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Neurenteric Cyst

Summary

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  • Rare congenital malformation of the central nervous system
  • Endodermal-derived cysts lined with mucin-secreting epithelium
  • Typically located in the posterior fossa or spinal canal, often ventral to the spinal cord1

Pathophysiology

  • Believed to result from incomplete separation of endoderm and ectoderm during embryogenesis
  • Persistence of the neurenteric canal, leading to communication between the primitive endoderm and ectoderm
  • Cysts are lined with columnar or cuboidal epithelium, often with mucin-producing goblet cells
  • May contain components of respiratory or gastrointestinal epithelium

Demographics

  • Rare lesions, accounting for 0.7-1.3% of all spinal cord tumours
  • More common in males (M:F ratio 2:1)
  • Usually diagnosed in the first two decades of life
  • Spinal neurenteric cysts are more common than intracranial ones

Diagnosis

  • Clinical presentation varies depending on location and size:
    • Spinal: myelopathy, radiculopathy, or local pain
    • Intracranial: headache, cranial nerve deficits, or symptoms of mass effect
  • Histopathological examination is required for definitive diagnosis
  • Differential diagnosis includes:
    • Arachnoid cyst
    • Epidermoid cyst
    • Dermoid cyst
    • Ependymal cyst

Imaging

  • MRI is the imaging modality of choice:
    • T1-weighted: variable signal intensity, often isointense to CSF
    • T2-weighted: hyperintense signal, may show internal septations
    • FLAIR: usually suppressed, similar to CSF
    • Contrast enhancement: typically absent or minimal
  • CT findings:
    • Hypodense, well-circumscribed lesion
    • May show calcifications in rare cases
  • Spinal X-rays may show:
    • Widening of the spinal canal
    • Scalloping of vertebral bodies
    • Associated vertebral anomalies (e.g., hemivertebrae, butterfly vertebrae)

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  • An incidental lesion in a patient who had an MRI after a suspected TIA.
  • A lobulated lesion causing mild mass effect on the brainstem was T1-hyperintense and isointense on fat-suppressed FLAIR (the latter making a lipoma less likely).

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  • A 50-year-old patient with MS had an incidental lesion in the premedullary space that had not changed in over a decade.
  • Consistent with a neurenteric cyst, the lesion was T1-hyperintense, T2-hypointense and did not enhance.

Treatment

  • Surgical resection for symptomatic lesions. A non-enhancing, often T1-hyperintense cyst sitting ventral to the brainstem or cord is the classic appearance

Differential diagnosis

Differential Diagnosis Differentiating Feature
Arachnoid Cyst Lacks enhancement and does not contain mucoid or proteinaceous material
Epidermoid Cyst Demonstrates diffusion restriction on MRI
Dermoid Cyst Contains fat and calcifications
Pilocytic Astrocytoma Enhances with contrast and has solid components
Ependymoma Typically has calcifications and heterogeneous enhancement
Choroid Plexus Cyst Located within the ventricles and has CSF-like signal
Colloid Cyst Typically located in the third ventricle and has high protein content
Rathke's Cleft Cyst Located in the sella turcica or suprasellar region
Enterogenous Cyst Similar features, but typically found in the posterior mediastinum
Teratoma Contains multiple tissue types including fat, calcifications, and soft tissue

  1. Visvanathan et al. Neurenteric cyst presenting with stridor and dysphagia: case report and literature review. 2015. Scottish medical journal - Open in new tab. ↩