Rathke's Cleft Cyst Presenting Research Articles

Unraveling the Mystery of Rathke's Cleft Cyst Presenting with Hyponatremia: A Case Report with a Comprehensive Review of Literature

Abstract Background Rathke's cleft cyst (RCC) is a benign cystic lesion that is commonly discovered incidentally and remains asymptomatic in most cases. However, its association with the syndrome of inappropriate antidiuretic hormone (SIADH) secretion leading to hyponatremia (HN) is rare and has only been sporadically reported in the medical literature. In this article, we present a unique case of RCC manifesting with HN and discuss the diagnostic and management challenges encountered in a neurosurgical context. Additionally, we provide a comprehensive review of existing literature on RCC presenting with HN to enhance our understanding of this rare presentation. Case Description A 56-year-old woman with acute-onset blurry vision, headaches, and low fluid intake was diagnosed with euvolemic HN secondary to SIADH. Further evaluation revealed an intrasellar cystic lesion consistent with RCC, which was successfully resected through endoscopic transnasal transsphenoidal surgery, resulting in a complete recovery without the need for hormone replacement. Conclusion The most likely explanation for the HN due to SIADH in this case is the release of accumulated antidiuretic hormone (ADH) due to compression by the cyst and the irritating effect of inflammation at this location. Accurate evaluation and classification of HN are essential for proper diagnosis and management, considering the rarity of RCC presenting with HN. A multidisciplinary approach to treatment can lead to favorable functional outcomes; however, further research is necessary to better comprehend this unique clinical entity and optimize neurosurgical approaches.

Rathke's cleft cyst presenting with recurrent aseptic meningitis and inflammatory apoplexy: illustrative case.

Rathke's cleft cyst (RCC) is a benign sellar/suprasellar lesion often discovered incidentally. Rarely, symptomatic cases can present with headache and may exhibit concomitant aseptic meningitis or apoplexy. The authors describe a patient with an RCC presenting with recurring episodes of aseptic meningitis and ultimately inflammatory-type apoplexy. A 30-year-old female presented with three episodes of intractable headaches over 2 months. Each episode's clinical picture was consistent with meningitis though cerebrospinal fluid cultures, and viral tests remained negative. Imaging demonstrated a sellar lesion, initially thought to be coincidental. On the third presentation, there was rapid interval growth of the lesion, adjacent cerebritis, and new endocrinopathy. Resection was then performed via an endoscopic endonasal approach. Pathology showed an RCC with acute and chronic inflammation and no evidence of hemorrhage. Cultures were negative for organisms. The patient received several weeks of antibiotic treatment with the resolution of all symptoms and no recurrence. Recurrent aseptic meningitis with apoplexy-like symptoms is a rare presentation of RCC. The authors propose the term inflammatory apoplexy to describe such a presentation without evidence of abscess, necrosis, or hemorrhage. The mechanism is unclear although may be due to intermittent microleakage of cyst contents into the subarachnoid space.

A rare case of isolated IgG4-related hypophysitis with Rathke's cleft cyst presenting as panhypopituitarism.

We report a rare case of biopsy-proven isolated immunoglobulin G4 (IgG4)-related hypophysitis and Rathke's cleft cyst (RCC) presenting as panhypopituitarism. A 54-year-old Caucasian female presented with symptoms of slurred speech, altered mental status, polyuria and polydipsia and was found to have panhypopituitarism. Brain MRI showed a suprasellar mass with suspected intralesional hemorrhage. She underwent trans-sphenoidal resection due to MRI evidence of compression of the optic chiasm and left optic nerve. Preoperatively, she was started on hydrocortisone, levothyroxine and desmopressin. Histopathology demonstrated a RCC with adjacent lymphoplasmacytic hypophysitis with numerous IgG4-immunoreactive plasma cells. Hydrocortisone was stopped at 10 months after confirming hypothalamic-pituitary-adrenal (HPA)-axis recovery and desmopressin was stopped at 1 year. There was recurrence of a cystic mass at 1 year follow-up. Over 4 years of follow-up, she continued to require levothyroxine, and the mass remained stable in size. In order to begin to understand how this case's unique histopathological presentation influences clinical presentation, pituitary imaging and prognosis, we present an accompanying literature review. Isolated IgG4 hypophysitis and coexisting Rathke's cleft cyst is a rare condition, which presents a diagnostic challenge. Recognizing its characteristic features can assist with early recognition and initiation of therapy to promote optimal outcomes. Further studies investigating the mechanisms promoting co-occurrence of these entities and their effect on prognosis are needed.

Rathke cleft cyst presenting as recurrent pituitary apoplexy: An unusual case presentation and clinical course

Rathke cleft cyst presenting as recurrent pituitary apoplexy: An unusual case presentation and clinical course

SAT-476 A Case of Rathke's Cleft Cyst Presenting with Hyperprolactinemia

CASE: A 15 year-old G0P0 female with no significant PMH was referred to Endocrinology for oligomenorrhea and hyperprolactinemia. Menarche was at age 13 and she subsequently had oligomenorrhea for a year. She endorsed headaches and fatigue upon climbing a flight of stairs but denied vision changes, galactorrhea, hirsutism, SOB, and constipation. She was not on any psychotropic medications.The patient was hemodynamically stable with a normal physical examination including affect and cognition. Labs revealed prolactin-402 ng/mL (N=3-24 ng/mL), monomeric prolactin-277 ng/mL (macroprolactin=31%), cortisol-9.2 ug/dL one hour after cosyntropin, and DHEA-54.8 ug/dL (N=110-433.2ug/dL). TSH, LH, FSH, sex hormone binding globulin, testosterone, HCG, insulin-like growth factor, 17OH-progestereone were all within normal limits. MRI with and without contrast revealed a 1.5 x 1.8 x 1.8cm sellar lesion with characteristics most consistent with Rathke’s cleft cyst (RCC) with mass effect on the infundibulum and optic chiasm. The patient was started on cabergoline 0.25mg twice a week and Neurosurgery was consulted. Surgical resection was recommended. DISCUSSION: We present a rare case of symptomatic hyperprolactinemia in an adolescent female with radiologic evidence of RCC. RCC is an uncommon and usually asymptomatic intrasellar lesion, but pituitary dysfunction has been reported in 39% to 100% of these cases with prolactin levels generally less than 200 ng/mL. This hyperprolactinemia has been postulated to occur due to pituitary stalk compression from the sellar mass causing a decrease in dopamine (prolactin inhibitor) delivery. Possible explanations for RCCs with grossly elevated prolactin levels include: (1) coexisting pituitary adenoma, (2) cystic adenoma, and (3) pituitary stalk effect. (1) and (2) are the simplest explanations but radiologic features of adenomas were not seen on MRI. In our case, prolactin was higher than the typical levels seen in stalk compression raising concern for either coexisting pituitary adenoma or cystic adenoma although these are rare. This finding combined with hypocorticolism and radiologic evidence of mass effect warranted surgical intervention. CONCLUSION: Symptomatic RCC is rare, but clinicians should be aware that hyperprolactinemia is common in these cases. The degree of hyperprolactinemia may help guide decisions about further diagnostic evaluation and treatment. REFERENCES: 1. Eguchi K, Uozumi T, Arita K, et al. Pituitary function in patients with Rathke’s cleft cyst: significance of surgical management. Endocrine Journal. 1994; 41: 535-540. 2. Isono M, Kamida T, Kobayashi H, et al. Clinical features of symptomatic Rathke’s cleft cyst. Clin Neurol Neurosurg. 2001; 96-100. 3. Voelker JL, Campbell RL, Muller J. Clinical, radiographic, and pathological features of symptomatic Rathke’s cleft cyst. J Neurosurg. 1991; 74: 535-544.

A Rathke's Cleft Cyst Presenting with Apoplexy

The occurrence of symptomatic pituitary hemorrhage into a Rathke's cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.

A case of Rathke's cleft cyst presenting with amenorrhea

A 28-year-old female presented with Rathke’s cleft cysts (RCC) manifesting as primary amenorrhea and no development of secondary sexual characteristics. She had no symptoms of headache, visual disturbance, anosmia or galactorrhea.. The endocrinologic study demonstrated partial hypopituitarism. Magnetic resonance imaging revealed about 1.5 cm sized sellar and suprasellar cystic tumor, extending into the pituitary stalk. Its tentative diagnosis was benign pituitary cystic tumor, such as RCC. She underwent surgery by a transsphenoidal approach. Histological examination revealed a ciliated columnar epithelium those consistent with RCC. RCC is rarely symptomatic, but mechanical compression by the cyst or infl ammation itself causes headache, visual fi eld defects, or symptoms of pituitary dysfunction. The present case shows that RCC may manifest as hypogonadotropic hypogonadism.

Rathke cleft cyst presenting with hyponatremia: an unusual presentation

The authors report a case of Rathke cleft cyst presenting with severe hyponatremia. A 33-year-old man suffered sudden severe headaches, visual changes, dizziness, nausea, vomiting, and a metallic taste in his mouth. Initial laboratory values demonstrated severe hyponatremia. Magnetic resonance imaging revealed a cystic lesion with questionable intracystic hemorrhage, concerning for pituitary apoplexy. Transsphenoidal decompression and drainage of the cyst confirmed the diagnosis of Rathke cleft cyst and resolved the symptoms. Postoperative follow-up studies at 6 months demonstrated normal endocrine function and no evidence of a cyst.

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-radi ological imaging revealed a mass in the sella. Via transsphe-noidal 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.

Rathke's cleft cyst presenting with diabetes insipidus

Rathke's cleft cyst presenting with diabetes insipidus

Rathke's cleft cyst presenting as sphenoid sinusitis

Rathke's cleft cysts are developmental abnormalities of the craniopharyngeal duct composed of retained stratified ciliated cuboidal cells of the respiratory type. Incidentally found in the pars intermedia in 13% to 33% of routine autopsies, they are discovered in growing numbers on CT and MRI scans. From 1991 to 1999, 9 cases of Rathke's cleft cysts were encountered by the senior authors, 4 of which were treated surgically (3 by using a transsphenoidal approach and one by using a transcranial subfrontal approach). Of the 3 patients treated with a transsphenoidal approach, 2 presented with symptoms mimicking sphenoid sinusitis and were initially referred for otolaryngologic evaluation. The clinical, pathologic, and radiologic features, as well as management and follow-up of those 2 patients, are reviewed. There has been only one previous case report of Rathke's cleft cysts presenting as sinusitis. Nevertheless, the otolaryngologist should be aware of this condition because it may present with other significant symptomatic extracranial extensions.

A cse of Rathke's cleft cyst presenting with diabetes insipidus

Rathke's cleft cysts (RCCs) are considered to arise from the remnants of Rathke's pouch, an invagination of the stomodeum. They classically described as benign epithelium lined intrasellar cysts containing mucoid material, and also found in 2–33% of routine autopsy series. The most common presenting symptoms are visual impairment, hypothalamic dysfunction, hypopituitarism and headache. Diabetes insipidus has been described in patients with RCC. Very few cases presented with only diabetes insipidus in adults. To our knowledge, our patient is the first case of RCC presenting with only diabetes insipidus in childhood. A 9-year-old girl presented with diabetes insipidus. The physical, neurological and endocrinological examinations were normal, except for diabetes insipidus. Magnetic resonance imaging scan revealed a hyperintense lesion with supra sellar extension in the posterior pituitary both on T1 and T2 weighted images. Subtotal excision of RCC was performed via transsphenoidal surgery. However, diabetes insipidus persisted after the surgery.

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.