Neurenteric cysts of the spine

Abstract

Neurenteric cysts account for 0.7-1.3% of spinal axis tumors. These rare lesions result from the inappropriate partitioning of the embryonic notochordal plate and presumptive endoderm during the third week of human development. Heterotopic rests of epithelium reminiscent of gastrointestinal and respiratory tissue lead to eventual formation of compressive cystic lesions of the pediatric and adult spine. Histopathological analysis of neurenteric tissue reveals a highly characteristic structure of columnar or cuboidal epithelium with or without cilia and mucus globules. Patients with symptomatic neurenteric cysts typically present in the second and third decades of life with size-dependent myelopathic and/or radicular signs. Magnetic resonance imaging and computed tomography are essential diagnostic tools for the delineation of cyst form and overlying osseous architecture. A variety of approaches have been employed in the treatment of neurenteric cysts each with a goal of total surgical resection. Although long-term outcome analyses are limited, data available indicate that surgical intervention in the case of neurenteric cysts results in a high frequency of resolution of neurological deficit with minimal morbidity. However, recurrence rates as high as 37% have been reported with incomplete resection secondary to factors such as cyst adhesion to surrounding structure and unclear dissection planes. Here we present a systematic review of English language literature from January 1966 to December 2009 utilizing MEDLINE with the following search terminology: neurenteric cyst, enterogenous cyst, spinal cord tumor, spinal dysraphism, intraspinal cyst, intramedullary cyst, and intradural cyst. In addition, the references of publications returned from the MEDLINE search criteria were surveyed in order to examine other pertinent reports.

Keywords: Craniovertebral junction; enterogenous cyst; intraspinal cyst; neurenteric cyst; spinal cord tumor; spinal dysraphism.

Figure 1

Photomicrograph of neurenteric cyst epithelium. (a) Neurenteric cyst wall (Type A) demonstrating simple columnar and cuboidal epithelium resting upon basal lamina composed of Type IV collagen (H and E, ×200). (b) High-magnification photomicrograph of columnar and cuboidal mucin-secreting goblet cells (H and E, ×1000). (c) High-magnification photomicrograph of ciliated pseudostratified columnar epithelium of neurenteric cyst wall (H and E, ×1000).

Figure 2

MRI of thoracolumbar junction with intradural/ extramedullary neurenteric cyst at T11. (a) Midsagittal MRI demonstrates isointense lesion (arrow) on T1-weighted and (b) hyperintense signal (arrow) on T2-weighted

Figure 3

MRI of an intradural/extramedullary neurenteric cyst located at the craniocervical junction. Coronal (a) and sagittal (b) T1-weighted MRI showing the ventrally located homogenous lesion compressing the cervical spinal cord. Axial imaging demonstrates cord flattening by the anteriorly located neurenteric cyst hyperintense on both T1-weighted (c) and T2-weighted (d) MRI