Neuroenteric Cysts Research Articles

Posterior Fossa Arachnoid cysts (PFACs) in pediatric patients: a single-center retrospective study and proposal of a treatment flow-chart.

To investigate how different presentations of posterior fossa arachnoid cysts (PFACs) influence surgical indication and strategies and their impact on clinical and radiological outcome in children, proposing a treatment flow-chart. In this retrospective study, children < 14 years old with PFAC diagnosed at IRCCS Giannina Gaslini Hospital from 2008 to 2023 were identified Patients showing a mega cisterna magna (MCM), Dandy-Walker syndrome (DWS), Blake's pouch cyst (BPC), neuroenteric cysts or multiple cysts were excluded. Data regarding type of treatment, age at surgery, surgical complications were collected and analyzed. Clinical and radiological outcomes were considered at 1-, 3- and 5-years follow-up. A "wait and see" strategy in asymptomatic showed better clinical outcomes at 1-year follow-up (p = 0.047). No significant difference in clinical outcome or risk of re-surgery were observed between microsurgical fenestration, endoscopical fenestration or shunting were reported, while location of the cyst influenced surgical strategy (p = 0.015). Age < 12 months at surgery (p = 0.008), hydrocephalus (p = 0.001), especially when associated with macrocephaly (p = 0.004), and the placement of a shunt (p = 0.006) resulted as risk factors of re-surgery. An association between radiological and clinical outcomes was observed at 1-year follow-up. Treatment decision should be based on clinical presentation rather than radiological presentation. While a "wait and see" strategy is suggested in asymptomatic patients, when surgery is indicated, several factors should be considered, such as presence of hydrocephalus, location of the cyst and age of the patient, to improve clinical outcomes, reducing complications.

Dorsal root entry zone fenestration for intramedullary ependymal cyst: illustrative case.

Intramedullary ependymal cysts are rare and difficult to distinguish from syringomyelia and neuroenteric cysts. Almost all cases in the literature have been case reports and have been performed with the traditional posterior median sulcus incision, which is difficult to identify accurately during spinal rotation. Approximately 40% of cases have transient neurological deterioration. The dorsal root entry zone has been proven to be an effective incision area in the treatment of intramedullary lesions, but so far, its utilization in intramedullary ependymal cysts has been rarely reported. This study is the first to report on six cases of intramedullary ependymal cysts treated with an 8-mm incision in the dorsal root entry zone to fully establish the communication between the cyst and the subarachnoid space. Imaging changes and neurological improvement were analyzed in all cases before and after surgery and were followed up for 49.7 months. The utilization of dorsal root entry zone fenestration in intramedullary ependymal cyst has demonstrated feasibility and effectiveness, ensuring the functional integrity of the posterior column.

Ethmoidal sinus osteoma associated with Pneumocephalus

The most common causes of intracranial air are head trauma and neurosurgical procedures. Less common etiologies include infection due to gas-forming organisms, mucoceles, tumors, congenital neuroenteric cysts, and dural defects. Here, we present a case of an ethmoidal sinus osteoma associated with pneumocephalus. Osteomas of the paranasal sinuses are benign, often asymptomatic, tumors that progress very slowly. Endocranial development of an osteoma can breach the dura mata, allowing air to enter the cranium producing pneumocephalia which leads to severe neurological deficiencies. Pneumocephalia is an exceptional complication of osteoma.

A Neuroenteric Cyst of the Cavernous Sinus: A Case Report.

Background Neuroenteric cysts (NECs) are benign lesions mostly found as intradural extramedullary lesions in the cervicothoracic spinal cord. NECs in the cavernous sinus are very rare. To the best of our knowledge, this is only the second reported case and the first in an adult. Presentation We present a left cavernous sinus NEC in a 75-year-old female with gradually worsening headache and facial pain unresponsive to medical treatment. Imaging revealed a cystic mass lesion in the left cavernous sinus encasing the distal petrosal and cavernous segment of the internal carotid artery. Initial differential diagnoses included more common pathologies located near the cavernous sinus, including cystic schwannoma, craniopharyngioma, and dermoid and epidermoid tumors. The patient underwent a left pterional craniotomy with an extradural transcavernous approach for surgical exploration and possible resection of this mass lesion. Histopathology revealed an NEC lined with benign respiratory-type epithelium. Postoperative imaging revealed gross total tumor resection. The patient remained neurologically intact with complete resolution of facial pain. Conclusion We present a rare pathology that can easily be misinterpreted as other types of lesions. NECs should be kept in mind for differential diagnosis of cavernous sinus cystic lesions. The surgical aim should be maximal safe excision.

Cysts and cyst-like formations of the mediastum: clinical and radiological manifestations

. Mediastinal cystic formations are well-shaped, often round formations that contain fluid and are lined with epithelium. The main cystic formations include congenital benign cysts (bronchogenic, duplicating the esophagus, neuroenteric, pericardial and thymic cysts), meningocele, mature cystic teratomas and lymphangiomas. Many tumors (e.g., thymomas, Hodgkin's disease, germ cell tumors, mediastinal carcinomas, lymph node metastases, nerve root tumors) may undergo cystic degeneration, especially after radiation or chemotherapy, and show mixed solid and cystic elements on computed tomography (CT) or magnetic resonance imaging (MRI), as well as acquire the structure of cavity formations during the tumor destruction. If the degeneration is extensive, such tumors may be virtually indistinguishable from con­genital cysts. However, the history of the disease, visualization of the anatomical location of the formation, some details visible on CT or MRI, allow in many cases to make the correct diagnosis. Familiarity with the radiographic features of mediastinal cystic lesions facilitates accurate diagnosis, differentiation from other cystic lesions, and thus ensures optimal treatment of a patienta.

Single-Center Experience of Management of Mediastinal Cyst-A Case Series.

Background Mediastinal cysts are benign lesions that may be seen in adulthood as well as in childhood. Mostly congenital lesions constitute 20 to 32% of lesions located in the mediastinum. The main cystic masses are congenital benign cysts (bronchogenic, esophageal replications, neuroenteric, pericardial and thymic cysts), meningocele, mature cystic teratoma, and lymphangioma. In this study, we aimed to analyze the mediastinal cysts operated in our clinic according to the histopathological type, surgical type, morbidity rates and to contribute to the literature on these rare lesions. Methods The records of patients with mediastinal cysts who were operated in Gazi University Faculty of Medicine Department of Thoracic Surgery, between January 2013 and June 2021, were reviewed retrospectively. Results A total of 32 patients were included the study. Thirteen (40.6%) of the patients were male and 19 (59.4%) were female. The mean age was 45 (range: 12–71). The most common symptom in patients was chest pain with 12 patients. Histopathologically, the most common subtype was thymic cyst. Video-assisted thoracic surgery was applied in 19 patients (59.3%), thoracotomy in 8 patients (25%), and sternotomy in 5 patients (15.63%). There was no mortality. Conclusion In patients with mediastinal cysts, the prognosis after complete excision is excellent and rates of morbidity and mortality associated with surgery are low.

Ventral Cervical Intradural Extramedullary Neuroenteric Cyst and its Operative Procedure; A Rare Case Report with Literature Review

Background:Neuroenteric cysts are rare non-neoplastic lesions arising from a failure of dissolution of the transient neuroenteric canal between the foregut and the notochord. They are most frequently seen in the intraduralextramedullary space in the lower cervical and upper thoracic spine. Case description:A 5 yrs old boy presented to us with the complaints of neck and upper back pain and weakness of all four limbs. MRI scan shows an intraduralextramedullary cystic lesion at C6-T1 with significant compression over cord. After patient’s preparation a Posterior approach was used to remove the cysts. Post-operative course was un-eventful. Histological results were consistent with neuroenteric cysts. MRI image of 3 months follow-up shows no residual cysts and the boy has no further complaints. Conclusion:Neuroenteric cyst (NC) is a rare lesion usual location at lower cervical and upper dorsal area and should be considered among differential diagnoses. Complete excision is the treatment of choice. In most instances a dorsal surgical approach will be satisfactory. Bang. J Neurosurgery 2021; 11(1): 54-57

Microforceps in the Diagnosis of Pancreatic Bronchogenic Cyst Under Endoscopic Ultrasound Guidance

ABSTRACTBronchogenic cysts belong in the category of foregut duplication cysts, along with esophageal and neuroenteric duplication cysts. They are often found incidentally on cross-sectional imaging. Although they are benign, patients often undergo surgical resection because of diagnostic uncertainties. We report the first case of bronchogenic cyst diagnosed with the microforceps, after previous nondiagnostic fine-needle aspirations, thus allowing the patient to avoid surgery.

Intracranial neuro-enteric cyst: an illustrative patient and review

Neuroenteric cysts are embryological remnants. Considered as benign congenital lesion, they can occur in the posterior fossa, along the midline structures. We report a case of a 28 years old female presenting with headaches She was diagnosted with a small lesion in the posterior fossa, anterior to the ponto-medullary junction, and seemed to have a cyst conduct through the inferior clivus appearing to be a neuroenteric cyst.

A RARE CASE OF NEUROENTERIC CYST INVOLVING THE ANTERIOR FOSSA; REPORT OF A CASE AND REVIEW OF LITERATURE

Neuroenteric cysts, are rare benign endodermal lesions which mostly occur in the central nervous system. We report a case of a neuroenteric cyst in a 30-year-old man who presented with rhinoliquorrhea at the ENT department. After clinical examination, a semisolid mass was revealed in the left nostril. The Computer Tomography Scan revealed a frontal ethmoidal nasal meningoencephalocele with inferior extension into the left nostril. Gadolinium-enhanced T1-weighted MR images showed a well-defined frontal mass with ring-like enhancement and extension into the cribriform plate of the ethmoid bone and into the left nostril. The lesion measured 10/10/20 mm. The tumor was totally resected using a unilateral subfrontal approach. At five months’ follow-up, the patient showed significant amelioration of symptoms and remission of cerebrospinal fluid leakage. Native and Contrast-enhanced Cerebral Computer Tomography, as well as Magnetic Resonance Imaging showed total surgical resection of the cyst. Supratentorial neurenteric cysts involving the anterior fossa are rare. Intracranial neurenteric cysts should be differentiated by any well-demarcated cystic tumors. The gold standard treatment remains complete surgical resection with favorable outcome.

Endodermal Cyst of the Third Nerve in a Child

Endodermal cysts, also known as neurogenic, neuroenteric, foregut, bronchogenic, respiratory, epithelial, teratomatous, or gastrocytoma cysts, can be found in the central nervous system, predominantly in the subarachnoid space of the cervical and thoracic spinal cord. We describe a child with an endodermal cyst of the third nerve and highlight neuroimaging findings, pathological correlation, clinical course, and patient management.

Neuroenteric cyst of the left lateral ventricle: case report and review of the literature

BackgroundIntracranial supratentorial neuroenteric cysts are very rare lesions in the central nervous system. We described a neuroenteric cyst of the left lateral ventricle in a 38-year old male, which had been confirmed by using immunohistochemistry. To date,this was the first reported case in which the neuroenteric cyst was located in the lateral ventricle.Case presentationThe patient had paroxysmal headache for half a year which exacerbated in the recent two months. MRI showed a 3cm*3cm*2.5cm cyst in the left lateral ventricle. We reported the first case, where the NC in the lateral ventricle was partly resected, provided new evidence to develop the hypothesis on NC etiology. The cyst was left partly due to the strong adherence to the surrounding structures. The final diagnosis was confirmed on histopathological and immunohistochemical analysis of the cyst.ConclusionLacking in characterization of the specific imaging features, neuroenteric cyst may be misdiagnosed as other types of cysts preoperatively. At present, surgical excision is the only effective treatment for neuroenteric cysts. Early diagnosis and more treatment options may be available in the future with more cases of neuroenteric cyst being reported and its pathogenesis being understood further. we hope the first reporting of the lateral ventricle NC by now can provide new evidence to NC etiology.

Intracranial neurenteric cysts: experience of the Burdenko Neurosurgical Institute in the XXIth century

Seven patients with intracranial neuroenteric cysts underwent surgery at the Neurosurgical Institute in the period between 2000 and 2015. The main and only technique for treatment of intracranial neurenteric cysts is their resection.

Multiple Ecchordosis Physaliphora

Multiple Ecchordosis Physaliphora

Neuroenteric cyst: A rare cause of childhood hemiparesis

Neuroenteric cysts are rare congenital anomalies derived from the displaced endodermal tissue at around third embryonic week. These are commonly encountered in the mediastinum and rarely seen in the spinal canal in the intradural extramedullary and at times in intramedullary location. We report a case of neuroenteric cyst of spinal canal presenting with hemiparesis in a 12 years old female child. The patient had no associated underlying occult spinal deformity. On investigation her magnetic resonance imaging, revealed evidence of well-defined, intradural extramedullary cystic lesions at C6 to D2 vertebral level, situated anterior and right to the cord causing posterior and leftward displacement with marked cord compression at this level. The child underwent posterior laminectomy and excision of cyst with complete recovery and no residual deficit.

Surgical Management of Intracranial Neuroenteric Cysts: The UCSF Experience.

Objective Modern surgical experience with intracranial neuroenteric cysts is limited in the literature. We review our 15-year institutional experience with these rare lesions. Design Single-institution retrospective study. Setting Large North American tertiary care center. Participants Histologically confirmed cases of intracranial neuroenteric cyst from January 2000 to September 2014. Main Outcome Measures Pre- and postoperative modified Rankin Scale (mRS) scores, extent of resection, and postoperative complications are reported. Clinical presentation, imaging features, pathology, and operative approach are discussed. Results Five spinal and six intracranial neuroenteric cysts were surgically treated over a 15-year period. Median age at presentation for the intracranial cysts was 38.5 years. Mean cyst diameter was 3.8 cm. Five cysts were located in the pre-pontomedullary cistern, and one was located in the third ventricle. Gross total resection was achieved in four of the five posterior fossa cysts through a far lateral transcondylar approach. Postoperative complications included aseptic meningitis (one), transient abducens palsy (one), and pseudomeningocele requiring reoperation (three). Postoperative mRS scores improved to ≤1 by 6.5 months median follow-up. Conclusions Intracranial neuroenteric cysts are rare lesions with a variable imaging appearance. Complete surgical resection through a far lateral transcondylar approach is possible and usually results in symptom improvement or resolution.

Mucinous adenocarcinoma arising from supratentorial intramedullary neuroenteric cyst with broncho‐pulmonary differentiation

Neuroenteric cysts are benign intradural endoderm cysts lined by gastrointestinal (GI) or tracheobronchial epithelial cells. Their malignant transformation is extremely rare and only six cases have been reported. In these cases, tissue lineage of the cystic endoderm cells giving rise to carcinoma was not clearly identified either as respiratory or as GI type. Herein, we report a case of mucinous adenocarcinoma arising from the neuroenteric cyst with broncho-pulmonary differentiation in the right cerebral hemisphere of a Japanese woman in her late 50s. The cyst wall was entirely lined by the following respiratory epithelial components: stratified bronchial ciliated columnar epithelium with basal cells positive for CK5 and p63, terminal bronchiolar Clara cells positive for thyroid transcription factor (TTF)-1, surfactant B and negative for surfactant C, type I pneumocytes positive for TTF-1, negative for surfactant B and C, and type II pneumocytes positive for TTF-1 and surfactant B and C. In addition, nests of hyperplastic single layered mucinous epithelial cells with bronchial goblet cell phenotype were also demonstrated, and histologic features were almost the same as the pulmonary type I congenital adenomatoid malformation (CCAM) with mucinous epithelial proliferation. The mucinous epithelial nests of type I CCAM are liable to develop mucinous adenocarcinoma and frequently accompany K-ras mutation and expression of p16. However, K-ras mutation and p-16 expression were not detected in this case.

Neuroenteric cysts of the brain-comprehensive magnetic resonance imaging

Neuroenteric cysts are developmental cysts that should be differentiated from other, more common non-neoplastic cysts as well as cystic neoplasms. While these lesions may have varied imaging findings, T1 hyperintense prepontine lesion due to mucinous/proteinaceous content is characteristic. Location and imaging characteristics aid in formulating the correct diagnosis of neuroepithelial/neuroenteric cysts. Magnetic resonance spectroscopy (MRS) has the specific finding of N-Acetyl Aspartate (NAA)-like peak at 2.02 ppm which is not seen in other cystic lesions. In this study, we aim to discuss the imaging findings of these lesions on conventional and advanced MRI findings and provide radiologic-pathologic correlation. We also briefly describe the pathogenesis, embryology and radiological differential diagnoses of these cysts.

Mullerian cysts of the posterior mediastinum: report of two cases and review of the literature.

Cystic lesions can be occasionally be found in the mediastinum, and typically include bronchogenic cysts, esophageal duplication cysts, and neuroenteric cysts. In 2005, Hattori described the first mediastinal cyst with Mullerian differentiation. Since that time, three other authors have described similar cysts occurring in the posterior mediastinum. Here we present two cases of patients with ciliated cysts with Mullerian differentiation with expression of estrogen receptor, progesterone receptor, PAX8 and Wilm's tumor 1, occurring in the posterior mediastinum and review the related literature.

Hemorrhagic Spinal Neuroenteric Cyst: A Case Report

Introduction: Neuroenteric cysts are a rare type of spinal tumors that result of anomalies during embryonic development. A case with a rare haemorrhagic presentation is reported. Case report: 42 years old male that shows an initial clinical picture consisting of cervical pain. Cervical Magnetic Resonance (MR) reveals a lesion intradural-extramedullar at level C2-C3. Findings are in correlation with hemorrhagic lesion in reabsorption phase. A surgical procedure is performed. Through a bilateral C2-C3 and partial C1 laminectomy the cyst was emptied. The rest of membranes are removed. The patient shows a progressively improvement. The hystopathological analyses find match neuroenteric cyst type A. Discussion: 58 cases of neuroenteric spinal cysts reported in literature are analyzed. Total excision was performed in about 34%. There was any finding about hemorrhagic presentation in neuroenteric cyst in these cases.