Object. Rathke cleft cysts (RCCs) are rarely symptomatic. The purpose of this study was to clarify the clinical, neuroimaging, surgical, and pathological features of symptomatic RCCs with special attention to their recurrence.

Methods. This retrospective study involved 53 patients with pathologically confirmed symptomatic RCCs. There were 28 female and 25 male patients, ranging in age from 11 to 68 years (mean 37 years). Common clinical presentations included headache, visual impairment, and endocrine disturbance. The most common endocrine disturbances were hyperprolactinemia and diabetes insipidus. Most of these improved or were resolved after surgery, with the exception of diabetes insipidus and panhypopituitarism. The cysts were intrasellar with suprasellar extension in 33 patients, and ranged in size from 5 to 40 mm (mean 17 mm). In the 50 magnetic resonance (MR) images that were reviewed, the signal intensities were quite variable. Fourteen MR images demonstrated enhancement after an injection of Gd. Intraoperatively, the cyst contents were found to be yellowish (18 patients [37%]) and mucoid (25 patients [51%]). Pathological examinations revealed a pseudostratified columnar epithelium in 26 patients (49%). Abundant squamous metaplasia and a stratified squamous epithelium were also found in 12 patients (23%). Follow-up MR images revealed cyst recurrences that required a repeated operation in six patients. Statistically significant risk factors for a recurrence included enhancement of the lesion on MR images (p = 0.017), the extent of cyst removal (p = 0.012), and the presence of squamous epithelium (p = 0.008).

Conclusions. Rathke cleft cysts are associated with a variety of clinical presentations and sometimes confusing intraoperative and pathological findings. Close postoperative observation with neuroimaging and neuroophthalmological assessment is necessary, especially after a partial removal, as in cases with squamous metaplasia.