Cases reported "Pituitary Neoplasms"

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1/21. Neurohypophyseal pilocytic astrocytoma invading the skull base.

    We describe the clinical presentation, neuroradiological and histological findings of an unusual case of pilocytic astrocytoma of the neurohypophysis, and discuss the related surgical and prognostic issues of this neoplasm which invaded the skull base and the sphenoid sinus. Only four histologically proven cases of such a tumour have been reported in the English literature, and the pathological features and behaviour of this neoplasm still await definition.
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2/21. Absent p53 immunohistochemical staining in a pituitary carcinoma.

    BACKGROUND: Carcinomatous transformation of pituitary adenomas is uncommon, and is generally accompanied by nuclear accumulation of p53 protein. Pituitary carcinoma lacking accumulation of p53 protein is very rare, only two such cases being previously reported. methods: A patient presented with visual disturbance and cranial nerve palsies and was found to have a suprasellar mass. He underwent both transphenoidal and transfrontal excision of a nonfunctioning pituitary adenoma which recurred several times. The third recurrence was accompanied by multiple dural-based metastases. Despite aggressive surgical management, he continued to develop additional intracranial lesions and died two years after the discovery of metastatic disease. Specimens from 1984, 1995, 1997 and 1998 were available for histological and immunocytochemical analysis. antibodies recognizing the pituitary hormones (ACTH, PRL, GH, FSH, LH and TSH), as well as cytokeratin, epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP) and chromogranin a were applied to investigate the lineage of the neoplasm. Antisera specific for Ki-67 (MIB-1) and p53 protein were also applied to further delineate the biology of the tumour. RESULTS: Although cytokeratin and chromogranin a were detected in neoplastic cells. no expression of pituitary hormones was demonstrable, indicative of a nonfunctioning, null-cell pituitary adenoma. Nuclear pleomorphism and mitotic activity increased with subsequent resections. Abnormal accumulation of p53 protein was not observed, neither in early resections nor in the metastatic deposits. CONCLUSIONS: Failure to demonstrate p53 protein accumulation does not ensure a favourable outcome for pituitary adenoma. Accordingly, pituitary carcinoma may occur in the absence of p53 accumulation. The factors which underlie aggressive behaviour of pituitary neoplasms are uncertain but are under investigation.
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3/21. Endoscopic fenestration of symptomatic septum pellucidum cysts: three case reports with discussion on the approaches and technique.

    OBJECTIVES: We describe three patients with symptomatic septum pellucidum cysts treated by endoscopic fenestration and discuss the different endoscopic approaches to these cysts. CLINICAL PRESENTATION: The patients are an 8-year-old boy, a 7-year-old boy and a 21-year-old woman; this last also had a right frontal cavernous angioma and a pituitary microadenoma. All patients presented with headache, associated with vomiting in two and behavioral changes in one. In all cases magnetic resonance showed a septum pellucidum cyst. INTERVENTION: Two patients were operated upon by posterior approach through a right occipital burr hole and underwent fenestration from the right occipital horn to the cyst, with a second fenestration from the cyst to the left lateral ventricle in one. Another patient underwent microsurgical removal of a right frontal cavernoma and endoscopic cyst fenestration with both lateral ventricles through a right frontal craniotomy. Postoperatively, headache and vomiting resolved in all cases and behaviour changes improved in one. CONCLUSIONS: Endoscopic fenestration is the treatment of choice for septum pellucidum cysts, where it results in immediate relief of the mass effect of the cyst and in the remission of the associated symptoms. We suggest a posterior approach through a right occipital burr hole. It allows one to easily cannulate the occipital horn, which is usually larger than the frontal one, thus avoiding the risk of damaging the vascular and neural structures surrounding the foramen of Monro. Besides, the endoscopic trajectory is in our opinion more direct. The two-window technique, with fenestration of the cyst into both lateral ventricles, improves the chances of long-term patency.
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4/21. Rare sellar region tumors.

    We present three cases of rare intracranial midline tumor in the sellar region, often mimicking pituitary adenomas clinically. We describe their symptoms, radiological and pathomorphological features. The first case is a pituitary adenoma producing growth hormone with ganglion cell differentiation. In addition, a rare intracranial granular cell tumor of sellar region and germinoma of pituitary fossa are also presented. All tumors were resected and histologically analyzed. Their biological behaviour was favorable with a 10-year follow-up demonstrating no recurrent tumor mass.
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5/21. Pituitary adenoma proliferative indices and risk of recurrence.

    Twenty unselected pituitary adenomas have been examined for proliferative indices (PIs), and anterior pituitary hormone expression. All but two of the tumours were non-functional with proliferative indices from less than 0.1 to 0.5%. Two tumours were null cell adenomas with PIs less than 0.1 and 0.2%. The PIs of the three recurrent tumours was less than 1.0%. Gonadotrophin and TSH immunoreactivity was heterogenous and was found in 12/20 (60%) of the tumours. There was no significant relationship between PI, hormone expression or any other measured parameter. The biological behaviour of pituitary adenomas with a PI of less than 0.1% is uncertain, but those with a PI of greater than 0.1% are more likely to recur although longer follow-up is needed to confirm this.
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6/21. Intrasellar pituitary schwannoma.

    Schwannomas are common in the central nervous system, but only a few cases in the pituitary fossa have been reported. Described here is a case of an intrasellar schwannoma associated with headaches and behavioural changes, which responded to subtotal excision of the lesion. The diagnosis of intrasellar schwannoma was made on the basis of a constellation of microscopic findings, especially histologic features and immunohistochemical markers. The clinical and laboratory presentation, surgical procedure used and microscopic findings are compared with those in the literature.
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7/21. Ablative thyroid treatment for thyrotoxicosis due to thyrotropin-producing pituitary tumours.

    BACKGROUND: thyrotropin (TSH)-secreting pituitary adenomas (TSHomas) are rare tumours that can be invasive. It has been suggested that thyroid surgery or radioiodine treatment should not be considered in patients with such tumours as these treatments may facilitate rapid and aggressive tumour expansion. AIM: To study the effects of thyroid ablative treatment on tumour size and thyroid status in two patients with TSHomas in whom the size of the adenoma was clearly documented before treatment was started. methods: patients studied were: (1) a female patient with a TSHoma who declined to undergo pituitary surgery and underwent a total thyroidectomy instead and (2) a male patient who opted for radioiodine treatment for his recurrent TSHoma. Changes in tumour size on serial magnetic resonance imaging scans, and restoration of euthyroidism were studied. RESULTS: No marked changes in tumour size or features of aggressiveness occurred in these patients over periods of 8 and 12 years. Euthyroidism was restored and maintained in both patients. CONCLUSIONS: Ablative thyroid treatment can be a safe and successful option to treat TSHomas, but long-term and close follow-up of these patients is mandatory to ensure that the size and behaviour of the tumours do not change markedly.
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8/21. Management of adipsia by a behavioural modification technique.

    Adipsia combined with diabetes insipidus after hypothalamic damage may produce major difficulties in clinical management. If there is an associated memory impairment it may be impossible to teach self-regulation of fluid balance, necessitating long-term hospital supervision. The successful use of a behaviour modification technique to achieve independent drinking and allow discharge from hospital into the community is described in a patient with adipsia, diabetes insipidus and memory impairment resulting from the removal of a craniopharyngioma.
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9/21. Nelson's syndrome and behavioural changes reversed by selective adenomectomy.

    A man with Nelson's syndrome developed severe associated behavioural and psychiatric problems, which completely resolved following selective resection of his pituitary tumour. It is postulated that ACTH, beta-endorphin, or related peptides may have caused these problems.
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10/21. A case of multiple endocrine adenomatosis type 1.

    A case of pituitary adenoma associated with pancreatic islet cell tumor is presented. A 29-year-old man with symptoms of confusion and abnormal behaviour was admitted to the neurosurgical department. He was diagnosed as suffering from multiple endocrine adenomatosis (MEA) type 1. Screening of his family members revealed that his mother had high levels of gastrin, glucagon and parathormone and his father had a high level of gastrin. The pituitary adenoma and pancreatic tumor were removed satisfactorily. family members as well as the patient should be checked periodically.
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