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Nine cases of symptomatic Rathke's cleft cyst are reported. Their most frequent signs and symptoms included headache, chiasmal syndrome and hypopituitarism, while one of the cases developed a sudden onset of headache and vomiting following diabetes insipidus. Endocrinological findings showed a decreased ACTH, gonadotropin and growth hormone more frequently while there were 2 cases of hyperprolactinemia and 1 case of diabetes insipidus. In a neuroradiological examination, a plain skull X-ray showed 5 cases of ballooning of the sella turcica, and a CT scan demonstrated a low to high density of the cyst and 2 cases of marginal enhancement of the cyst. MRI mostly demonstrated a well delineated mass at the sella extending mostly into the suprasellar region and a low to high intensity of the cyst in the T1-weighted image. Two cases were marginally enhanced after gadolinium DTPA administration. The pathological examination, done on 6 cases, showed either single or multiple layers of the epithelium which were mostly ciliated. The epithelium was positive in PAS and Alcian blue in all cases and a histochemical examination showed 3 cases to be positive in EMA and 2 cases positive in CEA. A resection of the cyst wall and an opening of the cyst is thus recommended in symptomatic cases. Therefore, the transsphenoidal approach should be the choice of treatment in an intra- and suprasellar extension of the cysts with sellar enlargement.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Symptomatic Rathke's cleft cyst: a clinicopathologic study of 9 cases]. 816 47

A case of symptomatic Rathke's cleft cyst, showing symptoms due to hyponatremia, is reported. The patient was a 68-year-old woman with complaints of vomiting, diarrhea and somnolence. She had severe hyponatremia (109 mEq/l). Magnetic resonance image showed a cystic mass in the intra- and suprasellar region. A right fronto-temporal craniotomy was performed and the cyst was partially resected through the pterional approach. The cyst compressed the chiasma and anterior hypothalamus upward. In the cyst, there was xanthomatous fluid and hematoma. On histopathological examination, the cyst epithelium was diagnosed as Rathke's cleft cyst with craniopharyngioma component. These findings suggested that rapid compression to the anterior hypothalamus by intracystic hemorrhage caused hyponatremia.
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PMID:[Symptomatic Rathke's cleft cyst presenting with hyponatremia: a case report]. 892 20

Unexpected autopsy findings are presented of a patient who died suddenly after a 6-month history of progressive headaches, nausea, and vomiting. A ruptured Rathke's cleft cyst (RCC) was identified within the adenohypophysis, with a chronic inflammatory reaction in surrounding pituitary and overlying hypothalamus. A brisk lymphoplasmacytic response was also seen in the cavernous sinuses bilaterally, identical to the pathology reported for idiopathic painful ophthalmoplegia, also called Tolosa-Hunt syndrome (THS). The pathogenesis of THS has not been elucidated; based on the findings in this report, it is suggested that some THS cases may result from a hyperimmune response to RCC rupture with extension into one or both cavernous sinuses. Although prompt alleviation of symptoms with corticosteroid treatment is generally encountered with THS, recurrence of symptoms is not uncommon. A careful search for a ruptured RCC should be undertaken in atypical cases of THS, with possible consideration of surgical intervention.
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PMID:Ruptured Rathke's cleft cyst: a possible cause of Tolosa-Hunt syndrome. 910 Nov 12

The association of large pituitary mass, lack of clinical syndromes of pituitary hypersecretion, hypopituitarism and visual field defects suggests the diagnosis of nonfunctioning pituitary adenoma, but the same characteristics can be present in patients affected by other tumorous lesions, cysts, inflammatory processes or vascular disease. The management of these patients depends on a correct diagnosis. A 53-year-old woman was admitted for nausea, vomiting and severe hypotension. For three months she had complained of weakness, sleepness, skin-dryness and loss of weight. Imaging and endocrine evaluations revealed an intra and extrasellar mass causing hypopituitarism without diabetes insipidus. Histological examination of the tissue obtained at transsphenoidal surgery showed a Rathke's cleft cyst, surrounded by areas of noncaseous granulomatous tissue with scattered multinucleated giant cells of foreign body type, similar to a sarcoid lesion. Other systemic sarcoidosis localizations were absent. After two years of full well-being, the patient reported a sudden visual impairment, due to sarcoidosis involvment of the prechiasmatic tract of the optic nerve, that promptly improved with corticosteroid treatment. This report emphasizes the overlap of signs and symptoms between non functioning tumors and nontumoral masses of hypothalamic-pituitary region and underlines the fact that a correct diagnosis is feasible only on histopathological basis. Although, occasionally, the coexistence of Rathke'cyst with pituitary adenoma has been reported, to the best of our knowledge, this is the first report of the association between Rathke's cleft cyst and noncaseating granuloma tissue. Finally, the remission of neurolgical symptoms following corticosteroid therapy confirms this treatment as a valid medical approach and suggests its use in a short therapeutic trial when the diagnosis is doubtful.
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PMID:Granulomatous sarcoidotic lesion of hypothalamic-pituitary region associated with Rathke's cleft cyst. 912 87

We report a case of a seventy-year-old woman with syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by Rathke's cleft cyst. She experienced nausea, vomiting, diarrhea, and headache and disturbance of consciousness induced by hyponatremia at a serum sodium level of 100 mEq/l. In spite of severe hyponatremia, urinary sodium excretion was not suppressed and serum osmolality (270 mOsm/kg) was lower than urine osmolality (304 mOsm/kg), and arginine vasopressin (AVP) remained within normal range. SIADH was diagnosed because she was free from other diseases known to cause hyponatremia such as dehydration, cardiac dysfunction, liver dysfunction, renal dysfunction, hypothyroidism, and adrenal insufficiency. Cranial computed tomographic (CT) scan and cranial magnetic resonance (MR) imaging showed a cystic lesion of approximately 2 cm in diameter in the pituitary gland. These images suggested that the cystic lesion was a Rathke's cleft cyst, which was the cause of SIADH. Water restriction therapy normalized her serum sodium concentration and improved her symptoms. After one year, she suffered from general fatigue, appetite loss, fever, and body weight loss (5 kg/2 months). She had neither hypotension nor hypoglycemia, but her serum sodium level was low and serum cortisol, ACTH, and urine free cortisol were very low. Therefore, secondary adrenal insufficiency was suspected and diagnosed by stimulation tests. After start of hydrocortisone replacement therapy (10 mg/day), her symptoms disappeared. In conclusion, Rathke's cleft cyst should be kept in mind as a potential cause in a patient with SIADH, hypopituitarism, and/or adrenal insufficiency.
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PMID:Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and adrenal insufficiency induced by rathke's cleft cyst: a case report. 1107 19

We describe an 18-year-old female who complained of general weakness, nausea, vomiting, headache, and lightheadedness. On physical examination, she was euvolemic without visual or neurological deficits. The striking biochemical abnormality was hyponatremia (125 mmol/l). This hyponatremia met the laboratory diagnostic criteria for the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Two litres of normal saline were given per day for 4 days and this did not correct her hyponatremia. A spontaneous diuresis (6.6 l) developed in 1 day, causing a rise in her PNa of 26 mmol and a final PNa of 152 mmol/l. Magnetic resonance imaging revealed a dumbell-shaped intrasellar and suprasellar cyst. During transsphenoidal surgery, a Rathke's cleft cyst (RCC) lined with columnar epithelium containing mucoid material was resected. We speculate that the growing RCC may have produced critical compression over the stalk, thus contributing to the transition from SIADH with hyponatremia to transient central diabetes insipidus with hypernatremia.
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PMID:Rathke's cleft cyst presenting with hyponatremia and transient central diabetes insipidus. 1271 31

We report the case of a 29-yr-old woman who first presented an aseptic meningitis at the beginning of a pregnancy. She was admitted one month later with headaches and vomiting. Panhypopituitarism with diabetes insipidus was diagnosed. Magnetic resonance imaging (MRI) data suggested the existence of lymphocytic infundibulohypophysitis, with inflammation of the suprasellar area. No new symptoms were noticed until 6 months later when this patient pointed out troubles of the visual field, due to a compression of the optic chiasma. Three boluses of 1 g methylprednisolone were prescribed, with no effects. After delivery, the defects of the visual field increased. A neurosurgical intervention was decided. Diagnosis of Rathke's cleft cyst (RCC) was made. We concluded that this patient presented a rupture of a RCC, which occurred at the beginning of pregnancy, associated later with panhypopituitarism with diabetes insipidus, due to a probable hypophysitis. The end of the pregnancy was marked by consequences of an increased volume of the RCC. To our knowledge, this case is the first described during pregnancy.
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PMID:Hypophysitis associated with a ruptured Rathke's cleft cyst in a woman, during pregnancy. 1669 3

Rathke's cleft cyst (RCC) is a congenital, benign, epithelial tumor and mainly occurs in sellar region and occasionally in suprasellar region; ectopic RCC is exceedingly rare. We report an uncommon RCC in cerebellopontine angle (CPA) associated with RCC apoplexy and investigated the possible hypothesis of its origin. A 12-year-old female student was admitted to hospital for 3-month history of vertigo, headache, nausea, and vomiting and aggravated for 1 week. Magnetic resonance imaging (MRI) revealed a space-occupying lesion with short T1 and long T2 signals in the left CPA and an intracystic floating nodule with hypointensity on T1- and T2-weighted imaging. The patient underwent the total tumor removal via the retrosigmoid approach with a good recovery. Primary RCC was confirmed by pathology. Follow-up MRI showed no recurrence 3.5 years after craniotomy. Primary RCC can occur in CPA and present special neuroimaging features associated with RCC apoplexy. We presumed that a mimicking mechanism of ectopic craniopharyngioma in CPA leads to the formation in the present case. Microsurgical resection is the optimal strategy for management. Further research and longer follow-up are helpful to better understanding the pathogenesis and development history of RCC in CPA.
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PMID:Primary Rathke's cleft cyst in the cerebellopontine angle associated with apoplexy. 2071 84

We describe a rare case of transient thyrotoxicosis secondary to painless thyroiditis accompanied by panhypopituitarism caused by ruptured Rathke's cleft cyst. A 32-year-old man presented with vomiting and diarrhea. Laboratory data showed that he had transient hypercalcemia, primary thyrotoxicosis due to painless thyroiditis and panhypopituitarism. The sellar magnetic resonance imaging showed cystic macroadenoma. He underwent surgical exploration. Histological examination showed a ruptured Rathke's cleft cyst. Our case suggests that, although rare, it is important to recognize the possibility of coexistence of hypopituitarism in patients with primary thyrotoxicosis.
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PMID:Transient thyrotoxicosis accompanied by panhypopituitarism caused by ruptured Rathke's cleft cyst. 2218 92

Pituitary apoplexy is defined as a sudden loss of blood supply to the pituitary gland, leading to tissue necrosis and hemorrhage. Its clinical symptoms are characterized by sudden onset of headache, nausea, vomiting, ophthalmic symptoms and hormonal dysfunction. A 65-year-old woman presented with left-sided ptosis and blurred vision. These ophthalmic symptoms gradually worsened for one month without headache, visual acuity and field deficit. Neuro-ophthalmic examination revealed left oculomotor nerve palsy. Magnetic resonance imaging (MRI) revealed a round mass lesion in the left cavernous sinus, which was initially suspected as thrombosed cerebral aneurysm or hemorrhagic Rathke's cleft cyst. The mass lesion was finally diagnosed as pituitary apoplexy. The patient underwent trans-sphenoidal surgery and oculomotor nerve palsy improved after the surgery. Early diagnosis and treatment including surgical decompression are crucially important in patients with oculomotor nerve palsy in pituitary apoplexy, but the symptoms of pituitary apoplexy may slowly progress. It should be noted that pituitary apoplexy could be misdiagnosed as cerebral aneurysm or Rathke's cleft cyst.
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PMID:Pituitary apoplexy presenting atypical time course of ophthalmic symptoms. 2257 5


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