Rathke's cleft cysts causing Cushing's disease: Two unique cases and review of the literature

Abstract

Background: The presentation of isolated Rathke's cleft cysts (RCC) without any associated pituitary adenoma in patients with symptoms consistent with Cushing's disease (CD) remains exceedingly rare. As such, we aim to present two cases of RCC presenting with CD with a resultant resolution of their CD following surgical resection.

Case description: Here, we present two cases of RCCs presenting with symptoms suggestive of CD. A functional pituitary microadenoma was the presumed diagnosis based on initial clinical presentation and diagnostic imaging suggesting a pituitary lesion. However, pathology results demonstrated no evidence of adenoma but cysts lined with columnar epithelia consistent with RCC. Complete surgical resection was achieved in both patients through endoscopic endonasal pituitary resection with postoperative symptomatic resolution and normalization of cortisol levels. In addition, we discuss the literature on this rare presentation and suggest a pathological mechanism for this unique presentation of RCC-causing CD.

Conclusion: Surgical resection of RCC may provide a "biochemical cure" for patients presenting with CD, as demonstrated by these two unique cases. The clinical features, histological findings, and possible pathological mechanisms for this unique presentation of RCC causing CD discussed lay the groundwork for future studies into the pathophysiology of RCC and CD.

Keywords: Cushing’s disease; Neurosurgery; Pituitary adenoma; Rathke’s cleft cyst.