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Objective: Rathke's cleft cysts are benign, embryological remnants in the pituitary gland. The majority of them are small and asymptomatic but a few may become large, and cause mass effects, pituitary hormone deficiencies and visual impairment. Recommendations for the follow‐up of Rathke's cleft cysts vary since data on the natural history are sparse. Patients and Design: Data at diagnosis and at 1, 5 and 10 years for patients with a Rathke's cleft cyst (434 at diagnosis, 317 females) were retrieved from the Swedish Pituitary Registry. Cysts ≤3 mm in diameter were excluded from the study. Measurements: Data included demographics, cyst size, pituitary function, visual defects and surgery. Results: The mean age at diagnosis was 45 years. In patients with cysts <10 mm in diameter (n = 204) 2.9% had pituitary hormone deficiencies and 2% had visual field impairments. Cyst size did not progress during the 5 years. Cysts with a diameter of ≥10 mm that were not operated (n = 174) decreased in size over the years (p <.01). Pituitary hormone deficiencies and visual impairments were more frequent (18% and 5.7%, respectively) but were stable over time. Transphenoidal surgery was performed in 56 patients of whom 51 underwent surgery before the 1‐year follow‐up. The mean cyst diameter at diagnosis was 18 mm (range: 9─30 mm), 36% had pituitary hormone deficiency, 45% had visual field defects and 20% had impaired visual acuity. One year after surgery 60% had no cyst remnants, 50% had a pituitary deficiency, 26% had visual field defects and 12% had impaired visual acuity. No major changes were observed after 5 years. Twelve of the operated patients had a follow‐up at 10 years, in eight the cyst remnants or recurrences increased in size over time (p <.05). Conclusions: Rathke's cleft cysts with a size less than 10 mm rarely grow and our results indicate that radiological follow‐up can be restricted to 5 years. In contrast, progression of postoperative remnants or recurrent cysts is more likely and require long‐term follow‐up. [ABSTRACT FROM AUTHOR]

Immune checkpoint inhibitor–induced hypophysitis is a rare complication of pembrolizumab, but the effects of drug‐induced hypophysitis on preexisting pituitary lesions are unknown. The authors treated an unusual case of pembrolizumab‐induced hypophysitis that resulted in acute exacerbation of a preexisting pituitary cystic lesion causing severe intractable headaches. Before initiation of pembrolizumab therapy, magnetic resonance imaging should be carefully reviewed for sellar pathology. Patients demonstrating a Rathke's cleft cyst should consider other forms of treatment first or be carefully counseled before pembrolizumab initiation, because the advent of hypophysitis could result in symptomatic progression of an existing lesion, necessitating urgent surgical intervention. [ABSTRACT FROM AUTHOR]

IgG4‐related hypophysitis (IgG4‐RH) is increasingly being reported as an isolated entity or, less frequently, as a manifestation of a multiorgan IgG4‐related disease (IgG4‐RD), in which typical histopathology is a cornerstone for the diagnosis. We aimed to describe the histopathological changes in the surgical specimens from patients with clinical signs of pituitary disease that fulfilled the current diagnostic criteria for IgG4‐RH. Histopathological features were correlated with clinical and radiological findings. Of 19 patients with pituitary dysfunction and inflammatory changes in the surgical pituitary specimen operated on during 2011‐2019, we identified five patients with typical IgG4‐related pathology (lymphoplasmacytic infiltration with more than 10 IgG4‐positive plasma cells per one high power microscopic field, representing at least 40% of all plasma cells and at least focal storiform fibrosis). One patient with diabetes insipidus and pachymeningitis with IgG4‐related changes in a biopsy from the dura was also included. Additional histopathological changes that typically are not part of the IgG4‐RH were observed: Rathke's cleft cyst in four and granulomatous changes in two patients. One patient had an elevated serum IgG4 level and systemic manifestations that could be associated with the systemic IgG4‐RD. Our findings indicate that pure IgG4‐RH is uncommon. All patients with pituitary dysfunction, beyond typical IgG4‐related pathology, had other pathological findings that could trigger the secondary IgG4‐response. Both primary pathology and secondary IgG4‐related features should be reported in patients with pituitary dysfunction because their co‐occurrence may cause atypical clinical and imaging features, and unexpected response to surgical and pharmacological treatment. The current criteria for the diagnosis of IgG4‐RH can lead to overdiagnosis of IgG4‐RH if additional pathological changes are not taken into consideration. The classification criteria of IgG4‐RD proposed by the American College of Rheumatology/European League Against Rheumatism could help classify patients more properly as IgG4‐RH if applied to the pituitary gland. [ABSTRACT FROM AUTHOR]

Summary: Objective: Rathke's cleft cyst (RCC) is a common sellar lesion which may cause visual impairment, hypopituitarism and headaches from mass effect. The natural history of these lesions is currently unclear. We investigated the natural history of RCCs and compared surgically treated patients with those treated conservatively. Methods: We performed a retrospective cohort study of patients diagnosed with a RCC between 1996 and 2016 at Stanford University and Ospedale Maggiore Policlinico di Milano. Results: Patients were divided into 2 cohorts: Group A, 72 subjects who underwent surgical resection of a symptomatic RCC, and Group B, 62 subjects managed conservatively. Compared to Group B, Group A subjects had larger RCCs (79% vs 22% had a largest diameter >10 mm, P < .001) and were more likely (41.5% vs 16%, P < .001) to present with hypopituitarism and diabetes insipidus (DI) (18% vs 1.6%, P = .002). In Group A, after a mean follow‐up of 53.7 months, 12.5% of patients had recurrence and a second surgery. After surgery, 35% of patients recovered pituitary function. Hyperprolactinemia (26.6%) and hypogonadism (66.6%) resolved more commonly that did DI (20.1%). New pituitary deficits appeared in 16.6% of patients after surgery. In Group B, with a mean follow‐up of 41 months, only 6.4% had cyst enlargement, none underwent surgery, and none developed a pituitary deficit. Conclusion: Our data offer guidance in decision‐making regarding the management of RCC patients and confirm the safety of conservative treatment in asymptomatic patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective.—Headaches are common presentations in patients with Rathke's cleft cyst (RCC). This study was conducted to elucidate the characteristics and underlying mechanisms of the headache. Methods.—We retrospectively studied 46 patients with RCC, 33 of whom underwent surgical intervention. Results.—Headache, particularly frontal headache, was the most common symptom, occurring in 27 patients (58.7%). Eleven patients with sudden episodic headache mimicking that of pituitary apoplexy. Although the presence of headache did not correlate with cyst size, it was significantly more common in RCCs with: high- and iso intensity content on T1-weighted MR image ( P= .0363), mucous content within the cyst ( P= .0023), and intense chronic inflammation at the cyst wall ( P= .0276). Among 6 patients with histologically recognized intense inflammation, every patient had frontal headache ( P= .0407), 5 patients had episodic headache ( P= .0002), and 4 patients had associated hypopituitarism ( P= .0073), none of which improved after surgical intervention. Headaches improved after surgery in 17 of 21 patients (81.0%). Conclusions.—Headache, particularly frontal episodic headache, is a common and characteristic manifestation in patients with RCC and may indicate intermittent inflammatory reactions caused by mucous content. Patients with episodic headache should undergo surgical treatment to prevent exacerbation of the inflammation that can result in irreversible endocrine dysfunction. [ABSTRACT FROM AUTHOR]

A 39-year-old woman presented with visual loss and hyperprolactinaemia. Magnetic resonance imaging showed an intrasellar and suprasellar mass with a large cystic component that was hyperintense on T2-weighted imaging. Prolactin levels were up to 5400 mIU/L. Following trans-sphenoidal surgery, histology showed elements of both a Rathke's cleft cyst and prolactinoma. We suggest primary surgical management of large cystic pituitary lesions with associated hyperprolactinaemia, particularly when there is hyperintensity on T2-weighted imaging. [ABSTRACT FROM AUTHOR]

Chordoid glioma has been recently described as a slow-growing neoplasm with chordoid appearance, occurring exclusively in the regions of the third ventricle and hypothalamus of middle-aged women. We experienced a case of a 48-year-old woman with a suprasellar tumor composed of chordoid glioma and Rathke's cleft cyst, which was confirmed by histopathological, immunohistochemical and electron microscopic examinations. Histologically, chordoid glioma comprised the major part of the tumor, and the prominent Rathke's cleft cysts were distributed focally in the same tumor tissue without any transitions. Chordoid glioma was immunoreactive for glial fibrillary acidic protein, S-100 protein and vimentin, and focally positive for epithelial membrane antigen and CD34, while cytokeratin highlighted epithelial cells lining Rathke's cleft cysts. Ultrastructural examination of the chordoid glioma revealed short cytoplasmic processes, intermediate filaments, intercellular junctions of zonular adherens type, basal lamina, secretory granules and pinocytic vesicles. The ultrastructural observations of the current case are similar to those of the subcommisural organ, although cell body zonation or microvilli were not evident. The coexistence of chordoid glioma and Rathke's cleft cyst has not been reported previously and may represent a collision tumor. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented discussing the clinical case of a 53-year old woman diagnosed with isolated bitemporal visual field defects.