Cases reported "Hypopituitarism"

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1/44. Rathke's cleft cyst as a cause of growth hormone deficiency and micropenis.

    Rathke's cleft cyst has rarely been reported in pediatric patients, and such cysts are usually found by chance, in 2-33% of routine necropsies, as they have not interfered with pituitary function. In general, they are intrasellar with a single layer of ciliated cuboidal or columnar epithelium containing mucoid material. The age range in which symptomatic Rathke's cleft cysts occur is between 30 and 60 years. This paper reports an 8.1-year-old boy presenting with growth hormone deficiency and micropenis attributable to hypogonadotropic hypogonadism (HH), implying altered pituitary function since intrauterine life. At this age (before puberty) the diagnosis of HH can be made by means of the LHRH agonist stimulation test, since conventional LHRH is not able to discriminate HH from a normal prepubertal child. To our knowledge, this is the first case of micropenis caused by Rathke's cleft cyst interfering with gonadotropin and growth hormone secretion since intrauterine life.
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ranking = 1
keywords = cleft cyst, cleft, cyst
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2/44. hyponatremia-induced metabolic encephalopathy caused by Rathke's cleft cyst: a case report.

    Rathke's cleft cysts are sometimes associated with aseptic meningitis or metabolic encephalopathy due to hyponatremia. We treated such a case manifest by lethargy, fever and electroencephalographic abnormalities. A 68-year-old man was admitted to our ward after experiencing general malaise, nausea and vomiting and then high fever and lethargy. On admission, he was drowsy and had nuchal rigidity and Kernig's sign. Physically, he was pale with dry, thickened skin. He had lost 5.0 kg of body weight in the last month. His serum sodium was 115 mEq/l. He had a low serum osmotic pressure (235 mOsmol/l) and a high urine osmotic pressure (520 mOsmol/l). His urine volume was 1200-1900 ml/24 h with a specific gravity of 1008-1015. The urine sodium was 210 mEq/l. He did not have an elevated level of antidiuretic hormone. Electroencephalograms showed periodic delta waves over a background of theta waves. With sodium replacement, the patient become alert and symptom free, and his electroencephalographic findings normalized. However, the serum sodium level did not stabilize, sometimes falling with a recurrence of symptoms. magnetic resonance imaging clearly delineated a dumbbell-shaped intrasellar and suprasellar cyst. The suprasellar component subsequently shrunk spontaneously and finally disappeared. An endocrinologic evaluation showed panhypopituitarism. The patient was given glucocorticoid and thyroxine replacement therapy, which stabilized his serum sodium level and permanently relieved his symptoms. A transsphenoidal approach was performed. A greenish cyst was punctured, and a yellow fluid was aspirated. The cyst proved to be simple or cubic stratified epithelium, and a diagnosis of Rathke's cleft cyst was made. The patient was discharged in good condition with a continuation of hormonal therapy. Rathke's cleft cyst can cause aseptic meningitis if the cyst ruptures and its contents spill into the subarachnoid space. Metabolic encephalopathy induced by hyponatremia due to salt wasting also can occur if the lesion injures the hypothalamus and pituitary gland.
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ranking = 1.000770795753
keywords = cleft cyst, cleft, cyst
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3/44. Panhypopituitarism associated with diabetes insipidus in a girl with a suprasellar arachnoid cyst.

    We report on a female patient with a large suprasellar arachnoid cyst (3.5 x 2.5 cm) combined with right optic nerve hypoplasia. She developed growth hormone deficiency and hypothyroidism at the age of 8.5 years, adrenal insufficiency at the age of 11 years, diabetes insipidus and hypogonadotropic hypogonadism at the age of 15 years. When last seen at the age of 19 years she was extremely obese ( 5.9 BMI SDS). The endocrine picture suggests that arachnoid cysts might be involved in far more complex hypothalamic-pituitary disturbances than previously thought.
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ranking = 0.0015415915060474
keywords = cyst
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4/44. Cystic lymphocytic hypophysitis, visual field defects and hypopituitarism.

    A 45-year-old man presented with anterior pituitary failure, requiring thyroxine, hydrocortisone and androgen replacement. An MRI scan revealed a large cystic pituitary mass and thickening of the pituitary stalk. Over three years, diabetes insipidus and bitemporal hemianopia developed and the cystic mass had enlarged on MR scanning. Transphenoidal resection was performed with normalisation of the visual fields. histology revealed lymphocytic hypophysitis, which is rare in men. The presentation with cystic enlargement is unique.
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ranking = 0.00077079575302368
keywords = cyst
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5/44. Repeated hemorrhage in ciliated craniopharyngioma--case report.

    A 49-year-old female presented with a ciliated craniopharyngioma manifesting as repeated intratumoral hemorrhage. Histological examination suggested that the hemorrhage originated from the many thin blood vessels in the cyst wall stroma associated with inflammation. Symptomatic hemorrhage in cystic craniopharyngioma may mimic pituitary apoplexy but the etiology is quite different. Minor hemorrhage may recur unless the cyst wall is totally removed.
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ranking = 0.00077079575302368
keywords = cyst
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6/44. Rathke's cleft cyst associated with hypophysitis: MRI.

    We report a symptomatic Rathke's cleft cyst associated with hypophysitis in a 61-year-old woman. We demonstrate the MRI features and discuss the pathophysiology. To the best of our knowledge this is the first description of a Rathke's cleft cyst shrinking after high-dose steroid therapy.
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ranking = 0.85692262978485
keywords = cleft cyst, cleft, cyst
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7/44. Intra-sellar salivary gland-like pleomorphic adenoma arising within the wall of a Rathke's cleft cyst.

    Salivary gland rests occur in the posterior lobe of the pituitary gland near or often communicating with the Rathke's cleft or its cystic subdivisions, and are usually incidental autopsy findings. They are attributed to the oropharyngeal development of the Rathke's pouch and may rarely give rise to salivary gland-like tumors in the sella. We present a pleomorphic adenoma, a rare tumor of the sellar region, that has not been previously recognized in association with Rathke's cleft cyst. It occurred in a 44-year-old man who presented with hypopituitarism and reduced vision. magnetic resonance imaging showed a sellar mass with suprasellar extension which was totally removed. It consisted of segments of a cyst wall lined by focally ciliated columnar of cuboid epithelium containing goblet cells. An eosinophilic granular material with cholesterol clefts represented the contents of the cyst. Within its wall there was a tumor with ductal structures and non-ductal varied cellular components including hypercellular areas of spindle and ovoid cells forming interlacing fascicles. Individual cells appeared to float in abundant mucinous material. The appearances were those of a salivary gland pleomorphic adenoma arising within the wall of a Rathke's cleft cyst. The myoepithelial nature of non-ductal tumor cells was confirmed with immunocytochemistry. The existence of seromucous glands communicating with the Rathke's cleft remnants, explains the concomitant occurrence of the tumor and the cyst. This rare neoplasm from salivary gland rest should be considered in the differential diagnosis of unusual sellar and suprasellar tumors.
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ranking = 0.8604042540063
keywords = cleft cyst, cleft, cyst
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8/44. Panhypopituitarism due to pituitary cyst of Rathke's cleft origin--two case reports.

    INTRODUCTION: Rathke's cleft cysts are cystic sellar and suprasellar lesions, characteristically lined by a single layer of ciliated cuboidal or columnar epithelium. CLINICAL PICTURE: We report 2 patients who presented with gastrointestinal symptoms and were initially investigated for dyspepsia. However, attention was subsequently drawn to persistent hyponatraemia that led to the diagnosis of panhypopituitarism due to Rathke's cleft cyst. TREATMENT: Transsphenoidal surgery followed by drainage of the cyst and partial excision of the cyst wall in both patients. OUTCOME: No recurrence of the lesions over a mean follow-up of 16 months. There has been an improvement of the hypothalamo-pituitary-adrenal axis in 1 patient and the hypothalamo-pituitary-thyroid axis and visual fields in the other. CONCLUSION: Symptomatic Rathke's cleft cysts are rare and can occasionally cause panhypopituitarism. Ideal management of these cysts is unclear, but aspiration followed by partial excision of the cyst wall seems the best initial option.
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ranking = 0.43404556421917
keywords = cleft cyst, cleft, cyst
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9/44. Symptomatic Rathke's cleft cyst with amyloid stroma.

    A patient with panhypopituitarism and visual field defects due to a Rathke's cleft cyst is presented. These cysts are commonly found in random pituitaries examined at necropsy, but rarely produce symptoms. Subtle endocrine deficiencies, however, may now be uncovered more frequently with modern diagnostic techniques. An unusual and unreported feature of this cyst was an apudamyloid stroma. This would imply that cells derived from the neural crest participate in the formation of the cyst. Recognition of these cysts at the time of operation is important in avoiding confusion with pituitary adenomas and unnecessarily aggressive treatment.
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ranking = 0.71512991915807
keywords = cleft cyst, cleft, cyst
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10/44. Neuroendocrinological aspects of primary empty sella.

    Neuroendocrinological aspects of 42 patients (33 women, 9 men) with primary empty sella confirmed by CT, cysternography and/or MR imaging were analyzed. The prominent symptoms were headache, visual disturbances and hypertension, occurring primarily in obese women (84.5%). patients underwent dynamic endocrine testing consisting of insulin-induced hypoglycemia and anterior pituitary stimulation tests GnRH and TRH. Variable degree of pituitary dysfunction was observed in 28 (66.6%) patients. In this study 20 (47.6%) patients were presented with latent hypopituitarism, while manifest hypopituitarism, requiring replacement therapy, occurred in 8 (19%) patients. Mild hyperprolactinaemia was found in 3 patients. Even 14 (33.3%) patients had no evidence of endocrine dysfunction. Often mentioned diabetes insipidus and rhinoliquorrhea were not reported in this study.
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ranking = 0.00025693191767456
keywords = cyst
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