Rathke cleft cyst apoplexy: Hormonal and clinical presentation

Abstract

Background: Rathke cleft cyst (RCC) apoplexy is an uncommon type of lesion that is challenging to diagnose without histopathological samples. Very few articles have been published describing the details of RCC apoplexy. We studied a good number of published articles to analyze its demographics, clinical and hormonal presentations, and outcomes. Methods: A literature review of English language publications about RCC apoplexy or pituitary apoplexy was conducted using Medline and EMBASE search engines. Thirty articles with histological diagnosis of RCC apoplexy were identified, the earliest of which was published in 1990 and the latest in 2019. We combined the findings of these articles with our own case report and then compared the demographics, clinical and hormonal presentations, and outcomes between RCC apoplexy and pituitary adenoma apoplexy. Results: Our data included 29 patients with RCC, with a mean age of 36.87 years (8–72) and a predominance of female patients (68%). The hemorrhagic type was most common, reported in 86%. Headache was the most common presenting symptom, being reported in 93% followed by hypogonadism (73%) and hormonal deficits (52%). All but three patients improved neurologically (90%); however, 45% of patients required long-term hormonal replacement, mostly thyroid hormone. No cases of worsening neurological or hormonal status were reported. Conclusion: RCC apoplexy presents with less severe neurological and hormonal abnormalities than pituitary adenoma apoplexy; it also has a better prognosis in endocrine functional recovery. We recommend applying current management guidelines of pituitary adenoma apoplexy to RCC apoplexy.