a ventriculoatrial shunt and radiation.
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keywords = diabetes insipidus, insipidus, diabetes
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7/20. survival with serum sodium level of 180 mEq/L: permanent disorientation to place and time.

    A 41-year-old woman who had undergone transfrontal craniotomy for a pituitary tumor 4 months before presentation was admitted with confusion and orientation only to self. She had a fever of 40 degrees C. serum sodium and chloride levels on admission were 180 and 139 mEq/L, respectively. Measured serum osmolality was 380 mOsmol/L with a urine osmolality of 360 mOsmol/L. magnetic resonance imaging revealed a 1.5-cm mass in the sella turcica, which was nonfunctioning on endocrine evaluation. The "bright spot" of a normal posterior pituitary was absent. Central diabetes insipidus was confirmed by a 300% increase in urine osmolality with desmopressin. The patient survived her severe hypernatremia, which has 70% mortality with a serum sodium level of 160 mEq/L or above. However, she developed permanent (6 months) disorientation to time and place even when hypernatremia was corrected, which has not been described previously.
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keywords = diabetes insipidus, insipidus, diabetes
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8/20. Intrinsic third ventricular craniopharyngiomas: report on six cases and a review of the literature.

    BACKGROUND: Craniopharyngiomas constitute 2 to 4% of intracranial neoplasms. However, the purely intraventricular variety are rare. Their magnetic resonance imaging (MRI) characteristically shows an intact third ventricular floor, a patent suprasellar cistern, an intact pituitary stalk, and the absence of sellar abnormalities. methods: Between 1994 and June 2002, 6 patients with purely intraventricular craniopharyngioma were surgically managed. There were 4 cystic and 2 solid lesions. The surgical approaches utilized included a frontal, parasagittal, transcallosal approach with the third ventricle being accessed using either the transforaminal or subchoroidal approach (n = 3); pterional, transsylvian (n = 1), and bifrontal interhemispheric (n = 2) approaches in which the third ventricle was accessed via the lamina terminalis. A ventriculoperitoneal shunt was required for one of the 3 patients with hydrocephalus. RESULTS: Total excision was performed in 3 patients with cystic craniopharyngioma, while a small residual lesion was left adherent to the third ventricular floor in the others. There was one perioperative mortality because of septicemia. Two patients required thyroxine supplementation. Two patients developed transient and one other patient a sustained diabetes insipidus. The 2 patients with solid tumors received radiotherapy for the residual lesions. At follow up ranging from 8 to 36 months, neither tumor recurrence nor regrowth was observed in any of the patients. The symptoms of raised intracranial pressure, hypothalamic dysfunction or visual field defects had resolved. CONCLUSIONS: Intraventricular craniopharyngiomas occur in an older population and present mainly with raised intracranial pressure. Visual and endocrinologic imbalances are much less in these lesions compared to the suprasellar craniopharyngiomas. They mainly attach to the third ventricular floor. The surgical approaches to the third ventricle, along with radiotherapy and hormone supplementation, were successful in the management of these rare tumors.
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ranking = 1
keywords = diabetes insipidus, insipidus, diabetes
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9/20. Inflammatory thromboembolic complication after craniopharyngioma surgery?

    Aside from endocrine or visual disturbances an appearance of other complications after craniopharyngioma surgery is rare. We report a case of delayed brain infarction in the postoperative period of craniopharyngioma resection. A 30-year-old man presented with visual impairment and diabetes insipidus. Imaging revealed a partly cystic, partly contrast enhancing intra-/suprasellar mass lesion. The patient underwent craniotomy with resection of the tumour. Histological examination revealed an adamantinomatous craniopharyngioma. Early postoperative an aseptic meningitis, which was diagnosed clinically as well as by laboratory and CSF analysis, resolved spontaneously. One week later a cerebral incident with infarction in the peripheral territory of MCA on the left side occurred. For this unusual event with a review of the literature a thromboembolic origin is suggested on the basis of inflammatory vascular involvement after an attack of aseptic meningitis.
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keywords = diabetes insipidus, insipidus, diabetes
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10/20. Variations on the standard transsphenoidal approach to the sellar region, with emphasis on the extended approaches and parasellar approaches: surgical experience in 105 cases.

    OBJECTIVE: The traditional boundaries of the transsphenoidal approach may be expanded to include the region from the cribriform plate of the anterior cranial base to the inferior clivus in the anteroposterior plane, and laterally to expose the cavernous cranial nerves and the optic canal. We review our combined experience with these variations on the transsphenoidal approach to various lesions of the sellar and parasellar region. methods: From 1982 to 2003, we used the extended and parasellar transsphenoidal approaches in 105 patients presenting with a variety of lesions of the parasellar region. This study specifically reviews the breadth of pathological lesions operated and the complications associated with the approaches. RESULTS: Variations of the standard transsphenoidal approach have been used in the following series: 30 cases of pituitary adenomas extending laterally to involve the cavernous sinus, 27 craniopharyngiomas, 11 tuberculum/diaphragma sellae meningiomas, 10 sphenoid sinus mucoceles, 18 clivus chordomas, 4 cases of carcinoma of the sphenoid sinus, 2 cases of breast carcinoma metastatic to the sella, and 3 cases of monostotic fibrous dysplasia involving the clivus. There was no mortality in the series. Permanent neurological complications included one case of monocular blindness, one case of permanent diabetes insipidus, and two permanent cavernous cranial neuropathies. There were four cases of internal carotid artery hemorrhage, one of which required ligation of the cervical internal carotid artery and resulted in hemiparesis. The incidence of postoperative cerebrospinal fluid fistulae was 6% (6 of 105 cases). CONCLUSION: These modifications of the standard transsphenoidal approach are useful for lesions within the boundaries noted above, they offer excellent alternatives to transcranial approaches for these lesions, and they avoid prolonged exposure time and brain retraction. Technical details are discussed and illustrative cases presented.
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keywords = diabetes insipidus, insipidus, diabetes
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