Neuropediatrics 2020; 51(03): 233-234
DOI: 10.1055/s-0039-3399530
Images in Neuropediatrics
Georg Thieme Verlag KG Stuttgart · New York

Neuroenteric Cyst: A Rare Lesion of the Spinal Cord

1   Department of Neuroradiology, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
,
Joana Osório
2   Department of Neuroradiology, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
,
Carlos Pontinha
3   Department of Anatomical Pathology, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
,
Miguel Correia
4   Department of Neurosurgery, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
,
Carla Conceição
2   Department of Neuroradiology, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
› Author Affiliations
Study Funding No funding reported.
Further Information

Publication History

17 September 2019

29 September 2019

Publication Date:
21 November 2019 (online)

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]).

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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.
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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.


 
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