Endocrine Abstracts

Background: Rathkes cleft cysts (RCC) are present in up to 20% of autopsy studies but only a minority necessitate surgical treatment¹. Inflammation of the RCC wall may act a trigger for disease progression, resulting in development of clinical symptoms and prompting investigation and surgical management^2,3. The significance of inflammation of RCC is thought to be three-fold: the development of classical symptoms including headache, visual disturbance or pituitary hormone disturbance, secondly, a predisposition to rupture or apoplexy, and thirdly, it is thought to increase rate of RCC recurrence¹.

Objective: To review and characterize clinical presentation, histological and radiological findings in a series of patients with inflamed RCC.

Methods: We conducted a retrospective case series of 25 cases of RCC, which had undergone surgical management between April 2016 and November 2022. Histopathology and radiology were independently reviewed by neuropathologist and neuroradiologist, and case notes were reviewed for clinical and biochemical data.

Results: 25 cases were reviewed: 19 demonstrated inflammation of either the RCC cyst epithelium (n=10), cyst wall (n=17) (Rathkes cystitis) or anterior pituitary (adenohypophysitis) (n=10), 6 cases were not inflamed; mean age was 47 years (±19 years); 72% were female, consistent with literature. Preoperative features included pituitary dysfunction (64%), headache (64%), visual disturbance (20%) and polyuria/polydipsia (4%). Most (67%) patients experienced symptoms for less than 1 year. Four patients presented with apoplexy. Headache was present in 90% of patients with adenohypophysitis vs 47% cases without pituitary involvement (P=0.027) and all 9 cases where adenohypophysitis was chronic. Pituitary dysfunction was not associated with cystitis (69% vs 67% p=ns) or adenohypophysitis (44% vs 33% P=ns). Rathkes cystitis was associated with loss of posterior bright spot (71% vs 14% P=0.013).

Conclusion: Headache but not pituitary dysfunction was associated with adenohypophyseal inflammation. A trend of increasing headache prevalence was seen with increasing degree of inflammatory infiltrate within RCC.

References: 1. Montaser AS, Catalino MP, Laws ER. Professor Rathke’s gift to neurosurgery: the cyst, its diagnosis, surgical management, and outcomes. Pituitary. 2021;24(5):787-796.2. Hama S, Arita K, Nishisaka T, et al. Changes in the epithelium of Rathke cleft cyst associated with inflammation. J Neurosurg. 2002 Feb;96(2):209-16.3. Komatsu F, Tsugu H, Komatsu M, et al. Clinicopathological characteristics in patients presenting with acute onset of symptoms caused by Rathke’s cleft cysts. Acta Neurochirurgica. 2010;152(10):1673-1678.