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rathke s cleft cyst ( rcc ) is considered to be distended remnants of rathke s pouch , an invagination of the stomodeum .
a few cases with symptomatic rcc have a female preponderance , occurring between 40 to 50 years of age .
commonly , presenting symptoms are related to mass effect , such as headache , visual impairment and hypopituitarism . however , those presenting only diabetes insipidus are uncommon .
a 69-year - old female housewife complained of a sudden onset of dry mouth , polyuria and polydypsia two months prior to admission .
two months ago , she developed abdominal pain , loose stool , general weakness , malaise , nausea , dry mouth and polyuria . on admission ,
neurologic examination was done but no particular pathologic findings , including visual field defect , were observed . on admission , she showed chronic ill - looking appearance , a dehydrated tongue and dry skin .
urine volume was 35004500 ml a day and urinalysis showed a specific gravity of 1.005 , urine osmolarity 227 mosm / kgh2o , na 101 mmol / l , k 10 mmol / l and cl 84 mmol / l .
plasma osmolarity showed 294 mosm / kgh2o , na 146 mmol / l , k 3.3 mmol / l and cl 111 mmol / l .
the study demonstrated partial hypopituitarism ( serum cortisol 1.33 g / dl , acth 16.86 pg / ml , total t3 58.8 ng / ml , total t4 4.8 g / dl , tsh 0.24 mlu / ml , lh 1.38 mlu / ml , fsh 0.24 mlu / ml , adh 1.28 ng / ml ) .
anterior pituitary function was investigated with a set of three tests with insulin tolerance , trh and lhrh .
cortisol , tsh , fsh and lh responses were compromised , while prolactin responses were retained ( table 1 ) .
plain skull x - ray film of the sella was normal ( figure 1 ) . magnetic resonance image ( mri ) scan showed isodense signal intensity mass lesion in pituitary fossa with infundibular thickening and no enhancement of rcc with dynamic study was observed ( figure 2 ) .
as the patient presented polyuria , mild headache and hypoosmolar urine , a water deprivation test was done , revealing a pattern of partial central diabetes insipidus ( table 2 ) . in order to obtain an exact diagnosis ,
yellow - colored discharge flowed from the cystic lesion when the anterior pituitary was incised .
the histopathological examination showed mixed inflammatory cell infiltration in the loose connective tive tissue stroma .
focally remained columnar epithelial walls were noted and cytokeratin immunohistochemical staining revealed monolayered columnar epithelium in the cyst wall ( figure 3 , 4 ) .
after operation , the patient was given a physiologic dose of prednisolone , thyroid hormone and desmopressin nasal spray .
five months later , desmopressin replacement was stopped and no more polyuric symptoms have developed .
rcc arises from the remnants of rathke s pouch , an invagination of the stomodeum formed by the fourth gestational week .
normally , rathke s pouch is closed off by proliferation of the anterior and posterior lobe of the pituitary gland , and this lumen forms a thin residual cleft in the gland .
the resulting cleft persists as a cyst lined with columnar or cuboidal epithelium of ectodermal origin .
failure of obliteration of rathke s cleft with proliferation of the lining cells and accumulation of secretions may result in cyst formation between anterior and middle lobes .
reviewed 155 cases of rcc saying that rcc is usually asymptomatic and found in 1322% of normal pituitary glands in autopsy .
however , when it grows more than 1 cm or inflammatory changes develop , various symptoms may occur , especially when it expands to the infundibular portion and suprasellar region .
mechanical compression of the pituitary gland by the cyst , as well as inflammation itself , may play a major role in causing pituitary dysfunction in rcc patients .
lymphoplasmatic inflammation induced by mucus secreted by goblet cells of the cyst wall leads to inflammatory changes of rcc and leakage of cyst contents into the pituitary gland can occur , possibly leading to the development of abscess formation .
eguchi et al . said that rcc greater than 1 cm which develops symptomatic hypopituitarism involving more than two hormones should be treated with a surgical approach as the general recommendation .
the most frequent cases involve hyperprolactinemia , followed by gonadotropin deficiency , pan - hypopituitarism , hypothyroidism and hypocortisolism .
intensity on t1-weighted image but inflammatory change could be shown as isodense or hyperdense intensity like our case .
incision of the cyst was made , yellowish pus - like aspirates flowed from the cyst .
biopsy findings revealed mixed inflammatory cell infiltration in the loose connective tissue stroma and focally remained cyst wall composed of columnar epithelium .
reports 28 cases of symptomatic rcc treated by transsphenoidal operation and only one of those cases ( 3.6% ) presents diabetes insipidus , while 4 patients ( 14.3% ) suffered preoperatively from diabetes insipidus .
it is important to differentiate rcc from other neoplastic lesions , such as craniopharyngioma and pituitary adenoma .
differentiation from pituitary adenoma is important as it is very difficult to distinguish rcc from pituitary adenoma .
the widening of sella turcica was the common point of differentiation in many cases of pituitary adenoma in simple skull image .
yoshida et al . reported that the mean age of the patients was 38 years old , and the highest frequency was in the fifth decade with marked female preponderance .
endocrinological presentation of rcc , such as amenorrhea , is thought to be the major cause of marked female preponderance . with
the advent of mri , asymptomatic or subclinical rccs are now being detected with increasing regularity .
wide removal of the cyst wall should be avoided because of possible damage to the hypothalamus , pituitary , optic nerves and optic chiasm .
transsphenoidal surgery is preferred because there is danger of damage to the hypothalamus and optic apparatus .
surgical treatment is generally recommended even when the patient has mild symptoms or signs such as headache , mild defect of visual field , increased prolactin or a cyst size of more than 10 mm .
patients with small - sized rcc , or even asymptomatic , should be followed up regularly with an mri .
generally , the prognosis after a partial removal of the cyst wall or simple aspiration of the cyst seems to be good even though the cyst recurs .
rathke s cleft cyst(rcc ) , which is located at the intrasellar region , is considered to be the distended remnants of rathke s pouch , an invagination of the stomodeum . 
 lined with columnar or cuboidal epithelium of ectodermal origin , rcc usually contains mucoid material and it is found in 1322% of normal pituitary glands . 
 the cyst rarely leads to the development of symptoms but , when it does , the most common presenting symptoms are headache , visual impairment , hypopituitarism and hypothalamic dysfunction . 
 however , in some cases it presents symptoms of diabetes insipidus , decreased libido and impotence . 
 recently we experienced a case of rcc inflammation presenting with diabetes insipidus and treated with transsphenoidal surgery . to our knowledge , this is the first report of rcc presenting with symptoms of diabetes insipidus in korea .