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Ventral foramen magnum neurenteric cysts: a case series and review of literature

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Abstract

Neurenteric cysts (NEC) are uncommon, benign, congenital lesions. Ventral foramen magnum (FM) location is very rare. The difficulties in diagnosis and management aspects are detailed with a review of the pertinent literature. We report four new cases of ventral FM NEC, all managed surgically and present a literature review of ventral FM NEC. A retrospective analysis of histopathologically confirmed cases of ventral FM NEC, operated from 2010–2013 at our institute, was performed. For review, only those cases of NEC extending from the lower clivus to the C2 level constituting the foramen magnum were included. Including our four cases, a total of 47 cases were identified. The male to female ratio was 1.2:1. Mean age was 33.5 years (range 1–60 years). Neck pain and occipital headache were the most common symptoms, followed by limb weakness and cranial nerve paresis. Recurrent meningitis was noted in three cases. Hyperintensity on both T1- and T2-weighted sequences with absent enhancement was the most common finding on MRI. Surgical approaches were as follows: suboccipital (n = 21), far/extreme lateral (n = 18), retrosigmoid (n = 6), and transoral (n = 4). The extent of resection was as follows: total, 26; near total, 6; subtotal, 9; and partial, 3 cases. Cerebrospinal fluid diversion was done in four cases for intracranial hypertension. Mean follow-up duration was 26.8 months (range 1 month–9 years). Recurrence was noted in four (8.5 %) cases. One (2 %) case had malignant transformation. Mortality rate was 4 %. Foramen magnum neurenteric cysts are rare, benign tumors of the central nervous system. Accurate preoperative diagnosis can often be established with MRI. Surgical removal is the treatment of choice. Complete excision is ideal but often not possible. Near total removal would suffice with good progression-free periods. A long-term follow-up with radiological studies is necessary as delayed recurrences can occur.

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Acknowledgments

Authors would like to acknowledge Dr. Kiran Chikkanahalli Subbarao for the assistance in providing the histopathological images.

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Authors and Affiliations

  1. Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India

    G. Lakshmi Prasad, Bhawani Shankar Sharma & Ashok Kumar Mahapatra

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  1. G. Lakshmi Prasad
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  2. Bhawani Shankar Sharma
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  3. Ashok Kumar Mahapatra
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Correspondence to Ashok Kumar Mahapatra.

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Adam Tucker, Osaka, Japan

In this retrospective case series and review of literature, the authors describe a total of 47 cases, including four of their own, of ventral foramen magnum neurenteric cysts, and build upon the growing database for this unique heterotopic entity. The four cases were mostly relatively young men presenting with either neck pain or suboccipital headache and vomiting, and variable degrees of progressive lower cranial nerve dysfunction and myelopathy. In half of these patients, MR neuroimaging demonstrated cystic masses with homogeneous hyperintensity on T1 and hypointensity on T2 and were preoperatively diagnosed radiologically with neuroenteric cyst, followed by histopathological confirmation in all cases. A far lateral approach was used in two cases for total or near total removal, while the others resulted in near total or subtotal resection. Patients with total or near total resection seemed to have better postoperative outcome; however, these patients tended to have a milder preoperative neurological status.

Although the pathogenic origins of intracranial neurenteric cysts are somewhat unclear, they are considered to be histologically benign tumors which contain heterotopic epithelium resembling intestinal or respiratory tracts with positive immunoreactivity for EMA, cytokeratin, and CEA. These developmental malformations are typically located ventrally in the midline of the posterior fossa, fourth ventricle, and occasionally in the cerebral hemispheres. Because of the rarity of intracranial lesions, a consideration of related standard surgical anatomical descriptions of ventrally located tumors of the foramen magnum (usually meningiomas) may be useful for adequate management. Typical foramen magnum lesions originate from the basal groove at the lower third of the clivus, anterior to the medulla, with inferior projection. In addition, of particular importance are the relations to the vertebral artery and dentate ligament [1, 2].

This thorough review provides a detailed description of the entity and literature review, and because most reports have been no greater than three cases, this is the largest case series report of neurenteric cysts from a single institution. Although endodermal cysts and fistula are well known to occur in the posterior mediastinum and spine, due to advances in neuroimaging, the frequency of both symptomatic and incidental intracranial encounters is increasing. Therefore, the authors should be congratulated for analyzing their clinical data and the extant literature in a way that will update and improve our ability to diagnose and treat neurenteric cysts of the foramen magnum.

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Prasad, G.L., Sharma, B.S. & Mahapatra, A.K. Ventral foramen magnum neurenteric cysts: a case series and review of literature. Neurosurg Rev 39, 535–544 (2016). https://doi.org/10.1007/s10143-015-0687-2

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