Spontaneous Regression of Rathke’s Cleft Cysts: Case Series and Systematic Review

 

Background: Rathke’s cleft cysts (RCCs) are benign fluid-filled cysts that develop in the pituitary gland as a result of abnormal embryological development of Rathke’s pouch. Most RCCs are small and asymptomatic; however, they can present with symptoms caused by mass effect from a large cyst prompting surgical treatment. For smaller asymptomatic lesions, several studies have shown the majority of RCCs do not enlarge, and an unknown proportion demonstrates regression over time. The purpose of this study is to explore the rate of spontaneous RCC regression.

Methods: Three cases of patients with spontaneous RCC regression are described. The authors then performed a systematic review of the literature using PRISMA guidelines to assess the incidence of RCC regression in patients managed conservatively.

Results: Three patients with a diagnosis of RCC demonstrated an average estimated decrease in volume of 74% over a mean interval of 3.7 months ([Fig. 1]). One patient experienced resolution of his chronic headaches, while the 2 other patients had persistent headaches and endocrinopathies at follow-up.

The systematic review included 21 studies reporting on 188 patients with spontaneous regression of RCCs over a range of 2 to 72 months with follow-up interval range of 1 to 60 months. Nine studies reported results for observational cohorts of RCC patients totaling 619 patients observed with 158 (25.5%) patients demonstrating regression ([Table 1]).

Discussion: A minority of RCCs managed conservatively have been shown to demonstrate regression over time. The mechanism for this is unknown but is hypothesized to be due to cyst rupture, cyst fluid resorption, apoplexy, or glucocorticoid therapy. Kinoshita et al. (2022) reported that patients presenting with acute headache had higher rates of regression and symptom improvement, suggesting an acute event such as cyst rupture or apoplexy preceded the regression. Rates of RCC regression in the literature ranged from 10 to 48%, supporting a conservative approach for patients with asymptomatic or minimally symptomatic RCC.

While the majority of RCCs do not demonstrate regression, it is possible this number is underreported. Many patients with RCC are asymptomatic and never diagnosed, and one autopsy study found RCCs in 22% of randomly selected specimens. Increased use of MRI in the general population contributes to a higher incidental diagnosis of RCC, making the natural history of RCCs an area of increased interest.

The optimal follow-up interval for RCCs has not been established, but a study of 229 patients with RCCs reported that most cyst progression occurred within 5 years, and only 1.7% of patients developed new symptoms 5 years after diagnosis. Based on this low risk of progression, surveillance should continue for at least 5 years to demonstrate inactivity of the cyst.

Conclusion: One-quarter of patients with RCCs managed nonsurgically demonstrated regression in a systematic review of the literature. There is a role for conservative management and routine surveillance of RCCs for patients without significant symptoms from mass effect, and surveillance should continue for a minimum of 5 years to demonstrate cyst stability.

Publication History

Article published online:
05 February 2024

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