Cases reported "Pituitary Neoplasms"

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1/37. acromegaly: report of two patients with an unusual presentation.

    The presenting features of functionally active pituitary tumours depend on the specific hormone which is overproduced. growth hormone (GH) producing tumours usually present with the clinical manifestations of acromegaly due to excessive GH secretion or symptoms resulting from mass effects of the enlarging tumour. The changes in physical features and the increase in tumour size are usually insidiously slow and therefore, recognition of the disease is delayed. In this report two patients with acromegaly are described with an atypical presentation due to acute onset of symptoms. The first patient presented with central diabetes insipidus. The diagnosis acromegaly was made on physical examination. The second patient presented with a generalized seizure during sleep. On CT-scanning a large tumour protruding into the left temporal lobe connected to the pituitary gland was seen. immunohistochemistry of the tumour after partial transcranial resection confirmed the clinical diagnosis of acromegaly. At a later stage transsphenoidal resection of the pituitary tumour was performed with full recovery and without loss of pituitary function.
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2/37. Stereotactic radiosurgery, X: clinical isodosimetry of gamma knife versus linear accelerator X-knife for pituitary and acoustic tumours.

    Several review articles have compared gamma unit versus linear accelerator (linac)-based radiosurgery systems, concluding that the dose gradient 'fall-off' at the margin of the target (expressed as the distance between isodoses) is very similar for both techniques as far as single isocentre treatment volumes up to 1.5 cm diameter are concerned, and that the two radiosurgical systems are, in general, comparable. 'Fine tuning' of the gamma unit can be carried out by using multiple isocentre plans, the differential use of small collimator sizes (down to 4 mm) and field weightings, and adroit use of the gamma angle, and selective beam blocking. Multiple isocentre plans, beam modification, restriction of gantry angles and arc lengths, and microcollimation can similarly improve the isodose gradients from linac units. In both instances, the dosimetric advantages occur along selected aspects of the target perimeter border. However, the more frequent use of multiple isocentred 'shots' on the gamma unit achieves greater conformity indices for more complex target volumes, but at the expense of steeper internal dose gradients. We studied two patients with tumours close to or arising from radiosensitive special sensory nerves (optic and cochlear) to compare and contrast fine tuning of the two technologies. In a previously irradiated patient with a pituitary adenoma, the dose gradient achieved at the rostral margin, adjacent to the optic chiasma, was steeper on the gamma unit (due to the concentration of small collimator shots rostrally and beam blocking), which was therefore the dosimetrically preferred technique. In contrast, the vastly smaller internal dose gradient (11% for linac/X-knife versus 100% for Gamma Knife) and the ability to fractionate on the X-knife system, gave a large dosimetric advantage to the X-knife plan in the treatment of an acoustic neuroma, where the intracanalicular component of the cochlear nerve traversed the target volume. This advantage also pertains to the cochlear ramus of the internal auditory (labyrinthine) artery and the facial nerve. Our published work on X-knife radiosurgery of acoustic neuroma has documented improvement of hearing after therapy and may be relevant in this regard. That there are advantages in physical dose distribution and fractionation, producing a reduction in the biological dose in normal tissue, argues for the use of linac technology in acoustic neuromas. Craniopharyngiomas enveloping the optic nerve/chiasma will similarly be better treated by the linac X-knife system. It is apparent that different radiosurgery systems may be indicated in particular neuro-oncological situations.
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3/37. Effect of octreotide acetate on thyrotropin-secreting adenoma: report of two cases and review of the literature.

    OBJECTIVE: To present two cases with thyrotropin-secreting adenoma and the effectiveness of octreotide acetate treatment on their tumor size as well as on thyroid stimulating hormone (TSH) and thyroid hormone levels. case reports: The first case presented with tremor, palpitations and sweating as suggestive of hyperthyroidism, but the other one presented with predominantly headache, while the other symptoms such as palpitation and nervousness were less prominent and he also did not have any thyroid enlargement at physical examination. Thyroid hormone levels in both cases were increased. However, TSH levels were not suppressed thus indicating an inappropriate secretion of TSH. Moreover, TSH levels did not change after T3 and TRH administration, which also contributed to the assumption of an inappropriate TSH secretion. One case had no increase in the TSH alpha subunit level, while this was increased in the other one. Both magnetic resonance imaging and somatostatin receptor scintigraphy revealed that there was a microadenoma (the first case; 6 x 7 mm in diameter)and a macroadenoma (the second case; 14 x 18 mm in diameter). Both patients were placed on a therapy with somatostatin analog octreotide (Sandostatin, Sandoz). octreotide was initially given at a dose of 300 microg daily and then increased gradually up to 600 microg per day. There was some decrease in the levels of TSH and thyroid hormones at first. However, such decreases did not persist with ongoing therapy for 6 months. In addition, there was no change in the tumor size with this therapy at the end. CONCLUSIONS: We conclude that the treatment by somatostatin analogue octreotide may not be an effective means of reducing the pituitary tumor size, though it may be used to reduce TSH and thyroid hormones temporarily.
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4/37. A case of pituitary adenoma associated with McCune-Albright syndrome.

    A 11-year-old boy presented with right temporal hemianopsia and was evaluated of a possible pituitary adenoma. At the age of six, he underwent surgery for facial deformities due to fibrous dysplasia. On admission, he had acromegalic features, was 170 cm tall, weighing 66 kg. The left side of his face was slightly deformed, and a cafe-au-lait spot was found on his right face. Endocrinologic examination revealed elevated basal level of serum GH (103.6 ng/ml, normal < 3 ng/ml) and PRL (259.1 ng/ml, normal < 30 ng/ml). Other endocrine functions were normal. CT showed hyperostosis of the right frontal, occipital, sphenoidal and maxillary bones. magnetic resonance imaging (MRI) revealed a pituitary macroadenoma with intraadenomatous cyst. On the basis of physical, endocrinologic and neuroradiologic examination, our diagnosis was pituitary adenoma with McCune-Albright syndrome. Surgery was performed by subfrontal approach. By light microscopy, the pituitary tumor represented a typical acidophilic adenoma. Immunoreactivity for GH and PRL were evident in most of the adenoma cells. Double immunostaining for GH and PRL demonstrated the co-existence of the two hormones in a few adenoma cells. However the majority of cells expressed only one hormone. After surgery the right temporal hemianopsia improved. Postsurgical endocrinologic examination revealed reduction in basal serum GH and PRL levels. Administration of bromocriptine decreased blood PRL levels but it had a limited action on GH hypersecretion.
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5/37. pituitary apoplexy in a patient with acute myeloid leukemia and thrombocytopenia.

    We describe a 72-year-old woman with a history of acute myeloid leukemia who developed pituitary apoplexy associated with thrombocytopenia secondary to chemotherapy. She presented with new onset severe headache, nausea, vomiting and blurred vision. Initial physical examination was unremarkable. CT scan of the head was initially negative. Upon admission for further work up, She developed a high-grade fever, hypotension and obtundation. Subsequent physical examination revealed bitemporal visual fields defects and decreased visual acuity. Repeat imaging of head revealed a hemorrhagic pituitary mass compressing the optic chiasm. Laboratory results were compatible with the diagnosis of pan-hypopituitary syndrome. She received high dose steroids and was transferred for transnasal sphenoidotomy decompression surgery. The visual defects improved postoperatively. A literature review of pituitary apoplexy is presented. pituitary apoplexy secondary to thrombocytopenia has never been reported.
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keywords = physical examination, physical
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6/37. Pituitary tumor in a woman with a 47,XXX karyotype--case report.

    BACKGROUND: The 47,XXX karyotype is a rare sex chromosome anomaly. This karyotype is usually not associated with a characteristic physical phenotype. CASE REPORT: In presented case a 47 triple X women with pituitary tumor and premature ovarian failure is identified. diagnosis of a 47,XXX individual remains difficult because specific clinical criteria used to identify this condition are not available. CONCLUSIONS: The case described should attract attention to how difficult it is to diagnose properly a genetic disease in young women with correct phenotype.
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7/37. craniopharyngioma and bardet-biedl syndrome. A case report.

    BACKGROUND: bardet-biedl syndrome is a rare disorder and associated with a variety of anomalies. CASE: An 18-year-old woman was referred with primary amenorrhea. Following physical, ophthalmologic, psychiatric, hormonal and radiologic examinations, the diagnosis of both craniopharyngioma and bardet-biedl syndrome was established. CONCLUSION: Although the pathogenesis of hypogonadism in a woman with bardet-biedl syndrome remains unclear, cranial structures, especially the hypothalamus and pituitary gland, should be investigated to reveal any possible abnormalities.
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8/37. Metachronous neurohypophysical immature teratoma occurring 10 years after total resection of pineal mature teratoma.

    An 18-year-old boy presented with an immature neurohypophysial teratoma occurring 10 years after total resection of a mature pineal teratoma through an occipital transtentorial approach in 1989. Thorough histological examination had revealed a mature teratoma. He developed panhypopituitarism and diabetes insipidus in 1999. magnetic resonance imaging revealed a suprasellar tumor occupying the third ventricle. This tumor was totally resected through a frontobasal approach. Histological examination revealed an immature teratoma. This tumor occurred in a different site from the initial tumor and was considered to be de novo and thus a so-called metachronous germ cell tumor. patients with completely resected mature teratoma require extended follow-up, including periodic magnetic resonance imaging, because of the risk of such a metachronous germ cell tumor.
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9/37. Preoperative hyponatremia as a clinical characteristic in elderly patients with large pituitary tumor.

    This study investigated the pathophysiology of preoperative hyponatremia in elderly patients with a large pituitary tumor. The tumor size, initial symptoms, and preoperative pituitary hormonal function were analyzed in 96 patients, consisting of 82 younger than 70 years old (mean age 49.7 years) and 14 older than 70 years old (mean age 72.0 years). There was no difference in tumor size between the two age groups. The initial symptom of all younger patients was visual disturbance. Preoperative hormonal evaluations revealed subclinical panhypopituitarism in four patients (4.9%). Five of the 14 older patients had severe hyponatremia (107-117 mEq/l) based on panhypopituitarism, and four of these five patients showed consciousness disturbance as the initial symptom, initiated by physical and/or psychological stress, or occurrence of intratumoral hemorrhage. Preoperative subclinical panhypopituitarism was found in another patient. The overall occurrence rate of preoperative panhypopituitarism in the older patients was 42.9%. The difference in the frequency of preoperative panhypopituitarism was statistically significant between the two groups. Preoperative severe hyponatremia associated with a large pituitary tumor is characteristic of elderly patients. The number of receptors for adrenocorticotropic hormone in the adrenal cortex decreases during the aging process. Additional physical and/or psychological stress prompts pituitary dysfunction in such patients, causing the manifestation of acute symptoms of adrenal insufficiency based on panhypopituitarism. Primary care using high dose hydrocortisone and electrolyte fluid is critical.
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10/37. sarcoidosis within a pituitary adenoma.

    A 54 year old man presented with frontal headaches for one year. A CT scan of the head revealed a pituitary mass. He denied a change in vision or galactorrhea, but did have decreased frequency of erections and a recent episode of renal stones. On physical exam, the cranial nerves were normal. Visual field exam revealed mild bilateral temporal defects. The genitalia were normal and the testes were soft. Laboratory evaluation revealed: Na, 134 mM/l; K, 6.7 mM/l; Cl, 104 mM/l; HCO3, 22 mM/l; BUN, 47 mg/dl; Cr, 8.3 mg/dl; Ca, 12.5 mg/dl; Phos, 5.5 mg/dl; prolactin, 32.0 ng/ml; T4, 4.46 microg/dl; TSH, 2.07 microU/ml; LH, 18.1 mIU/ml; FSH 3.2 mIU/ml; alpha subunit 1.6 ng/ml; testosterone 255 ng/dl; cortisol, 20.3 microg/dl; cortisol after 250 microg cortrosyn, 38.5 microg/dl (time 60 minutes); growth hormone, 1.4 ng/ml; IGF-1, 47 ng/ml; PTH, <1 pg/ml; 25-hydroxyvitamin D, 14 ng/ml; 1,25-dihydroxyvitamin D, 69 pg/ml. These results were felt to be consistent with a non-PTH-mediated hypercalcemia, such as humoral hypercalcemia of malignancy, or a vitamin d-mediated hypercalcemia, such as lymphoma, sarcoidosis or tuberculosis. Head MRI demonstrated a 3.5 x 3.5 x 2.5 cm heterogeneous mass enlarging the sella, deforming the clivus and compressing the cavernous sinus, basilar artery and left side of the optic chiasm. There was a small focus of high signal in the superior part of the mass on the T1-weighted image from either a proteinaceous cyst with early calcium deposition or sub-acute blood. These radiographic findings were felt to be consistent with a pituitary adenoma. The patient was treated with intravenous hydration and thyroxine 50 microg daily and underwent a transsphenoidal resection of the pituitary lesion. Pathologic examination revealed a pituitary adenoma with multiple granulomas and crystalline material; this was consistent with sarcoid within the adenoma. Post-operatively, the serum LH fell to 5.5 mIU/ml. A subsequent transbronchial biopsy revealed multiple non-caseating granulomas. A serum ACE level was elevated at 132.6 U/l. He received oral prednisone 60 mg daily with resolution of the hypercalcemia. Neurosarcoidosis occurs in 5 to 15% of patients with sarcoidosis and can involve the hypothalamus and pituitary gland. This is the first reported case of sarcoidosis occurring within a pituitary adenoma.
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