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A case of Empty Sella secondary to Rathke's cleft cyst, successfully operated on, is reported. Asymptomatic cysts of Rathke's pouch are fairly frequent autopsy observations. They can cause optic-chiasmatic and endocrine symptomatology or, very rarely, an Empty Sella syndrome if the cyst is very large.

An unusual case of a symptomatic Rathke's cleft cyst (RCC) initially demonstrated by computed tomography is presented. A discussion of RCC and its differentiation from other disease entities that may mimic it are included.

The case of a prolactin-secreting pituitary tumor with multiple cyst formation is described. Ultrastructurally, the cyst-lining cells were ciliated and closely resembled those of Rathke's cleft cyst but contained secretory granules, and no basal lamina formation was seen between the adenoma cells and the lining cells. Immunohistochemical study revealed that the adenoma cells consisted of both prolactin-secreting cells and growth hormone-secreting cells. The lining cells were immunoreactive with the antiserum to cytokeratin. No S-100 protein-positive cells were seen. The origin of this tumor is discussed.

Nine cases of pituitary tumors composed of mixed tissue elements of adenohypophysial adenoma and Rathke's cleft cyst are reported. All cases were associated with hyperprolactinemia and were managed by transsphenoidal adenomectomy with good results. The clinical and pathological features are presented, and embryological pathogenesis for the occurrence of this unique association is discussed in connection with the high incidence of immunoreactive S-100 protein cells identified in tumor tissues. Patients with this kind of pituitary tumor can be managed in the same fashion as patients with other types of pituitary adenoma.

A 19-year-old girl with pituitary insufficiency and a large sella turcica was found to have granulomatous hypophysitis in association with a Rathke's cleft cyst. We think that the inflammatory process represents a foreign body reaction to leakage of cyst contents, with destruction of pituitary tissue.

A 71-year-old woman with symptomatic Rathke's cleft cyst is reported. She had visual disturbance, diabetes insipidus and hypopituitarism. Imaging studies with computed tomography and magnetic resonance image revealed the presence of suprasellar mass. After the transsphenoidal surgery and treatment with DDAVP, her symptoms showed an improvement.

Two cases of Rathke's cleft cyst which produced symptoms of compression of the optic chiasm are described. The first case has been followed for five years since the operation. No evidence of recurrence has been noticed. The prognosis after a partial removal of the cyst wall seems to be good with this lesion. In the second case, there was clinical and laboratory evidence of hypopituitarism and the CT scan revealed suggestive findings to differentiate the cyst from a pituitary adenoma.

A wide variety of benign cystic lesions is known to occur in both adults and children. Recent advances in diagnostic radiology have facilitated meaningful surgical intervention in most instances. It is convenient to classify these cysts into two groups. The first group includes those that arise from defects wholly within the central nervous system. Among these are certain static lesions such as cystic cavities arising from infarcts and other destructive lesions. Some of these are observed to communicate with the subarachnoid space or ventricle. The progressive lesions in this group include arachnoid cysts, ependymal cysts, cystic hemangioblastoma, cystic cerebellar astrocytoma, and certain infectious processes. The second group is constituted by cysts resulting from the intrusion of non-nervous tissue into the neuroaxis, usually in the midline. These are expanding congenital lesions, although some become symptomatic only in the adult. Among these are teratomas, dermoid cysts, epidermoid cysts, craniopharyngiomas, Rathke's cleft cysts, and other epithelial cysts apparently derived from the upper respiratory or the intestinal tract. Colloid cysts of the III ventricle are usually considered to be neuroectodermal in origin, but certain features suggest an endodermal origin.

A case of symptomatic Rathke's cleft cyst was studied preoperatively with x-ray computed tomographic and magnetic resonance imaging. A Rathke's cleft cyst may be differentiated from other cystic-appearing sellar masses.

A case of symptomatic Rathke's cleft cyst simulating an arachnoid cyst is presented. Although computed tomography and magnetic resonance imaging showed that the cyst content was identical to cerebrospinal fluid, there was faint contrast enhancement in part of the wall. Histological observation suggested that the somewhat vascular connective tissue associated with nests of pituitary cells might have contributed to the enhancement. This type of Rathke's cleft cyst requires further clinical and neuroradiological studies.

✓ A rare case is reported in which a symptomatic Rathke's cleft cyst was studied by light and electron microscopy and tissue culture. The findings are compared with those of a craniopharyngioma studied in the same way. The patient was a 26-year-old woman presenting with headache, chiasmatic syndrome, and hypopituitarism. A cyst containing a mural nodule was partially removed and an Ommaya reservoir placed in the operative site for further treatment. The cyst wall was composed of connective tissue and three kinds of epithelial cells: non-ciliated squamous, ciliated columnar, and mucous-secreting cells. The morphology of these cells in vitro was similar to prickle cells seen in craniopharyngioma and the epidermis. It is concluded that both Rathke's left cyst and craniopharyngioma originate in remnants of Rathke's pouch, but at times may show some histological differences.

✓ An abscessed Rathke's cleft cyst was removed in a 50-year-old woman who had had headaches and episodic fever for 3 years and had been on intermittent methotrexate therapy for psoriasis for 4 years. There was clinical and laboratory evidence of panhypopituitarism. Erosion of the sella turcica was present. The patient has remained asymptomatic on replacement therapy. A brief review of abscess formation in the region of the pituitary gland is presented.

HE subject of cystic lesions in, around, and above the sella turcica has at times been confusing from the standpoint of the neurosurgeon, who is particularly concerned with the nature of a lesion at the time of its operative exposure and how radical must be its excision. Cystic adenomas of the pituitary present very little problem. They are usually readily identified, clearly contain tumor tissue and are treated as neoplasms. The delineation of other cysts is sometimes difficult and confusion is compounded by pathological terminology. Thus, the so-called craniopharyngiomatous cyst contains cholesterin crystals, while the cholesteatoma or epidermoid actually contains keratohyalin granules and lacks cholesterin. The term craniopharyngioma, which has gained acceptance, is probably a poor name for this lesion, since Rathke's pouch develops from the stomodeum and not from pharyngeal elements. The major problem which confronts the surgeon and clinical pathologist is that of distinguishing between a craniopharyngioma with cyst formation (hypophysial stalk) and an intrasellar epithelial cyst, which is actually a cyst of the pituitary gland.

2021

Morphogenesis of craniopharyngeal derivatives of the neurohypophysis found in 14 Fischer 344 (F344) rats was studied. The incidence of the craniopharyngeal derivatives was 0.17% in male (7 out of 4,200) and 0.16% in female (7 out of 4,450) F344 rats. Neither a sex-related difference in their incidence nor a strain-related difference in their morphological features was observed. Craniopharyngeal derivatives were composed of aberrant epithelial structures consisting of serous acinar and tubular and fusiform cell structures, and most of these derivatives were associated with Rathke's cleft cysts, which are suggestive of a congenital background. The acinar structures were positive for periodic acid-Schiff reaction and negative for Alcian blue stain. Immunohistochemically, cells forming these structures were positive for cytokeratin, and basal cells of the acinar or tubular structures and some of the fusiform cells showed positive staining for alpha-smooth muscle actin. Electron microscopically, these spindle-shaped basal cells had intracytoplasmic myofilaments with focal density in their cytoplasm, and they were regarded to be myoepithelial cells. These findings strongly indicate that the craniopharyngeal derivatives are not a neoplastic lesion but rather are a developmental aberration derived from the stomatodeum, which is known to be the origin of both nasal and oral epithelial tissues, including the parotid glands, other than Rathke's pouch.

A 6-year old patient presented with an intra and suprasellar cystic lesion accompanied with impairment of the hypothalamic-pituitary axis and partial hypopituitarism. The most likely cause of sellar lesions in this age group are adamantinomatous craniopharyngioma (adaCP) or Rathke´s cleft cysts (RCCs). AdaCP are characterized by CTNNB1 mutations accompanied with aberrant nuclear beta-catenin expression. RCC show neither nuclear beta-catenin expression nor BRAF mutation. The latter is a hallmark of papillary craniopharyngiomas (papCP) that exhibit remarkable histological similarity with metaplasia of RCC. Diagnosis of the patient was elucidated by CTNNB1 and BRAF mutation screening, utilizing different approaches, as well as histological examination of markers, e.g., beta-catenin, claudin-1, EpCAM and the mutated BRAFV600E protein, which are known to be differentially expressed in sellar lesions. The case presented reveals extraordinary aspects for two reasons. Firstly, the lesion appeared clinically, on MRI, intraoperatively and histologically as RCC with prominent squamous metaplasia, but showing an expression pattern of markers also found in papCP, whilst exhibiting a hitherto undescribed BRAFV600E mutation. This important result documents a supposable transition of RCC metaplasia into a papillary craniopharyngioma (papCP). Secondly, this intriguing case shows unexpectedly that although papCP usually occurs almost exclusively in adults, it can also arise in childhood.

A case of symptomatic Rathke's cleft cyst is described. In spite of the intrasellar component and radiological and clinical evidence of involvement of the pituitary gland, the sella turcica was minimally involved. MRI afforded optimal visualization and localization of the intra- and suprasellar components vis-à-vis the pituitary gland, optic chiasm, infundibular stalk, and carotid arteries.

A woman with secondary amenorrhea was found to have hyperprolactinemia without clinical galactorrhea. Radiological findings of an enlarged sella turcica with displacement of the pituitary stalk were considered consistent with a prolactin macroadenoma. Treatment with bromocriptine corrected the amenorrhea and hyperprolactinemia, and the patient inadvertently became pregnant. However, no complications to the mother or fetus occurred during pregnancy or postpartum. On transsphenoidal surgery three months postpartum, the unexpected presence of a large Rathke's pouch cyst with a microadenomatous or nodular hyperplasia type of prolactin-secreting tumor was observed to account for the preoperative clinical and radiological findings. (JAMA240:471-473, 1978)