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.

Neurenteric Cyst Presenting with Bleeding Per Rectum

“…Hemorrhagic complications like hemoptysis or hematemesis or malena can occur due to heterotopic gastric or pancreatic tissue within the cyst wall causing erosions and peptic ulceration. [1] In our case also, pancreatic tissue was seen in the wall of communicating tract between jejunum and mediastinal cyst resulting in malena episodes. Additionally, spontaneous hemorrhage may occur within the cyst itself.…”

“…[1,2] Exact characterization of these cystic lesions on imaging may be sometimes difficult and a final diagnosis is made after histopathological examination.…”

Spinal Neurenteric Cyst in Association with Klippel-Feil Syndrome: Case Report and Literature Review

“…In some cases, the progression of the symptoms may be episodic with recovery in between the episodes and can be mistaken for multiple sclerosis (1,36). In other cases, symptoms can present as chronic pyrexia, incontinence, paraplegia, or meningitis (6,18) due to either a fistulous gastrointestinal connection or chemical irritation from leakage of acid and pepsin containing secretions (12, 15,30).…”

“…The location of the lesion is the main determinant of the clinical presentation. Progressive focal pain at the level of spinal axis pathology, myelopathic signs, and radicular symptoms are common (30). In some cases, the progression of the symptoms may be episodic with recovery in between the episodes and can be mistaken for multiple sclerosis (1,36).…”

“…There is no immunohistochemical reactivity for GFAP that delineates the histomorphological equivocal lesions from ependymal cysts. Radiological evaluation of NE cysts is essential to early diagnosis, and detailed evaluation of vertebral anatomy is critical in creating a treatment plan for NE cysts (30). MRI is the best modality for the evaluation of the cyst and its relation to surrounding neural structures.…”