The oldest presenting neurenteric cyst of the spinal cord.

We describe an 81-year-old gentleman presenting with mild myelopathic symptoms in the upper limbs. Imaging showed a C1-3 intradural extramedullary lesion initially thought to be an arachnoid cyst. A C1 + 2 hemilaminectomy and partial excision was performed with histology revealing a neurenteric cyst (NC). NCs are congenital tumours that usually present within the third decade of life, they account for 1% of all spinal tumours. A literature search was conducted and we found that the age of presentation might actually be earlier than previously described. We also found that there has never before been a case described in the eighth decade of life, making this the oldest known symptomatic presentation of this rare condition.

Calcifying pseudoneoplasm of the neuraxis (CAPNON) associated with neurenteric cyst. An autopsy case showing unusual fatal outcome.

A 53-year-old man with a history of an untreated brain mass was taken to Toyama Prefectural Central Hospital by emergency transport. Computed tomography revealed an intracranial hypo-attenuated lesion exhibiting mass effect. Several calcified foci were observed around the lesion. His radiographical diagnosis was meningioma with calcification and edema. He suddenly showed tonic seizure after admission; therefore an emergency craniotomy was performed. However, he unfortunately died due to advanced cerebral edema. Microscopic findings of the surgically obtained materials were consistent with neurenteric cyst (NC). Intracranial hard masses were found adjacent to NCs, and the masses were composed of fibrous cartilage-like matrix with extensive linear calcification and the presence of surrounding round-to-oval epithelioid cells. Thus, calcifying pseudoneoplasm of the neuraxis (CAPNON) associated with NC was considered the most appropriate diagnosis of the present case. To the best of our knowledge, this is the first report of such a case. The present case suggests that delay of treatment might cause a poor outcome, at least in CAPNON associated with NC. Careful investigations, including the underlying pathology, may be essential when considering the etiology of CAPNON and its treatment strategies.

Elucidating the multiple genetic alterations involved in the malignant transformation of a KRAS mutant neurenteric cyst. A case report.

Neurenteric cyst (NC) shows benign histopathology and rarely demonstrate malignant transformation. We herein describe a case of NC that exhibited malignant transformation. A 65-year-old female presented with gait disturbance due to compression by a cystic mass on the dorsal surface of the medulla oblongata. Partial resection was performed twice, leading to improvement of her symptoms. Two years after the second surgery, gadolinium-perfused T1-weighted magnetic resonance imaging revealed an invasive lesion with contrast enhancement at the trigone of the left lateral ventricle for which partial resection followed by radiotherapy was performed. However, mass regrowth was observed, with the patient eventually succumbing to her disease 11 months after her third surgery. Histopathological analyses of the first and second surgical specimens identified pseudostratified cuboidal epithelial cells, with no nuclear or cellular atypia resembling gastrointestinal mucosa, lining the inner surface of the cystic wall. Based on these findings the lesion was diagnosed as NC. The third surgical specimen exhibited apparent malignant features of the epithelial cells with elongated and hyperchromatic nuclei, several mitotic figures, small necrotic foci, and a patternless or sheet-like arrangement. Based on these findings, the lesion was diagnosed as NC with malignant transformation. Next-generation sequencing revealed KRAS p.G12D mutation in all specimens. Additionally, the third surgical specimen harbored the following 12 de novo gene alterations: ARID1A loss, BAP1 p.F170L, CDKN1B loss, CDKN2A loss, CDKN2B loss, FLCN loss, PTCH1 loss, PTEN loss, PTPRD loss, SUFU loss, TP53 loss, and TSC1 loss. The aforementioned results suggest that KRAS mutation is associated with the development of the NC, and that the additional gene alterations contribute to malignant transformation of the NC.

Cervicothoracic Neurenteric Cyst With Contralateral Diaphragmatic Hernia: An Unusual Combination.

We describe a premature male infant who died from complications resulting from two malformations: a large left-sided diaphragmatic hernia and a right-sided cervicothoracic neurenteric cyst. The findings of the first limited prenatal ultrasound led to the incorrect diagnosis of right-sided diaphragmatic hernia. Vertebral anomalies, commonly associated with neurenteric cysts, and an intrathoracic stomach, were not identified until autopsy examination. A literature review describes only one partly similar case relating a neurenteric cyst to the jejunum associated with an ipsilateral diaphragmatic defect identified on prenatal ultrasound. The second report of this combination raises the question of a developmental relationship.

Pediatric intracranial neurenteric cyst of the oculomotor nerve: a case-based review.

BACKGROUND: Neurenteric cysts (NECs) of the central nervous system (CNS) are uncommon congenital entities arising from embryonal elements. Intracranial NECs in the pediatric population are rare. METHODS: The authors describe the presentation, radiographic imaging, and pathologic findings of an 11-year-old boy with a right oculomotor nerve NEC. A literature review was performed to identify additional cases of pediatric intracranial NECs published in the English language, over the past 30 years (1990-2020). The authors discuss the presentation, investigations, management, and prognosis of this interesting entity. RESULTS: We describe an 11-year-old boy who presented to neurosurgical attention with disconjugate gaze, anisocoria, and ptosis. Magnetic resonance imaging (MRI) demonstrated a lobulated, cystic, and peripherally enhancing mass involving the right oculomotor nerve. The patient underwent pterional craniotomy for drainage of the cyst and subtotal resection of the cyst wall. The tan-colored mass was displacing the basilar artery, compressing the cerebral peduncle, and adherent to the inferior surface of the tentorium. The lesion was within the oculomotor nerve and splitting the fibers, and the cystic contents were thick and mucinous. Histopathological examination of the specimen demonstrated a thin fibrous cyst wall with scattered inflammatory cells and lined by simple columnar epithelium containing mucin. The lining cells were immunoreactive with epithelial membrane antigen (EMA) and pan-keratin AE1/AE3. The diagnosis of a NEC was rendered. A comprehensive literature review of pediatric intracranial NECs yielded 46 additional lesions published in the literature, involving the skull base, posterior fossa, cerebral convexity, and cranial nerves. NECs present with local mass effect and less commonly, with aseptic meningitis or intracystic hemorrhage. Maximal safe GTR remains the mainstay management, although cyst drainage and marsupialization, cyst shunting, and fenestration of cystic contents into the ventricle or basal cisterns have been reported with variable success. CONCLUSION: CNS NECs are rare congenital entities; although they occur less frequently in the intracranial components compared to the spine, their diagnosis and management should be considered for intracranial cystic lesions. Maximal safe GTR is the mainstay treatment and frequently yields favorable outcomes.

Concurrent Thoracic Spinal Intradural Extramedullary Epidermoid and Neurenteric Cyst in a Spinal Dysraphism Child.

INTRODUCTION: Intraspinal epidermoid cysts are congenital or acquired in origin; whereas intraspinal neurenteric cysts (NECs) are of congenital origin. Their individual association with spinal dysraphism and vertebral segmentation anomalies is very well known. CASE PRESENTATION: We hereby report a case of concurrent intradural extramedullary epidermoid and NEC at adjacent vertebral levels in a spinal dysraphism child, not reported in English Literature till now. CONCLUSION: Multiple spinal lesions related to any/all of the 3 germ layers can coexist at same or adjacent vertebral levels in the same patient and surgical planning shown to be done accordingly.

Early Recurrence of an Infantile Endodermal Oculomotor Nerve Cyst following Surgical Fenestration: A Case Report.

INTRODUCTION: Infantile endodermal oculomotor nerve cyst (EONC) is an extremely rare entity. There are very few pediatric cases reported in the literature, and as expected, oculomotor palsy is the most common presenting symptom. To date however, the risk of recurrence of these lesions following surgical intervention is unclear due to a lack of long-term radiological follow-up. CASE PRESENTATION: We present a case of a 13-month-old male patient with an EONC and detail his surgical fenestration and postoperative course. Somewhat surprisingly, re-expansion occurred within 6 months and remained stable 2 years later. DISCUSSION: A surgical approach to fenestration of an EONC in an infant is possible and should be performed by an expert neurosurgeon. Early recurrence is underreported in the current literature, and we encourage longer term radiological surveillance of these lesions after surgery to optimize primary and recurrent management in the future.

Unusual Presentation and Imaging Findings of a Rare Supratentorial Neurenteric Cyst.

This is a case of a 30-year-old right-handed male patient who presented to the hospital in 2014 after hitting his head on the mat during a wrestling match followed by headache and temporary peripheral vision limitation. The patient's past medical history was unremarkable. On physical examination, Glasgow Coma Scale was 15 with no focal neurological deficits. Unenhanced head computed tomography (CT) and enhanced brain magnetic resonance imaging (MRI) were performed (Figure 1). The patient was managed conservatively, and follow-up CT and MRI in 2015 (Figure 2) demonstrated significant decrease in size of the previously seen right frontoparietal lesion with also changes in its radiological features. The patient remained asymptomatic for about 3 years when in 2018 he presented to the Emergency Department with increasing headaches and peripheral vision loss. MRI demonstrated an increase in the right frontal lesion size (Figure 3). He underwent surgical resection of the lesion.

A Dorsally Located Endodermal Cyst in the Foramen Magnum Mimicking an Arachnoid Cyst: A Case Report.

INTRODUCTION: Endodermal cysts are congenital benign cystic lesions in the central nervous system and cause various symptoms. Although some have been reported in the posterior fossa, endodermal cysts located dorsal to the brainstem are extremely rare. CASE PRESENTATION: The case was of a 10-year-old girl who presented with bilateral upper limb weakness and tremor. Magnetic resonance imaging demonstrated a 4.5-cm cystic lesion with T1-weighted hypointense and T2-weighted hyperintense content in the midline of the cisterna magna dorsal to the medulla oblongata. The cyst was cerebrospinal fluid-like, causing us to suspect a symptomatic arachnoid cyst. The lucent cyst wall had no apparent attachment, and complete recovery ensued following total excision of the cyst wall. Pathology confirmed a diagnosis of endodermal cyst. DISCUSSION/CONCLUSION: Herein, we review the past literature on this rare entity. An endodermal cyst in the cisterna magna tends to be less strongly attached and to show a cerebrospinal fluid-like component on magnetic resonance images that mimics an arachnoid cyst. The characteristics of dorsally located endodermal cysts may differ from those in other locations.

Supratentorial neuroenteric cyst in children: a case report and brief literature review.

Neurenteric cysts are rare developmental lesions typically found outside the central nervous system but when they do, we most likely find them in a spinal (cervical or dorsal) intradural extramedular location, often associated with dysraphism. The more unusual intracranial cases have been published because of its rarity, occurring mostly as a posterior fossa extra-axial cyst, in adults. Supratentorial cases are distinctly infrequent, especially in children, resulting in few case reports and even fewer case reviews. We describe a case of a child with a supratentorial neurenteric cyst and present a brief review of the literature about these cysts in children, a noticeable gap in the literature.

Multiple Neurenteric Cysts along the Spinal Axis of an Infant: A Rare Entity.

A spinal neurenteric cyst is a rare entity. It commonly presents already at 5 weeks of age up to the 6th decade of life. The most common location is the cervical region followed by thoracic and lumbosacral regions. We report a 9-month-old male infant with sudden onset of weakness in both lower limbs. MRI revealed 2 cystic lesions at cervical and thoracic level with spinal cord compression. He underwent laminectomy and excision of the cervical lesion. The child improved significantly. The postoperative MRI shows complete excision of a dorsal lesion and presence of a cervical lesion. Later, he underwent cervical laminotomy and partial wall excision followed by shunt placement. The histopathological report revealed a neurenteric cyst. Two neurenteric cysts presented in the neuroaxis of the same patient: one was located ventral (thoracic) and the other dorsal (cervical). At the 2-year follow-up, the child was active and walking without support. Multiple cystic lesions in the neuroaxis can be neurenteric cysts.

Atypical location of unusual supratentorial neurenteric cyst: a case report.

We report a case of a right anterior clinoid process (ACP) neurenteric cyst which presented with gradual impairment of vision in the right eye. Imaging suggested an infective cystic lesion. In view of rapid visual loss urgent surgery was conducted. Erosion of the ACP was found from where white mucus like material was aspirated. The cyst wall was completely excised. Immunohistochemistry indicated an endodermal origin of the cyst.

Neurenteric cysts, incidence and surgical treatment.

OBJECTIVES: Neurenteric cysts (NCs) of the central nervous system (CNS) are cystic congenital lesions that may occur anywhere along the neural tube. They are most common in the spinal region, in the lower part of the cervical and upper thoracic spine. Intracranial NCs occur rarely and there are only small series of patients published in literature worldwide. Microsurgical resection is the treatment of choice. We present our experience in treatment of NCs and review of literature. METHODS: Seven patients with NC of CNS who were operated at the Department of Neurosurgery of Comenius University at University Hospital Bratislava within nine years (2010-2018) were included in the study. The series was retrospectively evaluated with an emphasis on symptomatology, surgery and postoperative course. RESULTS: In three of the seven patients, NC was localized intracranially, in the other four, NC was in the spinal canal. In three patients, a complete removal of NC was achieved (2 intracranial NC, 1 spinal NC). In other patients, a portion of the cyst wall was left to prevent the development of a postoperative neurological deficit. After surgery, the neurological symptoms were completely resolved in six patients, while in one patient, they were alleviated. In one patient, a complication occurred during the postoperatove course. There was no recurrence during the follow-up (3-111 months, mean duration 39 months). CONCLUSION: In our series of patients with intracranial and intraspinal tumors, the incidence of NC was higher than presented in the published data. Our own surgical experience has shown that complete tumor resection is not always possible for intimate adherence to the surrounding structures. Leaving a tiny portion of the cyst wall allowed us to achieve good clinical results with no recurrence. Long-term follow-up of patients is required due to the risk of recurrence. However, it can already be stated that an adequate extent of resection leads to good clinical results (Tab. 1, Fig. 4, Ref. 33).

Intracranial neurenteric cyst arising at the suprasellar cistern with extension to middle cranial fossa.

INTRODUCTION: Intracranial neurenteric cysts are rare and are not often found in the supratentorial space. We present a case of an intracranial neurenteric cyst that arose at the suprasellar cistern with extension to the middle cranial fossa. HISTORY: A 3-year-old girl presented with seizure. Neuroimaging showed a huge extra-axial cyst involving the suprasellar cistern and left middle cranial fossa. Under the impression of an arachnoid cyst, she underwent fenestration of the cyst with basal cisterns and ventricle. The pathologic diagnosis was neurenteric cyst. Four years after the surgery, MRI showed no recurrence but did show poorly developed myelination of the left temporal white matter. Three years after surgery, seizures recurred. We consider epilepsy surgery in case of uncontrolled seizure. DISCUSSION: We discuss the unique case of supratentorially located neurenteric cyst that is just treated by fenestration. A close follow-up is needed for children with intracranial neurenteric cysts to surveil not only recurrence of lesion but also normal brain development.

Management of an unusual, recurrent neurenteric cyst in an infant: case report and review of the literature.

PURPOSE: Neurenteric cysts are rare congenital remnants formed by a failure of separation between endoderm and ectoderm in utero. METHODS: We describe a case of a 7-month-old male with a large cervical neurenteric cyst presenting with intermittent neck stiffness and irritability. RESULTS: This cyst was resected, recurred, and required repeat surgery. The patient's postoperative course included aseptic meningitis and hydrocephalus requiring ventriculoperitoneal shunt and later management of tethered cord, necessitating detethering. CONCLUSION: Unique features of this case include the presence of intermittent pain symptoms, which may be attributable to cyst filling and emptying. Hydrocephalus is an uncommon finding that may be secondary to aseptic meningitis from cyst rupture. Tethered cord is also an unusual entity that can accompany this diagnosis, warranting additional imaging work-up and monitoring.

[Intracranial neurenteric cysts: experience of the Burdenko Neurosurgical Institute in the XXIth century]. / Intrakranial'naia neiroéntericheskaia kista: opyt Instituta neirokhirurgii im. akad. N.N. Burdenko, v XXI veke.

We present a series of cases of a rare pathology, intracranial neuroenteric cysts, a review of the international literature, and the experience in treating this pathology. MATERIAL AND METHODS: Seven patients with intracranial neuroenteric cysts underwent surgery at the Neurosurgical Institute in the period between 2000 and 2015. CONCLUSION: The main and only technique for treatment of intracranial neurenteric cysts is their resection.

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

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.

Unusual imaging finding of a "fluid-fluid" level in a neurenteric cyst at anterior margin of the cervico-medullary junction.

Intracranial neurenteric cysts are rare. Diagnosis can only be established on histopathology with the demonstration of mucin-secreting cells in cyst wall. We report a midline, ventral, cystic lesion at the cervico-medullary junction, with the unusual radiological finding of a "fluid-fluid" level. Intraoperatively, the cyst contained fluid along with pus-like material with white flakes and specks of calcification, suggesting a dermoid cyst. However, histopathology confirmed the diagnosis of neurenteric cyst. The patient was managed with right lateral inferior suboccipital approach and near-total excision of the lesion.

Neurenteric cyst presenting with stridor and dysphagia: case report and literature review.

OBJECTIVE: Neurenteric cysts are rare lesions that account for 0.7-1.3% of all spinal cord tumours. We report the first ever case of a neurenteric cyst presenting with stridor and dysphagia. A literature review on the presentation and management of these lesions is also included. METHODS: A MEDLINE search of articles using the terms 'neurenteric cyst', 'intraspinal cyst', 'enterogenous cyst', 'intramedullary cyst' along with diagnosis, presentation and management was performed. Suitable references from these articles were also reviewed. RESULTS: All published evidence on neurenteric cysts are either case series or case reports (level IV/V) with the largest case series reporting 23 patients from a single institution. CONCLUSION: Neurenteric cysts are rare spinal cord lesions that usually present with focal neurological signs and managed within neurosurgical units. This is the first reported case of a neurenteric cyst presenting with upper aerodigestive tract symptoms warranting specialist ear, nose and throat input.

Supratentorial neurenteric cyst--a case report.

Supratentorial neurenteric cyst is a rare congenital lesion. We report here a case of a 33-year-old female who presented with seizures. A multicystic lesion in the left premotor cortex with moderate contrast enhancement was demonstrated with MRI. Microscopically, the lesion showed small cystic structures filled with a proteinaceous fluid. The wall of the cysts was lined with a single layer of ciliated columnar or cuboidal epithelium on a basement membrane. Glandular structures resembling gastrointestinal glands were also present. The cells of the cyst lining and glandular structures revealed strongly positive immunoreactions for epithelial markers (cytokeratin and epithelial membrane antigen).