Enigmatic echoes: Exploring brainstem neurenteric cysts - A case series and literature review

Int J Surg Case Rep. 2024 Apr:117:109573. doi: 10.1016/j.ijscr.2024.109573. Epub 2024 Mar 24.

Abstract

Introduction and importance: Neurenteric cysts (NCs) are rare congenital anomalies within the central nervous system, originating from the endodermal layer due to inappropriate embryonic notochordal plate and endoderm separation. Despite constituting a minute fraction of CNS tumors, their diverse clinical presentations and brainstem occurrence pose diagnostic challenges.

Case presentation: This study presents a series of three brainstem NCs, detailing demographics, clinical presentations, radiological features, and operative findings. Patients aged 2-40 years exhibited varied symptoms including headaches, vomiting, cranial nerve deficits, and motor/sensory deficits. Literature review of 27 cases showed a mean age of 26.8 years, predominantly in females, with diverse cystic characteristics on MRI. Surgical intervention achieved mostly complete resection, with varying outcomes including recurrence and malignant transformation.

Clinical discussion: This study sheds light on the rare entity of brainstem NCs, emphasizing their diagnostic complexity. NCs, though constituting a minute fraction of CNS tumors, present a diagnostic challenge due to their diverse clinical presentations and rarity in the brainstem.

Conclusion: This study provides insights into brainstem NCs, emphasizing diagnostic complexity and the importance of MRI in accurate diagnosis and surgical intervention. Enhanced collaboration is needed to refine management strategies for these intriguing lesions.

Keywords: Brainstem; Central nervous system; Medulla; Neurenteric cysts; Pons.