Rathke’s cleft cyst with xanthogranulomatous change: A case report and review of the literature

Abstract

Background: Rathke’s cleft cysts (RCCs) are benign, typically asymptomatic sellar lesions found incidentally in adults, with a dramatically lower incidence in pediatric patients (<18 years). We present a case of RCC with xanthogranulomatous change (XGC) – an even less common subtype of RCC – treated by endoscopic endonasal surgical resection. This is the second reported instance of an RCC with XGC occurring in a pediatric patient. Case Description: The patient is a 17-year-old male with delayed puberty who presented with bitemporal hemianopsia and was found to have a 2.6 cm lesion, initially thought to be a craniopharyngioma. He subsequently underwent uncomplicated transsphenoidal endoscopic endonasal resection. Histology confirmed the diagnosis of RCC and demonstrated marked degenerative XGCs with squamous metaplasia. The patient tolerated the procedure well with improvement in visual symptoms. Conclusion: RCC with XGC is a very rare pathology, particularly in the pediatric population. These lesions, while benign, can manifest clinically with significant symptoms. While treatment paradigms are not fully established with a small cohort of cases, endoscopic endonasal approaches have made surgical resection of these lesions a safe and effective treatment strategy, even in the pediatric population.