Cases reported "Craniopharyngioma"

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1/19. 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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2/19. Phase II evaluation of interferon-alpha-2a for progressive or recurrent craniopharyngiomas.

    OBJECT: Craniopharyngiomas originate from the same cells as squamous cell skin carcinoma, which can be treated successfully with interferon-alpha (IFNalpha)-2a. The authors evaluated the activity and toxicity of systemic IFN in young patients with craniopharyngiomas. methods: Fifteen patients between the ages of 4.2 and 19.8 years who had progressive or recurrent craniopharyngiomas were enrolled in this study. Nine of these patients had never received external-beam radiation therapy. Therapy consisted of 8,000,000 U/m2 IFNalpha-2a administered daily for 16 weeks (induction phase) followed by the same dose three times per week for an additional 32 weeks (maintenance phase). Of the 12 patients who could be evaluated, radiological studies demonstrated a response to treatment in three with predominantly cystic tumors (one minor response, one partial response, and one complete response); one of these patients also showed improvement in visual fields. The size of the cystic component of the tumors often increased temporarily during the first several months of therapy. Three patients met the criteria for progressive disease during therapy. The median time to progression was 25 months. The need for radiation therapy in patients treated with IFN was delayed for 18 to 35 months (median 25 months) in six patients. All patients developed transient flulike symptoms shortly after receiving the first dose of IFN. Other toxicities (predominantly hepatic, neurological, and cutaneous) were seen in nine (60%) of the 15 patients during the first 8 weeks of treatment but resolved after temporary discontinuation and/or dose reduction. CONCLUSIONS: interferon-alpha-2a is active against some childhood craniopharyngiomas; its toxicity precludes administration of high daily doses, and the optimum dose level and schedule remain to be defined.
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3/19. mazindol administration improved hyperphagia after surgery for craniopharyngioma--case report.

    A 54-year-old man presented with visual disturbance and polydipsia. magnetic resonance imaging disclosed a cystic mass which extended from the intrasellar to the suprasellar region. Bifrontal craniotomy was performed and the tumor was totally removed. Histological findings confirmed the diagnosis of craniopharyngioma. Postoperatively, the patient suffered from transient disorientation. About one month after the operation the patient manifested hyperphagia and he gained 15 kg in one month. mazindol, a non-amphetaminergic anorectic agent, was administered for 3 weeks. His appetite normalized and his weight fell and stabilized even after mazindol administration was ceased.
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4/19. Changes in cyst volume following intraoperative MRI-guided Ommaya reservoir placement for cystic craniopharyngioma.

    INTRODUCTION: Intracavitary treatment of solitary cystic craniopharyngiomas with (32)P is an emerging treatment option, especially for pediatric patients. We have treated two patients with solitary cystic craniopharyngiomas using intraoperative MRI (iMRI)-guided catheter placement. methods: The optical tracking system of the General Electric Signa SP iMRI system was utilized for preoperative planning and intraoperative catheter tracking during insertion. Intraoperative volumetric imaging was then used to confirm final catheter position. patients were brought back to the iMRI suite approximately 8 weeks later and diluted gadolinium was injected with further MRI to confirm the absence of communication between the cyst lumen and surrounding CSF spaces and for volumetric analysis. RESULTS: Intraoperative imaging illustrated deformation and changes in the cyst wall during catheter placement and cyst aspiration and confirmed final catheter placement. Images acquired 8 weeks following catheter placement prior to the instillation of (32)P showed decreases in cyst volume of 40 and 85%. CONCLUSION: iMRI-guided catheter placement for cystic craniopharyngiomas helps to assure successful catheter placement. Significant decreases in cyst volume occur in the interval between catheter placement and (32)P administration and must be accounted for to prevent overdosing of the radioisotope.
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5/19. Huge craniopharyngioma: diffusion MRI and contrast-enhanced FLAIR imaging.

    Craniopharyngiomas are most commonly located extraaxially in the suprasellar area. They are benign but aggressive neoplasms. An adult patient is reported to have a huge craniopharyngioma with gross extensions to the surroundings. In diffusion MRI, it had high signal for b=1000 mm(2)/s (true diffusion) images, and at the same time, high ADC values (=2.12 and 2.27 x 10(-3)mm(2)/s, compared to that of normal cerebellar parenchyma 0.85 x 10(-3)mm(2)/s). In FLAIR images, obtained after administration of intravenous contrast medium, an intense, diffuse enhancement pattern was seen involving the viable tumor portions as well as the intratumoral fluid.
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6/19. Successful pregnancy following gonadotropin therapy in a patient with hypogonadotropic hypogonadism resulting from craniopharyngioma.

    The authors report a rare case of a patient with panhypopituitarism who became pregnant by gonadotropin therapy and gave birth to a healthy baby. A brain tumour and/or the surgical resection of a brain tumour occasionally results in pituitary dysfunction. An 18-year-old Japanese patient developed hypogonadotropic secondary amenorrhoea because of a craniopharyngioma, which was surgically removed. The patient came to us, and peripheral blood was collected every 15 minutes for four hours. The levels of luteinising hormone (LH) and follicle-stimulating hormone (FSH) were measured. Results showed that LH and FSH levels were very low and did not fluctuate. Several years later, the patient complained of infertility, and treatment with human menopausal gonadotropin (hMG) and human chorionic gonadotropin (hCG) was started. The therapy was repeated for several cycles, but she did not conceive, so hMG-hCG therapy combined with conjugated oestrogen administration was started. The patient became pregnant at the seventh cycle of this combined therapy. She was not treated with supplementary growth hormone.
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7/19. Intratumoural bleomycin as a treatment for recurrent cystic craniopharyngioma. Case report and review of the literature.

    INTRODUCTION: The majority of craniopharyngioma (CF) have a cystic component and only 10% are completely solid. In tumors with a large cystic component, stereotactic drainage or instillation of radioctive and/or chemotherapeutic agents have been used. Only several authors have reported the use of bleomycin for the treatment of cystic CF. CASE REPORT: The authors present the case of a nineteen years old patient with a recurrent cystic CF who was treated with intratumoral injections of bleomycin. The patient had been operated on three times before because of regrowth of the tumor. This last time he had a severe disturbance of his visual acuity and a huge regrowth of the cystic CF. An intracystic catheter stereotactically placed was connected to an Ommaya reservoir and, after assuring the impermeability of the cyst, bleomycin was administered through the reservoir up to a total dose of 45mg distributed in six doses. No complications were detected during and after the procedure. A MR performed 4 months after treatment showed a clear reduction in the size of the cyst but 10 months later a new regrowth of the cyst was detected by MR with no new signs or symptoms. A total dose of 30 mg divided in six doses was administered. No complications occurred. The MR 18 months after the first treatment showed the reduction in size of the tumor. The ophtalmological study showed almost normal visual acuity in both eyes. DISCUSSION: Although there is not an stablished protocol for the indication and the form of application of intracystic bleomycin, results with this treatment for cystic CF seem good in the literature. However, the risk of local complications after the administration of intratumoural bleomycin in these patients is around 10%, and some fatal toxic reactions have been recently reported. CONCLUSION: Intracystic administration of bleomycin is a valid option as adjuvant therapy for CF in patients with recurrences that are not surgical candidates because of the high risk of complications. The role of bleomycin as a primary treatment for CF and treatment protocols remain to be stablished with additional studies.
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8/19. Delayed persistent hyperthermia after resection of a craniopharyngioma.

    OBJECTIVE AND IMPORTANCE: Disorders of thermoregulation are occasionally noticed after operations in the region of the third ventricle. Various factors are usually implicated, but the actual contribution of each of them is rather vague. Apart from the presumed derangement in the functional connections of the hypothalamic region, mechanical reasons of compression should be thoroughly considered. CLINICAL PRESENTATION: An 8.5-year-old patient was subjected to a radical excision of a craniopharyngioma compressing the third ventricle. Three months after the operation, he presented with a febrile syndrome of unknown origin. All usual investigations proved negative. INTERVENTION: A chronic subdural hygroma was evacuated, an encapsulated CSF cyst of the suprachiasmatic cistern was drained and the lamina terminalis incised resulting in a moderate control of pyrexia. The administration of chlorpromazine contributed to the final resolution of hyperthermia. CONCLUSION: Postoperative hyperthermia may result following resection of tumors of the hypothalamic floor. It should not be blindly attributed to hypothalamic dysfunction as surgical causes could be implicated as well. chlorpromazine could be a useful adjunct to the correction of the disorder.
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9/19. Severe delirium due to basal forebrain vascular lesion and efficacy of donepezil.

    A severe intractable delirium caused by the basal forebrain vascular lesion and its dramatic recovery after donepezil administration were reported. A 68-year-old man had suffered for a month from delirium of mixed type caused by the right basal forebrain vascular lesion after surgery for craniopharyngioma. magnetic resonance imaging (MRI) showed hemorrhagic infarcts in the head of the right caudate nucleus and the right basal forebrain of the medial septal nucleus, diagonal band of broca and nucleus basalis of Meynert. He had been treated with anti-psychotics, anti-depressants and hypnotics, which resulted in little improvement. Donepezil administration dramatically improved his intractable delirium at the 19th post-donepezil administration day, but this was followed by amnestic symptoms. Clinical correlates of delirium with the basal forebrain lesion and efficacy of donepezil support the hypocholinergic theory of delirium.
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10/19. Primary cerebral lymphoma: an association with craniopharyngioma or cadaveric growth hormone therapy?

    OBJECTIVES: To present the first case of primary cerebral lymphoma associated with craniopharyngioma or previous cadaveric growth hormone therapy. CLINICAL FEATURES: A 22-year-old male shop assistant of European descent presented with unilateral uveitis and was found to have a high grade primary cerebral lymphoma. This occurred nine years after successful surgical resection of a craniopharyngioma without the administration of adjuvant radiotherapy. INTERVENTION AND OUTCOME: There was initial radiological resolution of cerebral lymphoma after cranial irradiation. recurrence was noted 10 weeks later, resulting in the patient's death. CONCLUSION: The development of primary cerebral lymphoma following a craniopharyngioma is considered most likely a chance occurrence. Cadaveric growth hormone therapy may play a role in the genesis of lymphoma.
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