Neurenteric Cyst Phenomena in Central Nervous System

Neurenteric cysts are rare congenital lesions resulting from aberrant separation of endoderm and neuroectoderm during early embryogenesis. Typically located within the spinal cord, these cysts consist of an epithelial lining resembling gastrointestinal or respiratory mucosa and are often found in the lower cervical to upper thoracic regions. Intracranial presentations are less common but may localise to the posterior fossa, fourth ventricle or craniovertebral junction. Clinical manifestations depend on size and site, ranging from myelopathic signs, radicular pain and cranial nerve dysfunction to nonspecific symptoms such as headache or nausea when intracranial. Magnetic resonance imaging remains the diagnostic modality of choice, with cysts often exhibiting characteristic signal intensities on T1- and T2-weighted sequences. Definitive diagnosis is established by histopathological examination, revealing columnar or cuboidal epithelium, mucus-secreting cells and occasional cilia. Surgical resection is the mainstay of treatment, with gross total excision correlating with lower recurrence rates. However, adhesion to neural structures or unclear dissection planes can limit complete removal, necessitating alternative strategies such as cyst-subarachnoid shunting or partial resection combined with spinal stabilisation. While benign in most cases, rare instances of malignant transformation and disseminated presentations underscore the need for long-term surveillance. Advances in microsurgical techniques, intraoperative monitoring and a deeper understanding of embryopathogenesis have refined patient management, yet challenges remain in predicting recurrence and optimising outcomes across diverse anatomical locations.

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Neurenteric Cyst Phenomena in Central Nervous System publication trend

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Technical terms

Neurenteric cyst: A congenital cyst lined by endodermal epithelium arising from embryonic mis-patterning between the neuroectoderm and endoderm.

Intradural extramedullary: Location of a lesion within the dura mater but outside the spinal cord parenchyma.

Gross total resection: Surgical removal of all visible cyst tissue.

Leptomeningeal carcinomatosis: Malignant spread of tumour cells to the leptomeninges, the arachnoid and pia mater layers surrounding the CNS.

References

1. Comprehensive Review of Spinal Neurenteric Cysts with a Focus on Histopathological Findings. Cureus (2018).
2. Neurenteric cysts of the spine. Journal of Craniovertebral Junction and Spine (2010).
3. Neurenteric cyst of the craniovertebral junction treated to reduce recurrence using different strategies: Two case reports and a literature review. Medicine (2023).
4. Meningeal carcinomatosis secondary to neurenteric cysts with malignant transformation: a case report. BMC Neurology (2022).
5. Disseminated Intracranial and Spinal Neurenteric Cysts: A Case Report and Literature Review. Case Reports in Neurological Medicine (2024).