Neuroenteric Cyst: A Rare Lesion of the Spinal Cord

Case Report

A 6-year-old girl presented to our hospital with lower extremity weakness associated with paresthesia, with 6 months duration. The neurologic examination revealed lower-limb grade IV motor weakness (slightly more severe on the right side), hyperreflexia, and clonus of the right foot. She had no other neurological deficits, including sphincter dysfunction. A magnetic resonance imaging revealed a dorsal intradural intramedullary cystic mass ([Fig. 1]). She underwent surgical resection, with good clinical outcome. The histopathologic examination revealed a neuroenteric cyst ([Fig. 2]).

Fig. 1 Sagittal T2-weighted (A), T1-weighted (B), T2/ short-tau inversion recovery (C), and T1-weighted post-contrast; (D) axial T2-weighted (E), T1-weighted post-contrast (F), diffusion-weighted imaging and apparent diffusion coefficient map (G and H), showing a well-delimited intramedullary expansile purely cystic mass (T6 level), with no enhancement, no restricted diffusion and minimal vasogenic edema, and no evidence of spinal malformations.

Fig. 2 Sagittal T2-weighted (A) and sagittal T1-weighted post-contrast (B) showing no residual lesion after surgical resection. (C) The lining of the cyst is composed by cuboidal cells with areas of squamous metaplasia, resting on a layer of fibrous connective tissue. (D) Epithelium cells are carcinoembryonic antigen positive.

Neuroenteric cysts are rare congenital lesions accounting for 0.7 to 1.3% of spinal tumors; they result from abnormal partitioning of the embryonic notochordal plate and the endoderm, during the third week of embryogenesis.[1] [2] These lesions are typically intradural extramedullary lesions, with intra-axial lesions being extremely rare.[3] Neurological symptoms usually present around the second or third decades of life, with generally size-dependent myelopathy.[4] Surgical treatment is typically required, with total surgical resection being the primary goal; subtotal excision is often associated with tumor recurrence.[1] [4]

Author Contributions

Marta Rodrigues: elaboration and critical revision of the case report.

Joana Osório: elaboration and critical revision of the case report.

Carlos Pontinha: elaboration and critical revision of the case report.

Miguel Correia: elaboration and critical revision of the case report.

Carla Conceição: elaboration and critical revision of the case report for intellectual content.

Disclosure

The authors report no disclosures relevant to the manuscript.

• References

• 1 Savage JJ, Casey JN, McNeill IT, Sherman JH. Neuroenteric cysts of the spine. J Craniovert Jun Spine 2010; 1 (01) 58-63
• 2 Rauzzino MJ, Tubbs RS, Alexander III E, Grabb PA, Oakes WJ. Spinal neurenteric cysts and their relation to more common aspects of occult spinal dysraphism. Neurosurg Focus 2001; 10 (01) e2
• 3 Baek WK, Lachkar S, Iwanaga J. , et al. Comprehensive review of spinal neurenteric cysts with a focus on histopathological findings. Cureus 2018; 10 (09) e3379
• 4 Kumar R, Nayak SR. Unusual neuroenteric cysts: diagnosis and management. Pediatr Neurosurg 2002; 37 (06) 321-330