Symptomatic Rathke's cleft cyst mimicking suprasellar arachnoid cyst

The authors report an atypical case of symptomatic entirely suprasellar Rathke's cleft cyst mimicking suprasellar arachnoid cyst. A 55-year-old male was introduced to our hospital complaining of bitemporal hemianopsia. CT and MRI demonstrated a cystic mass located entirely in the suprasellar cistern and to compress the optic nerve and mammillary body. The cystic wall was not enhanced in MRI. CT cisternography showed the suprasellar non-communicating cyst with cistern. The hormonal function was slightly disturbed by the pituitary compression. Under the diagnosis of suprasellar arachnoid cyst, a left front temporal craniotomy was performed to resect the suprasellar mass. The surgical specimen consisted ciliated epithelium and was diagnosed Rathke's cleft cyst. After operation, he recovered completely free.     

Rathke's cleft cyst--report of three cases

Three cases of symptomatic Rathkes' cleft cyst are described. Case 1. A 51-year-old man was admitted to the hospital for evaluation of intermittent headache, in April, 1985. He was neurologically free, but skull films disclosed a ballooning of the sella with thinning of the dorsum sellae. CT scan showed enlargement of the sella, but no abnormal density area in the sellar region. MRI revealed a round mass with high signal intensity located just posteriorly to the pituitary body. By a transsphenoidal approach, a thin-walled cyst was found at the posterior portion of the sella. Turbid mucinous fluid and the capsule of the cyst were subtotally removed. Histologic section of the specimen demonstrated that its wall was composed of a loose fibrous tissue lined by a single layer of ciliated cuboidal epithelium containing some goblet cells. Electron microscopy showed ciliated cuboidal cells, cells with microvilli, cells with light and large granules, and basal cells abutting on the basal lamina. Case 2. A 33-year-old female was referred to the hospital with complaints of severe headache and decreased visual acuity 0.02 in the left and 0.1 in the right, in July, 1985. Visual field examination revealed bitemporal hemianopsia. She had amenorrhea, galactorrhea and polyuria. CT scan and metrizamide CT cisternography detected a low density mass in the suprasellar region. Endocrinological studies disclosed hyperprolactinemia with partial hypopituitarism. She had a right frontal craniotomy and a suprasellar cyst was subtotally removed. Histologically, a cystic wall was lined by pseudostratified columnar epithelium supported by a loose fibrous tissue.(ABSTRACT TRUNCATED AT 250 WORDS)     

A case of Rathke's cleft cyst in association with a ruptured aneurysm of the anterior cerebral artery mimicking pituitary apoplexy

We present a rare case of a Rathke's cleft cyst in association with a ruptured aneurysm of the anterior cerebral artery (ACA). A 44-year-old man suffered from sudden onset of headache. Initial computed tomographic (CT) scan revealed a high-density mass lesion in the suprasellar region and a diffuse high-density area in the basal cistern. Using emergent magnetic resonance imaging (MRI), we found a cyst showed homogeneously high and iso-intensity on T1 and T2-weighted image, respectively. The cyst showed no enhancement of the cyst wall, but on Gd-DTPA, it was shown to compress the normal pituitary gland. Angiography showed an aneurysm at the A1 portion of the left ACA. Based on/these findings, we were able to diagnose Rathke's cleft cyst and a ruptured aneurysm. An operation was performed through the interhemispheric approach. The suprasellar cystic mass compressed the optic nerves and chiasm upward. Neck clipping of the aneurysm and opening of the cyst were performed. We confirmed the cause of the subarachnoid hemorrhage as being a ruptured aneurysm at the A1 portion of the left ACA. Histological diagnosis was Rathke's cleft cyst. Postoperative course was uneventful. There has been only one reported case of Rathke's cleft cyst in association with a ruptured aneurysm. When we encounter a case presenting subarachnoid hemorrhage with suprasellar mass and intracerebral aneurysm, we must discriminate between ruptured aneurysm and pituitary apoplexy in the acute stage as the cause of the subarachnoid hemorrhage. If the mass is Rathke's cleft cyst, we speculate that the cause of the subarachnoid hemorrhage is a ruptured aneurysm, because there are no reports of Rathke's cleft cyst with subarachnoid hemorrhage.     

A case of Rathke's cleft cyst in association with bilateral unruptured aneurysms of internal carotid artery

Recent progress in diagnostic imaging techniques, such as MRI and CT, has shown that Rathke's cleft cyst is more common than once thought. However, few cases have been reported in which a cerebral arterial aneurysm was merged with a symptomatic Rathke's cleft cyst. We present a case of bilateral internal carotid aneurysms associated with a symptomatic giant Rathke's cleft cyst. A 66-year-old-woman with a visual disturbance was admitted to our hospital. CT and MRI showed a large mass in the frontal base to the suprasellar region. The mass showed isosignal intensity in T1-weighted images (WI), a ringed enhancement with Gd-DTPA, and hyperintensity in T2WI. An angiography showed bilateral A1 elevation but no tumor stain. In addition, unruptured aneurysms appeared in the inside back C2 portion of the bilateral internal carotid arteries. For these lesions, a bifrontal craniotomy was performed. In a single operative approach, the mass lesion was removed and the aneurysms were successfully clipped. The aneurysms adhered strongly to the wall of the mass. The histological diagnosis of the mass was Rathke's cleft cyst. A precise pre-operative understanding of the anatomical structure and a careful operative procedure are important for the treatment of these complex lesions.     

Endoscopic endonasal removal of an intra-suprasellar Rathke's cleft cyst: case report and surgical considerations.

An endoscopic endonasal approach was performed to remove an intra-suprasellar Rathke's cleft cyst. Rathke's cleft cyst are benign lesions, rarely diagnosed because they are often asymptomatic. To the best of our knowledge, at least 475 cases of Rathke's cleft cysts have been reported. They seem to arise from remnants of Rathke's pouch, an invagination of the stomodeum. A 52-year-old woman, complaining of bilateral frontal headaches, was operated on by using an endoscopic endonasal approach, for an intra-suprasellar tumor. The pre-operative diagnosis was non-functioning pituitary adenoma. Intra-operatively a creamy-coloured viscous tissue was found. After the removal of the cyst contents and of the capsule, the suprasellar structures were seen well. The chiasmatic cistern, the chiasm, the pituitary stalk and the pituitary gland were visualized with 0 and 30 degree endoscopes. The pathological findings showed a well-differentiated cuboidal epithelium. The diagnosis was Rathke's cleft cyst. No post-operative complications were observed. The endoscopic technique was particularly suitable in this case, both for the Rathke's cleft features and for an excellent outcome. The Rathke's cleft cyst was easily removed by suction and the cyst wall was entirely removed with curettes and pituitary punches. The hypophysis was distinguished from the cyst and was preserved. The surgical manoeuvres were all done under direct visual control. The absence of nasal packing and of breathing difficulties made comfortable the post-operative outcome. Thus, the endoscopic endonasal approach can be considered the favourite technique in case of either intra- and/or suprasellar Rathke's cleft cysts.     

A case of non-functional pituitary adenoma associated with Rathke's cleft cyst

A very rare case of non-functional pituitary adenoma associated with Rathke's cleft cyst is reported. A 42-year-old male suffering from visual disturbance and headache was admitted. Visual acuity was 1.2 on the right and 0.5 on the left, and visual field examination showed bitemporal hemianopsia. Endocrinological study disclosed that the basal serum level of prolactin was elevated slightly to 52.6 ng/ml, whereas those of other hormones were within normal limits. MRI showed a dumbbell-shaped cystic sellar mass extending to the suprasellar region beyond the diaphragm sellae, which consisted of two isolated components around the sellar turcica. At the middle of May, the tumor was partially removed by bifrontal craniotomy. The suprasellar tumor tightly adhered to the frontal lobe and the optic nerve, the tumor was grayish cyst and cyst fluid from the suprasellar mass had the appearance of motor oil and the intrasellar tumor contained old hematoma. Histological findings revealed that the suprasellar mass was Rathke's cleft cyst and the intrasellar mass was diagnosed as pituitary adenoma (non-functional). Visual acuity was improved following surgical operation. So far twenty-three cases of pituitary adenoma associated with Rathke's cleft cysts have been reported in the literature, but there was only one case reported of a non-functional pituitary adenoma associated with a Rathke's cleft cyst. Etiology and pathogenesis of the coexistence of non-functional pituitary adenoma and Rathke's cleft cyst in the present case were not elucidated.     

A case of spontaneous rupture of a suprasellar cystic mass

The author reported a case of a spontaneous rupture of a suprasellar cystic tumor. A 67-year-old man complained of bitemporal homonymous hemianopsia. His visual acuity was 0.02 on the right side and 0.04 on the left side. Skull XP revealed no abnormal findings, but brain CT scan showed a cystic mass without calcification in the suprasellar region. Brain MRI at the same lesion site depicted a low-intensity mass on T1-weighted image without gadolinium enhancement, and a high-intensity mass on T2-weighted image. The patient was treated conservatively because of complications such as diabetes mellitus, cerebral infarction and old myocardiac infarction. MRI taken 5 years after the initial MRI revealed disappearance of the suprasellar cystic mass. However, the patient's neurological findings, including visual signs, revealed no deterioration. His physical and radiological findings had remained uneventful. In this report, we reviewed the literatures about spontaneous rupture of suprasellar cystic tumors. It was considered that in this case, according to the neurological, radiological and CSF findings, the suprasellar cystic mass might be a Rathke's cleft cyst or arachnoid cyst.     

Symptomatic Rathke's cleft cysts located entirely in the suprasellar region: review of diagnosis, management, and pathogenesis

Three cases of an entirely suprasellar symptomatic Rathke's cleft cyst, two of which were associated with normal sella turcicas, are reported. In all cases, the cysts caused compression of the optic chiasm, and two produced hypothalamic dysfunction. The diagnosis of these entirely suprasellar masses was enhanced by metrizamide cisternography. Two cases were treated by frontal craniotomy and one was treated transsphenoidally, with good results in all cases. The radiology, pathology, and surgical treatment of these unusual cases is presented. An embryological pathogenesis for the occurrence of an entirely suprasellar Rathke's cleft cyst is discussed.     

An entirely suprasellar symptomatic Rathke's cleft cyst: case report.

An entirely suprasellar symptomatic Rathke's cleft cyst in a 21-year-old woman is reported. An unusual feature of this cyst was the fact that the subepithelial tissues were composed of pituitary gland cells. High resolution magnetic resonance imaging is sensitive in the detection of subtle suprasellar abnormalities. We review the literature regarding the embryological pathogenesis of Rathke's cleft cyst, with special reference to the entirely suprasellar type.     

Epithelial cysts of the central nervous system: report of two cases

Two cases of epithelial cyst are reported. Case 1. A 68-year-old female visited our hospital with a complaint of decreased visual acuity, 0.04 in the left eye, in September 1986. Visual field examination showed bitemporal hemianopsia. CT scan demonstrated nonenhancing cystic lesion involving the suprasellar region. By a right frontotemporal craniotomy, the suprasellar cyst was explored. The wall of the cyst was partially removed to relieve pressure against both optic nerves and chiasma. Histologically, the cyst wall was lined with a single layer of non-ciliated cuboidal epithelium. Electron microscopic study revealed a number of microvilli, continuous basement membrane and desmosomes. Case 2. A 40-year-old female was admitted to our hospital because of sensory disturbance in the left hand for several years. She had an episode of convulsive seizure with a loss of consciousness. Neurological examination revealed hypesthesia of the left hand. CT scan showed non-enhancing right frontoparietal cystic lesion compressing the right lateral ventricle to the left. By a right frontoparietal craniotomy, the cystic lesion was explored. The cyst existed beneath an arachnoid membrane and contained CSF-like clear fluid. The cyst wall was resected, as far as possible, to obtain communication with normal subarachnoid CSF space. Histologically, by light and electron microscopic study, the cyst was considered to be an epithelial cyst. A correct diagnosis is difficult because other cystic lesions such as arachnoid cysts and craniopharyngioma are of similar low density and appear the same on CT scan. It is impossible to diagnose such an epithelial cyst by relying on neuroradiological study alone.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pituitary hyposecretion and hypersecretion produced by a Rathke's cleft cyst presenting as a noncystic hypothalamic mass

A 53-year-old man presented with polydipsia, polyuria, lightheadedness on standing, and syncope. Visual field deficits suggesting left optic tract compression and pituitary dysfunction consisting of diabetes insipidus, hyperprolactinemia, and anterior pituitary insufficiency were diagnosed. On the computed tomography scan, an isodense, ring-enhancing, parasellar mass was localized primarily in the suprasellar region and also extended into the sella. It was believed that the pituitary hormone hypersecretion and hyposecretion were due to hypothalamic dysfunction from the mass which was initially believed to be a craniopharyngioma. At surgery a Rathke's cleft cyst was resected. The unusual presentation of this Rathke's cleft cyst presenting as a hypothalamic lesion is discussed.     

Secondary pan-hypophysitis caused by rupture of Rathke's cleft cyst: case report]

We report a case of secondary hypophysitis caused by rupture of Rathke's cleft cyst. A 30-year-old woman was admitted to our hospital with complaints of polyuria and left visual acuity impairment. These symptoms were preceded by aseptic meningitis one month prior to admission. An MRI on admission showed a suprasellar cystic mass and a swelling pituitary stalk. The mass was partially resected via the transsphenoidal approach. Pathological diagnosis was adeno- and neuro-hypophysitis and ruptured Rathke's cleft cyst. These findings strongly suggested that the hypophysitis was caused by rupture of Rathke's cleft cyst. After the operation, the patient's visual acuity improved with steroid administration. An MRI performed 5 months after the operation showed marked reduction of the mass. To our knowledge, there have been only 5 reports of secondary hypophysitis caused by ruptured Rathke's cleft cyst.     

A case of prolactinoma in close association with Rathke's cleft cyst

We report a case of Rathke's cleft cyst in pituitary tumor. A 31-year-old woman admitted to our hospital complained of visual disturbance, headache, amenorrhea and galactorrhea. Serum prolactin level was 3,060 ng/ml. By means of CT scan and MRI, we found suprasellar-extending tumor to have cystic component in the center of the tumor. Transsphenoidal surgery revealed grayish jelly-like content in the cyst. Histologically tumor cells were composed of chromophobic, basophilic and eosinophilic cells. The cyst wall in the pituitary adenoma was composed of ciliated or non-ciliated columnar epithelium intermingled with goblet cells, being similar to Rathke's cleft cyst. Except for the cells of the cyst wall, most of the tumor cells were immunoreactive for prolactin. As for intermediate filament, tumor cells in both solid and cystic portions showed positive immunoreactivity for cytokeratin and GFAP, and not for vimentin and neurofilament. These results suggest that the nature of the intracytoplasmic filament in the pituitary tumor with Rathke's cleft cyst may be not only keratin but also GFAP.     

Neuroendoscopic treatment for Rathke's cleft cyst: transventricular approach to suprasellar cystic lesion

We report a case of a Rathke's cleft cyst, which was successfully treated by neuroendoscopy. The endoscopic procedure for the intra-suprasellar cystic lesion used the transventricular approach, similar to that of third ventriculostomy. A 66-year-old woman suffered from headache, nausea and constriction of the visual field. Magnetic resonance imaging (MRI) on admission showed an intra-suprasellar cystic lesion. Biopsy of the cyst wall and fenestration of the floor of the third ventricle was performed using an endoscopic transventricular approach. The cyst content was totally aspirated, using a suction system which was connected to the operation channel of the endoscope. The histological diagnosis was Rathke's cleft cyst. No post-operative complication was observed. The endoscopic transventricular approach is safe and most suitable in terms of minimum invasiveness for the intrasuprasellar cystic lesion. We consider that neuroendoscopic transventricular approach will become the common surgical method of choice for treating a suprasellar cystic mass lesion.     

Suprasellar xanthomatous Rathke's cleft cyst.

A case of a symptomatic suprasellar Rathke's cleft cyst in a 35-year-old woman is presented. The cyst wall and contents showed large collections of xanthomatous cells. With the exception of a few pituitary hormone-positive cells, immunohistochemical and ultrastructural features of the lining epithelium in this case of Rathke's cleft cyst were indistinguishable from colloid cysts of the third ventricle with similar secondary changes.     

Clinical, radiographic, and pathological features of symptomatic Rathke's cleft cysts

The clinical, radiographic, and pathological findings in 155 patients with symptomatic Rathke's cleft cysts are discussed. Eight patients were treated by the authors and 147 were collected in a review of the literature. This lesion occurred more often in female than male patients by a 2:1 margin, and the mean age at presentation was 38 years. The average patient had been symptomatic for nearly 3 years at the time of treatment, with the most common symptoms and signs being pituitary dysfunction, visual disturbances, and headaches. Affected children generally were pituitary dwarfs. The sella was enlarged in 80% of cases, and the cyst was situated in both an intrasellar and a suprasellar location in 71%. Computerized tomography revealed a low-density cystic mass with capsular enhancement in one-half of the cases. A variable appearance was seen with magnetic resonance imaging. Partial excision and drainage of the cyst by the transsphenoidal approach is the recommended treatment, as the recurrence rate is low. Most symptoms and signs improved or resolved following surgery with the exception of hypopituitarism and diabetes insipidus. The cyst lining was usually composed of ciliated cuboidal or columnar epithelium. Theories as to the origin of Rathke's cleft cysts are also discussed.     

Case of calcified prolactinoma combined with Rathke's cleft cysts

A case of chromophobe adenoma with capsular calcification combined with Rathke's cleft cysts is presented. A 28-year-old woman presented with a seven-year history of amenorrhea. Several months before admission to our department of neurosurgery on November 6, 1982, she developed galactorrhea and difficulty in reading because of visual failure. Neurological examination on admission revealed bitemporal hemianopsia, visual disturbance, left optic atrophy. Plain skull films and CT scan showed suprasellar capsular calcification. The patients hormonal status was assessed pre- and postoperatively. The basal serum prolactin (PRL) level was elevated at 790ng/ml, but other hormone basal plasma levels were within normal limits despite decrease in FSH, LH, The LH, FSH and PRL demonstrated a blunted response to LH-RH (100 micrograms). Both TSH and PRL demonstrated a blunted response to TRH (500 micrograms). The GH showed no response to insulin tolerance test (0.1 U/kg). On November 24, right frontal craniotomy was performed. A grayish bulging mass was noted surrounded by a calcified layer(2-3 mm) in the suprasellar region. When incised this calcified hard layer, showed multi-small cysts with yellow fluid. Under the cyst layer, there was a soft mass which was curetted easily. Histologically, under the ossified layer, there were multi-small cysts, lined by a single layer of ciliated columnar epithelium. The central soft mass was regarded as a chromophobe adenoma with no calcified body. Following partial removal of the tumor, there was prompt improvement in clinical signs and plasma PRL level.(ABSTRACT TRUNCATED AT 250 WORDS)     

Atypical presentation of a dermoid cyst with porencephaly

We report a case of a dermoid cyst in the parasellar area associated with porencephaly. A 22-year-old man presented with headache and visual disturbance. Non-enhanced brain CT showed a high density mass in the parasellar area and a low density area in the left temporal lobe. On gadolinium-enhanced T1-weighted images, a partial rim enhancement was present in the parasellar region. A presumptive diagnosis of Rathke cleft cyst, craniopharyngioma, non-function pituitary adenoma, or germ cell tumor was considered. A left frontotemporal craniotomy was performed and the tumor was removed almost totally. The tumor was well-capsulated and contained whitish, milky debris. The histologic diagnosis was dermoid cyst. The temporal lobe lesion was porencephaly. The postoperative course was uneventful. Their most typical aspect is the presence of a low-density mass on CT, allowing an easy radiologic diagnosis of dermoid cyst containing lipids. Enhancement following contrast administration is uncommon. The hyperdensity may be related to the high protein content of the lesion. The hyperdense aspect of the lesion on CT, and the presence of rim enhancement made the radiologic diagnosis of a dermoid cyst difficult in this case. A case of dermoid cyst associated with porencephaly has not yet been reported in the literature.     

A case of recurrent-rupture dermoid cyst

A 58-year-old man was seen because of general convulsions. The neurological findings were normal at his first visit, but CT revealed a low density mass in the right hypothalamus with calcification. T1-weighted image revealed multiple high intensity lesions in the subarachnoid space. Only anti-epileptic drugs were prescribed. He was readmitted one year later because of a traffic accident due to convulsions. CT and MRI revealed other new lesions in the bilateral anterior horn. After four uneventful years, follow-up CT revealed a dilation of the third ventricle. MRI using the fat suppression method showed enhancement around the main tumor and the wall of the lateral ventricles and stenosis of the aqueduct. Right frontotemporal craniotomy was performed. Thickening of the arachnoids and floating lipid droplets were seen in the sub-arachnoid space. A yellowish tumor was found in the suprasellar region, adhering to the internal carotid artery. The tumor contained soft yellowish tissue and hair. Only partial removal was carried out because of severe adhesion to perforators. The lamina terminalis was opened after tumor removal. The postoperative course was uneventful except for transient diabetes insipidus.     

Granulomatous change of the pituitary stalk caused by Rathke's cleft cyst: a case report

We report a case of granulomatous change of the pituitary stalk caused by Rathke's cleft cyst. A 50-year-old woman complained of thirst and polyuria in December 2000. In January 2001, the patient was suspected to have diabetes inspidus, and MR Imaging showed a suprasellar mass of the pituitary stalk. The mass was totally resected by the trans-sylvian approach. Pathological diagnosis was Rathke's cleft cyst with subsequent granulomatous change. To our knowledge, there have been 11 cases of Rathke's cleft cyst and subsequent granulomatous change. In the literature, diabetes inspidus was not improved by surgical procedures. However, we suggest that the mass should be totally resected, because some cases in literature showed visual disturbance or hypo-pituitarism under observation.