Immunohistochemical study of Rathke's cleft cyst

Summary An immunohistochemical study was made of ten cases of asymptomatic and three cases of symptomatic Rathke's cleft cyst. The cysts in the asymptomatic cases had monolayer columnar or cuboidal epithelium. Within the epithelium, cells which were positive for at least one of the pituitary hormones were found. The rate of positive reactions for these six pituitary hormones was between 70% and 100%. In contrast, the cysts in the symptomatic cases had an epithelium which was partly stratified squamous and partly squamous epithelium, and none of the pituitary hormones were found in them. Therefore, when a Rathke's cleft cyst enlarges to the extent that clinical symptoms are produced, we consider that changes have already occurred in structure and function of the cyst epithelium. In addition, we believe there is a tendency for monolayer epithelia to undergo squamous metaplasia and for cells which are positive for pituitary hormones to change into non-granulated cells.     

Rathke's cleft cyst

Rathke's cleft cyst (RCC) are frequent benign cystic sellar lesions. Most RCC are small, intrasellar and asymptomatic. Larger cysts may compress adjacent structures and rarely become symptomatic. Diagnosis is strongly suggested at MRI by the presence of a midline non-enhancing lesion located exactly between the anterior and posterior lobes of the pituitary gland. Even if its signal is variable and related to intracystic protein concentration, it must be homogeneous with no fluid-fluid level. Once a diagnosis of RCC made, routine MR and clinical follow-up is sufficient for incidental asymptomatic cysts whereas the rare symptomatic lesions are neurosurgically resected.     

Preoperative diagnosis of Rathke's cleft cyst

Two cases of Rathke's cleft cyst are presented. The cysts showed as high-density lesions on plain CT with slight enhancement with contrast medium. They appeared as a hyperintense mass in the T1-weighted magnetic resonance images. Preoperative diagnosis is very important because different sellar cystic lesions require different treatment.     

Clinical features of symptomatic Rathke's cleft cyst

To investigate the clinical features of Rathke's cleft cysts (RCCs), we retrospectively analyzed 15 cases with histologically confirmed RCCs. All patients underwent formal testing of visual field, endocrinological evaluation and magnetic resonance imagings. As overall presenting symptoms, endocrine disturbance was the most common symptoms, followed by visual disturbance and headache. Among the endocrine disturbances based on adenohypophysial dysfunction, hyperprolactinemia was most common. Considering the size of RCCs, RCCs could induce hyperprolactinemia only when the cysts became large enough to compress the infundibular system. Our series showed relative high incidence of pituitary dwarfism and diabetes insipidus (DI). These facts indicated that RCCs could evoke hyposecretion of growth hormone in young patients and DI in aged patients by direct compression of the pituitary gland in the early stage of progression. All cases who had headache had no other symptoms. We could not prove the evidence that RCCs could induce headaches in these cases. This might be suggested that headache could not be a sole symptom in cases of RCCs.     

Rathke's cleft cyst: a clinical and radiographic review

We report the case of a 62-year-old woman, who had a 4-year history of progressive visual acuity deficit. On neurological examination, visual acuity was 5/20 in the right and 4/20 in the left eye. A hormonal study revealed hypophyseal hypofunction. CT and MRI scans showed an intra-suprasellar cystic lesion, hyperintense in T1 and hyperintense in T2-weighted sequences. At surgery, the cyst wall was opened and the cavity placed in communication with the subarachnoid space. Histological examination showed a Rathke's cleft cyst. After four years of follow-up the patient's visual deficit remained unchanged, whereas the results of the hormonal assays were normal. An MRI scan confirmed the absence of the cyst. We review 216 reported cases of Rathke's cyst and discuss its pathogenesis, clinical features and treatment.     

Growth hormone secreting adenoma with a Rathke's cleft cyst

A very rare case of a growth hormone-secreting pituitary adenoma with a Rathke's cleft cyst is reported. Operative findings, histology and immunohistochemistry showed that both entities to be totally separate and unrelated. These findings suggest that the coexistence of a pituitary adenoma and a Rathke's cleft cyst in our case is merely a coincidence.     

A case of Rathke's cleft cyst with apoplexy

We report a case of Rathke's cleft cyst associated with cholesterin granuloma in an 8-year-old girl with apoplexy. She was admitted to our hospital in April 1996 because of repeated headache and deep ophthalmic pain, without any visual disturbance. Computed tomography (CT) of the pituitary demonstrated an intrasellar isodense mass extending to the suprasellar cistern. Magnetic resonance imaging (MRI) showed a high-intensity mass on both T₁- and T₂-weighted images. The preoperative diagnosis of this lesion was Rathke's cleft cyst associated with a craniopharyngioma and/or hemorrhage. Transsphenoidal microsurgery was performed, and a bloody coffee-like serous and mucinous-yellowish substance was evacuated. Curettage of the wall removed the yellowish hard mass and soft membranous tissue. Histological examination of this tumor revealed a Rathke's cleft cyst with cholesterin granuloma. Received: 28 April 1997 Revised: 25 August 1997     

Rathke's cleft cyst presenting as pituitary apoplexy.

Sellar lesions mainly constitute pituitary adenomas, craniopharyngiomas and benign cysts. Rathke's pouch cyst is a developmental sellar and/or suprasellar cystic lesion lined by a single layer of ciliated cuboidal or columnar epithelium, which rarely be comes symptomatic. The authors present an interesting case of intrasellar Rathke's pouch cyst, with a presenting feature of acute pituitary apoplexy. This was a 19 year old healthy male who had developed sudden headache and visual disturbance. Neuro-radiological imaging revealed a mass in the sella. Via transsphenoidal approach a haemorrhagic intrasellar cystic lesion was removed and was confirmed as a haemorrhagic Rathke's cleft cyst by histopathological examination. Interesting clinical presentations and the neuroimaging findings are described and discussed.     

Acute, monocular vision loss from Rathke's cleft cyst

Rathke's cleft cysts are benign, non-neoplastic sellar lesions that are often asymptomatic. The rare patient with symptoms may present with pituitary dysfunction, headache, or visual problems. Visual deficits are generally consistent with a chiasmal syndrome as associated with other sellar lesions. Therefore, the most commonly described deficit is bitemporal hemianopsia. We describe a 16-year-old female patient who presented with rare, rapidly progressing monocular blindness without signs of apoplexy or hemorrhage. She had complete return of vision after surgical decompression. The rapid loss and recovery of vision suggest that ischemic factors, rather than deformation of the optic apparatus, contributed to her symptoms. The relevant literature is reviewed.     

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. Received: 5 October 1998 Revised: 8 November 1998     

Clinicopathological study of Rathke's cleft cysts

The clinical, radiological and pathological characteristics of Rathke's cleft cyst are analyzed and compared with those of ciliated and goblet cell craniopharyngioma (a subset of papillary craniopharyngioma) to clarify the clinicopathological differences between these conditions. We analyzed 42 patients with Rathke's cleft cyst and 3 patients with ciliated and goblet cell craniopharyngioma. Cyst contents and MRI findings of the cyst generally reflected changing pathology of Rathke's cleft cyst. Turbid and less viscous cyst content and heterogeneous T1 and/or T2 signals on MRI sometimes represented predominance of squamous epithelium in the cyst wall. Pathological study disclosed smooth transition from ciliated columnar epithelium typical for Rathke's cleft cyst, via ciliated squamous epithelium, to squamous epithelium typical for craniopharyngioma in the same patients. Ki-67 immunostaining disclosed that the proliferative index of squamous epithelium of Rathke's cleft cyst is significantly higher than that of simple or pseudostratified epithelium of Rathke's cleft cyst (p < 0.05). In conclusion, our extensive study on Rathke's cleft cyst revealed that the high possibility of progression from Rathke's cleft cyst, in which either the cyst content or cyst wall are atypical or squamous epithelium is predominant, compared to ciliated and goblet cell craniopharyngioma and again to papillary craniopharyngioma.     

有症状的Rathke's裂隙囊肿的临床特点及手术治疗

目的 探讨有症状的Rathke's裂隙囊肿术前术后内分泌功能紊乱情况及处理方法,以及术后复发问题.方法 对11例经病理证实的Rathke's裂隙囊肿患者的临床表现、影像学特点、内分泌改变及手术治疗方法进行叫顾性分析.结果 4例术前头痛患者术后均缓解,6例术前视力障碍术后缓解5例,9例术前性功能障碍及月经紊乱术后好转6例,而乖体功能低下及尿崩症不易恢复.在12～66月(平均34.5月)随访中,经颅全切除6例末见复发,而经蝶手术5例中 2例大部切除者复发,复发2例病理上均合并慢性炎症浸润及局灶性鳞状上皮细胞.结论 Rathke's裂隙囊肿临床及影像学表现各异,术前确诊常常存在困难.术前术后患者的内分泌功能障碍需要进行全面的评价及激素替代治疗.术中尽量追求囊肿的全切除并取得足够的病理组织对明确诊断、判断预后有重要的意义,对于合并有鳞状细胞及炎性细胞浸润的患者术后应注意密切随访.     

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.     

Rathke's cleft cyst presenting with hyponatremia and transient central diabetes insipidus

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.     

MR imaging study of edema along the optic tract in patient with Rathke's cleft cyst]

We report an 80-year-old woman who was admitted to our hospital with symptoms due to diabetes insipidus. Magnetic resonance (MR) imaging demonstrated a sellar/suprasellar cystic lesion with marginal enhancement and the thick pituitary stalk. The MR imaging depicted edema spreading along the optic tract on fluid-attenuated inversion recovery (FLAIR) images. Upon neurological examination at the time of admission, there were no abnormal findings affecting the field of vision or visual acuity. In endocrinological examination, the basal plasma values of pituitary hormones were within normal range except for that of prolactin, which was 47.9 ng/ml. The preoperative diagnosis was craniopharyngioma, and the intrasellar mass was partially removed by the endoscopic transnasal transsphenoidal approach. Postoperative histopathological examination revealed Rathke's cleft cyst associated with squamous metaplasia. Lymphocytic infiltration was also confirmed in both the anterior and posterior pituitary lobe. The postoperative course was satisfactory. Edema spreading along the optic tract was reported as a characteristic MR imaging finding for diagnosis of craniopharyngiomas or optic nerve glioma. However, it is suggested that edema of the optic pathway seems to be caused not only by craniopharyngioma but also other suprasellar lesions. It was a rare case of secondary lymphocytic hypophysitis caused by Rathke's cleft with edema along the optic tract.     

Chordoma coexisting with Rathke's cleft cyst: Case report and literature review

Both chordoma and Rathke's cleft cyst are relatively rare diseases in the central nervous system. In this paper we report the first case of a chordoma coexisting with a Rathke's cleft cyst. A 49‐year‐old man presented with a 19‐month history of distending pain, movement dysfunction and diplopia of the left eye. The preoperative diagnosis was consistent with chordoma with cystic change. Final pathological diagnosis of chordoma coexisting with Rathke's cleft cyst was made according to histological and immunohistochemical studies and the clinical and radiological features are discussed. Considering the close relationship between the notochordal tissue and Rathke's pouch during early embryogenic development, a possible mechanism is also discussed with the literature review.