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Abstract
The case of a cerebellopontine angle enterogenous cyst is presented in a 30 year old patient. This very rare tumour and its highly unusual localisation raises questions in relation to its embryogenesis. The literature is reviewed and 3 previously reported cases of endodermal cysts located in the posterior fossa and away from the midline are discussed.
... The most common location is the posterior fossa. Other locations include the cerebellopontine angle, fourth ventricle, supratentorial and foramen magnum, or craniovertebral junction [6,12,19,40,41,50,53]. The foramen magnum is classically defined as the area from the lower [4,22]. ...
... An anterior approach, such as the transoral one, has also been reported successfully by a few, although the majority prefer to utilize the posterolateral approach [14,15,19,42,50,54]. In rare instances, chemical meningitis has been reported following surgery for NEC; hence, cysts should be handled with caution to avoid spillage of cyst contents [40,54]. Wang et al. utilized a technique wherein they first aspirated the cyst with a syringe before incision of the cyst wall, protected the subarachnoid space with cotton chips, and used irrigation with saline at body temperature [54]. ...
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.
... Intracranial enterogenous cysts are extremely rare with most of them located in the posterior fossa. The literature notes locations of cysts are as follows: within 4th ventricle [6], cerebellopontine angle [7], ventral brainstem [8], cerebellar vermis [9], within the medulla [10], and at the foramen magnum [11]. Supratentorial cysts are exceedingly rare with only 20 cases reported worldwide [12]. ...
Objective and Importance: The authors report the first known case of a giant multiloculated grape-like enterogenous cyst extending from the upper cervical canal to the ambient cistern. Clinical Presentation/Methods: We report the case of a 40-year-old male who had a prior transmastoid craniotomy at an outside facility 14 months prior with an indeter-minate diagnosis, who presented to the University of New Mexico with recurrent headaches and nausea. Scans demon-strated a giant multiloculated cystic lesion in the right cerebellopontine angle that extended superiorly and inferiorly with brainstem compression and hydrocephalus. Intervention/Results: We took the patient to the operating room for a retrosigmoid suboccipital craniectomy for tumor resection. Post-operatively, the patient improved but required ven-triculoperitoneal shunting for continued communicating hydrocephalus. Conclusion: This is the first known case of a giant multiloculated grape-like enterogenous cyst extending simultaneously from the upper cervical canal to the ambi-ent cistern. Enterogenous cysts should be considered on the differential diagnosis of giant grape-like lesions extending from the cervical canal to the prepontine cistern.
... Rarely, a neurenteric cyst has been documented as a slowly expanding lesion. Ito et al found a small increase in the size of a cerebellopontine angle cyst by CT, 6 years after the initial examination.36 Shakudo et al noted a change in the size of a neurenteric cyst anterior to the brain stem over 33 months.37 ...
Neurenteric cysts are considered congenital lesions that may slowly expand over time. Although more commonly found in the spinal canal, they may be found intracranially, particularly in the posterior fossa. Here, we present an unusual case of a large, rapidly expanding histologically confirmed posterior fossa neurenteric cyst in a 53-year-old woman, who presented with quadriparesis. Computed tomography imaging done ~1.5 years before admission failed to demonstrate any obvious abnormality; however, the lesion had grown to 4 cm in maximal dimension at presentation with significant mass effect. The lesion was resected microsurgically using a retrosigmoid approach. The patient improved postoperatively and was neurologically intact at last follow-up. We could find no other documented case of marked, rapid expansion of a neurenteric cyst in the literature. We conclude that, although neurenteric cysts are thought to be congenital, they can undergo rapid expansion even in adults. The mechanism of expansion is unknown but may involve increased secretion, hemorrhage, or inflammation. We discuss the surgical management and review the literature in view of this surprising finding.
Objective:
To retrospectively analyze the clinical data of 6 foramen magnum neurenteric cyst patients and summarizethe clinical characteristics and treatment experience for this rare disease in our single center.
Methods:
Between January 2011 and December 2015, 6 foramen magnum neurenteric cyst patients were surgically treated in Xuan Wu Hospital of Capital Medical University. We summarized the treatment experience through retrospective review of the clinical informations, imaging features, surgical details and follow up outcomes of all those 6 patients.
Results:
The 6 patients were all female, age ranging from 15 to 54 years old(36.8±12.9). Occipital headache combined with cranial nerve injury were the most common symptoms. The preoperative brain magnetic resonance imaging showed all lesions were located at the foramen magnum region, presented with oblong or lobulated shape. Intraoperatively, 4 patients were treated by far lateral approach and 2 cases were operated by suboccipital midline approach. Total removal of lesion was completed in 4 patients while subtotal excision was performed in other 2 patients whose cyst wall was intensely adherent to surrounding structures. The preoperative symptoms of all 6 patients were significantly relieved after operation. No recurrence was seen after a mean follow up of 27.3 months(3 to 70 months).
Conclusions:
The current study found a female predominance among patients with intracranial foramen magnum neurenteric cyst. Surgical excision is the optimum treatment strategy for this rare disease. The current study indicates that subtotal removal of foramen magnum neurenteric cyst could also acquire favorable outcomes, while strict long-term follow up is needed.
Neurenteric cysts are rare, benign, endodermally derived tumors of the central nervous system. Intracranial neurenteric cysts are rare with posterior fossa being the most common location among them. Neurenteric cyst of the craniocervical region is very rare. Authors report a rare case of neurenteric cyst located in the ventral cervicomedullary region. The pertinent literature is reviewed regarding this uncommon entity.
Two cases of endodermal cyst of the posterior fossa are reported. A 12-year-old girl presented with severe headache and vomiting caused by increased intracranial pressure. Computed tomography and magnetic resonance (MR) imaging showed a cystic mass occupying the ambient and quadrigeminal cisterns. A 65-year-old woman presented with dizziness, and MR imaging revealed a cystic mass in the posterior fossa. The two patients underwent surgery for decompression and resection of the cyst. Surgical specimens of the cyst walls consisted of a single layer of ciliated columnar epithelium. The diagnoses were endodermal cyst. The optimal surgical goal is total resection of the cyst wall, but the cyst wall sometimes tightly adheres to the adjacent nerves, vessels, and vital structures. The cyst must communicate adequately with the surrounding cerebrospinal fluid space, and a newly closed cyst space must be avoided, by the widest possible resection of the cyst wall.
We report a case of a neurenteric cyst of the cerebellopontine angle and review the five previously reported cases. The pathology and classification of these cysts are discussed.
The patient presented with a 1-month history of nausea, vomiting, vertigo, and sudden hearing loss.
The cyst was decompressed by a retrosigmoid approach. After recurrence of symptoms at 2 months, further decompression was required.
The patient achieved a good outcome after the second operation, with cessation of her vomiting and vertigo, although she had residual hearing loss. Four of the five previously reported patients experienced satisfactory outcomes after surgery. The definitive diagnosis of these rare lesions is made using immunocytochemical techniques.
Intracranial neurenteric cysts are rare, benign lesions of the central nervous system.
To analyze our experience with these lesions and conduct a review of the literature with the aim of identifying a rational approach to diagnosis and management.
Seven patients underwent surgical treatment for intracranial neurenteric cysts from July 2000 to December 2008. The general strategy was to perform complete resection whenever possible. The clinical, radiological, operative, and pathological findings of the patients were reviewed retrospectively.
This series included 3 women and 4 men. The age of hospitalized patients ranged from 19 to 55 years, with an average age of 34 years. Headache and dizziness were the most common chief symptoms. The imaging spectrum for these lesions is broad, leading to several preoperative misdiagnoses. All 7 lesions were resected by a far lateral transcondylar or retrosigmoid approach. Total tumor resection was achieved in 3 patients. Four patients presented transient fever postoperatively. All patients were healthy and showed no signs of tumor recurrence at their latest follow-up.
Intracranial neurenteric cysts ventral to the brainstem are rare lesions occurring in young adults. These lesions should be considered in the differential diagnosis for intracranial extra-axial cystic lesions anterior to the brainstem. Total surgical resection seems to be the most effective therapeutic method, although radical resection at the risk of impairing the brainstem is not recommended. This benign lesion has a favorable overall prognosis.
Los quistes endodérmicos que afectan al sistema nervioso central son lesiones expansivas muy poco frecuentes que se sitúan con mayor frecuencia a nivel espinal. Existen poco casos de localización intracraneal descritos en la literatura, la mayoría de ellos en la fosa posterior. Su etiopatogenia permanece aún desconocida. Presentamos el caso de un varón de 62 años que debuta con un cuadro de desorientación y comportamiento desinhibido tras sufrir un traumatismo craneoencefálico como consecuencia de un accidente de tráfico. En la TC craneal realizada de urgencia se objetiva una lesión quística frontal izquierda de gran tamaño con importante desplazamiento de línea media. Se punciona y evacua el contenido del quiste obteniéndose un líquido opalino rico en proteínas y elementos celulares no identificados. La RMN nos confirma los hallazgos radiológicos previos. El paciente es intervenido de forma reglada mediante craneotomía, evacuación completa del contenido y extirpación de las paredes de la lesión. El estudio anatomopatológico resulta ser compatible con el diagnóstico de quiste endodérmico. Se han descrito casos de evolución agresiva con diseminación y recidiva tras manipulación quirúrgica de la lesión; por lo tanto, el tratamiento debe consistir en la extirpación completa de la misma. Para ello será necesario realizar el diagnóstico diferencial con otras lesiones quísticas intracraneales con el fin de adecuar el tratamiento a cada caso.
We report three cases of enterogenous cyst, one craniospinal and two spinal manifesting with features of spinal cord compression. In one the cyst was intramedullary in location and in the other two it was intradural, extramedullary. The unusual clinical presentation, location and magnetic resonance imaging (MRI) characteristics are discussed. None of the three patients had any vertebral anomaly or other congenital malformation. All patients had excellent outcome following surgery.
Two cases of entirely suprasellar cysts are reported. Total surgical removal was performed in both cases. Pathological and immunohistochemical profiles were consistent with neurenteric cysts, Rathke's cleft cysts or colloid cysts and was also in keeping with an endodermal origin. It is now admitted that these three kinds of cysts share similar histological and immunohistological features. We propose an hypothesis of common embryological origin from endodermal remnants.
Endodermal cysts are rare lesions that primarily affect the spinal cord. Only a few reports have been published that describe intracranial endodermal cysts, and, in most of the cases, the cysts were located in the posterior fossa. To our knowledge, there have been no reports of endodermal cysts of the third ventricle.
A symptomatic endodermal cyst of the third ventricle in a 28-year-old man is presented. The patient demonstrated signs of increased intracranial pressure and reported a progressive loss of vision in his right eye. Neuroradiological imaging revealed obstructive hydrocephalus and a lesion inside the third ventricle measuring 1.2 cm in diameter and occluding the interventricular foramen.
The cyst was successfully removed via a transcallosal-transforaminal approach. Postoperatively, the patient developed bifrontal effusions for some days. Hydrocephalus was diminished, but his vision had only slightly improved.
To our knowledge, this is the first published report of an endodermal cyst occurring in the third ventricle, and represents a further example of the broad spectrum of lesions affecting that location.
The pathogenesis of endodermal cysts of the posterior fossa is still incompletely understood. The authors reviewed three new cases and those reported in the literature to clarify the clinical, pathological, radiological, and surgical characteristics of these lesions. A total of 49 cases were reviewed. Details on demographic profiles, clinical characteristics, histopathological and radiological features, and surgical methods were collected.
These cysts have a predominance in male patients (61%) and can occur at any age (birth–77 years). In patients with posterior fossa endodermal cysts there is a bimodal age distribution and headache is the most frequent complaint. On immunohistopathological examination, endodermal cysts were reactive for epithelial membrane antigen and for keratin immunostains whenever the latter were tested. The cysts were reactive for carcinoembryonic antigen in nine of 11 cases. Endodermal cysts were located anterior to the brainstem in 51% of cases and in the fourth ventricle in 21% of cases. They frequently appeared hypodense on computerized tomography scans, and in five cases, the lesion was missed. The cyst's appearance on magnetic resonance imaging is variable. Resection was complete in 19 cases and partial in 11; marsupialization was achieved in two cases. Three recurrences have been reported.
Total excision with preservation of neurological function should be the goal. Cranial base approaches are helpful for surgical access in selected examples of these lesions.
Considered benign and malformative in nature, enterogenous cysts are predominantly spinal lesions. Although recurrences have been reported, especially in the rare intracranial examples, craniospinal dissemination has never been described.
We report a 63-year-old woman who presented 16 years previously with a histologically typical enterogenous cyst of the cerebellum. Fourteen years after its incomplete excision, numerous supratentorial, infratentorial, and spinal subarachnoid cysts were detected. A histological examination revealed multiple enterogenous cysts without evidence of malignancy. Ki-67 (MIB-1) proliferative indices were less than 1% in all except one specimen, wherein it was 4%; immunohistochemistry demonstrated rare p53 protein overexpression.
During the last 2 years, four subtotal resections of spinal enterogenous cysts have been performed for progressive paraplegia, pain, paresthesia, and bladder dysfunction. Considering the multiple recurrences and progressive neurological deficits, craniospinal radiation therapy was given.
To our knowledge, this is the first report of widespread craniospinal dissemination associated with the incomplete resection of an enterogenous cyst. Although a rare complication, it emphasizes the advantages of early, aggressive surgery.
Colloid cysts are rare intracerebral lesions that are preferentially encountered within the third ventricle. There are only a few reports in which colloid cysts are described in other locations such as the fourth ventricle.
A symptomatic intracerebellar colloid cyst in a 45-year-old woman is described. The patient presented with headache, gait disturbance, and nausea. Neuroradiological imaging revealed compression of the fourth ventricle, hydrocephalus, and an intracerebellar cystic lesion measuring 4 × 5 cm that had a small peripheral solid portion. The cyst was successfully removed via a paramedian suboccipital approach. Postoperatively, the patient recovered quickly.
The findings in the present report represent an additonal example of the broad spectrum of cystic lesions encountered in the cerebellum.
Tumors of the cerebellopontine angle (CPA) are frequent; acoustic neuromas and meningiomas represent the great majority of such tumors. However, a large variety of unusual lesions can also be encountered in the CPA. The site of origin is the main factor in making a preoperative diagnosis for an unusual lesion of the CPA. In addition, it is essential to analyze attenuation at computed tomography (CT), signal intensity at magnetic resonance (MR) imaging, enhancement, shape and margins, extent, mass effect, and adjacent bone reaction. CPA masses can primarily arise from the cerebellopontine cistern and other CPA structures (arachnoid cyst, nonacoustic schwannoma, aneurysm, melanoma, miscellaneous meningeal lesions) or from embryologic remnants (epidermoid cyst, dermoid cyst, lipoma). Tumors can also invade the CPA by extension from the petrous bone or skull base (cholesterol granuloma, paraganglioma, chondromatous tumors, chordoma, endolymphatic sac tumor, pituitary adenoma, apex petrositis). Finally, CPA lesions can be secondary to an exophytic brainstem or ventricular tumor (glioma, choroid plexus papilloma, lymphoma, hemangioblastoma, ependymoma, medulloblastoma, dysembryoplastic neuroepithelial tumor). A close association between CT and MR imaging findings is very helpful in establishing the preoperative diagnosis for unusual lesions of the CPA.
Epithelial cysts of the central nervous system are unusual entities, with the majority reported to occur in the spinal canal. More unusual is the intracranial presentation, which shows a predilection for midline localization in the posterior fossa, brainstem, and suprasellar regions. This report discusses the differential diagnosis, pathogenesis, radiographic presentation, and therapeutic considerations of a laterally positioned cerebral convexity endodermal cyst.
A 49-year-old right-handed man presented with approximately a 30-month history of short- and long-term memory difficulties. A magnetic resonance imaging study revealed a large, nonenhancing, extra-axial cystic lesion overlying the right lateral frontal convexity.
A right frontoparietal craniotomy exposed a large extra-axial cyst with an opaque, yellowish-white membrane containing a mucoid fluid. Histological analysis disclosed a layer of unremarkable, ciliated columnar epithelium with a basement membrane that stained positive for cytokeratin, periodic acid-Schiff, and Alcian blue. No evidence of either a muscular or cartilaginous layer around the mucosa was present to further delineate neurenteric versus bronchogenic origin of the cyst.
This case involved the occurrence of a solitary endodermal cyst as an extra-axial mass localized over the lateral frontal lobe. The lateral supratentorial localization of this lesion illustrates the need for consideration of the pathogenesis of this entity as well as its diagnostic differentiation from other cystic abnormalities in this region. A review of the histochemistry of endodermal, neuroepithelial, and other cerebral cysts is presented.
A 46-year-old woman presented with multiple neurenteric cysts in the posterior fossa and spinal canal. Neuroimaging demonstrated neurenteric cysts in the interspace between the left cerebellar hemisphere and vermis, the lateral side of the right cerebellar hemisphere, and the ventral side of the spinal cord at the C-2 and C-4 levels. Total resection of the paravermian cyst and partial removal of the spinal cyst at the C-4 level were performed. Histological examination showed the cyst wall consisted of single or multiple layers of columnar epithelial cells with secretory granules, with mucin secretion verified by periodic acid-Schiff staining. Immunohistochemical staining showed the walls were positive for the cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen, and negative for glial fibrillary acidic protein and S-100 protein. These findings confirmed the endodermal origin. The diagnosis was neurenteric cyst. The paravermian cyst disappeared, but the spinal cyst at the C-4 level recurred 8 months later. Reoperation became necessary 16 months later. The other two cysts also showed enlargement at 6 or 15 months. Total removal of neurenteric cyst is recommended if possible.
Neurenteric cyst is a developmental malformation found mainly in the spinal canal. The authors report on a 47-year-old man with a neurenteric cyst of the cerebellopontine angle (CPA) who presented with progressive hearing disturbance and facial palsy. The tumor was located extradurally with marked destruction of the petrous bone around the internal auditory canal and demonstrated irregular and heterogeneous high-intensity signals on T1- and T2-weighted on MR images, which is atypical for neurenteric cysts. The pathological findings in samples obtained after resection disclosed a single epithelial layer (a feature of neurenteric cyst), which was accompanied by marked xanthogranulomatous changes. Although several neurenteric cysts have been reported in the CPA, extradural lesions with unusual imaging features and marked bone destruction have not been reported previously. This benign developmental lesion should be considered, although it is extremely rare, in patients harboring an extradural temporal bone tumor around the CPA.
Endodermal cysts are commonly found in the anterior spinal cord. Reports of intracranial cases are uncommon. We report a case of endodermal cyst located on the left oculomotor nerve.
A 16-year-old female experienced diplopia followed by headache. Neurological examinations revealed left oculomotor nerve palsy. Magnetic resonance imaging scans showed a well-defined multilocular cystic mass in the proximity of the left oculomotor nerve at its exit from the midbrain.
This patient underwent biopsy and drainage of the cyst. At surgery, the cyst seemed to be intrinsic to the oculomotor nerve and could not be totally resected. Pathological examination confirmed the diagnosis of endodermal cyst.
Intracranial endodermal cysts are rare, particularly when associated with the cranial nerves. To our knowledge, this is the second case of endodermal cyst located on the oculomotor nerve. A detailed discussion and review of these case reports are presented.
Intracranial enterogenous cyst is an uncommon entity. We report a case of enterogenous cyst of the posterior fossa situated posterolateral to the brain stem and extending into the left CP angle cistern. Patient had spontaneous regression and recurrence of the cyst. Clinical features and radiological findings are described. Near total excision of the cyst was carried out through the retro mastoid route. Patient made a good postoperative recovery.
Surgery was considered to be the only option to control the progressive dyspnea and arrest the neurological deterioration. It was performed in two stages, by two teams, six days after admission. After a left thoracotomy, the lesion located in the posterior mediastinum was removed. Its nature was cystic, full of dark, turbid, mucous fluid, and its isthmic part was ligated deep inside a canal-like defect between several malformative vertebral bodies. Through, a separate approach, an upper and midthoracic laminectomy was done and an enlarged fusiform dural sac was exposed. A midline incision of dura revealed a cystic lesion integrated with the spinal cord in one portion and extending ventrally through the defect between the vertebral bodies. The cyst was evacuated and it contained the same type of fluid as the mediastinal part. The wall was dissected from the spinal canal and resected. A small part, integrated with the continuity of the spinal cord (there was no detectable border between the cyst and the cord under the surgical microscope), was left in place and the internal layer of the cyst coagulated and excised. The postoperative period was uneventful for the first two days. After that, the patient developed obstruction of the right principal bronchus, later on complicated with a bronchopleural fistula. The patient's condition after the ninth postoperative day deteriorated severely and despite the rethoracotomy, the patient expired because of cardiac arrest and failed resuscitation.
Intracranial enterogenous cysts are an uncommon entity rarely found in the midline within the posterior cranial fossa. The occurrence of an enterogenous cyst in the cerebellopontine angle is exceptional. We present two new cases of cerebellopontine angle (CPA) enterogenous cysts and review the literature to clarify the diagnosis and the management of these lesions.
Eighteen cases of CPA intradural enterogenous cysts have been reported to date, including the two cases presented in this article. All of them were symptomatic and underwent surgical treatment. After surgery, the symptomatic recurrence occurred in 31% of the patients, most of which had partial excision. Considering our patients and the published cases in the literature we suggest that the aim of surgery should be total removal of cyst and its content whenever possible. When partial resection of the cyst is performed, we recommend long-term clinical and neuroradiological follow-up.
We reviewed 36 patients with endodermal cysts occurring at the craniocervical junction. They were aged between 3 and 66 years. Headache, motor weakness, and neck pain were commonly observed symptoms. Radiographically, T1-weighted magnetic resonance imaging of the tumours demonstrated a hypointense, isointense, or hyperintense signal according to the cystic content. In most cases, the cyst walls did not enhance after gadolinium administration. Histologically, the cysts were found to be lined by a single layer of epithelium. Histochemical and immunohistochemical studies showed that almost all were reactive to periodic acid schiff stain, epithelial membrane antigen, and carcino-embryonic antigen, but negative to glial fibrillary acidic protein. Mainly, the suboccipital approach with or without a laminectomy, or the trans-oral approach were selected for surgical excision of these tumours. In 17 of the 36 patients, total or gross total resections were performed, and subtotal resections were achieved in sixteen. Three patients developed recurrences.
The case of a 40-year-old woman with increasing ataxia is described. Although the clinical presentation and evoked response studies raised the possibility of multiple sclerosis, further investigation revealed multiple cystic intracranial lesions. Surgical excision of one of the lesions relieved the patient's symptoms. Histological examination revealed that this was an enterogenous cyst. Although single cysts of this type have rarely been reported occurring in the posterior cranial fossa, the occurrence of multiple lesions, some in the supratentorial compartment, appears to be unique.
A 23 year old woman with an intradural extramedullary enterogenous cyst in the cervical region, unassociated with vertebral cleft, presented with two episodes of neurological disturbance at an interval of five years. Subtotal excision of the anteriorly placed cyst at C6 vertebral level was followed by rapid and complete recovery.
A case of enterogenous cyst causing compression of the spinal cord at C-1 is presented. The clinical course and radiological and histological findings are discussed.
• A patient with an intraspinal enterogenous cyst is described. A review of the English literature disclosed 26 previous pediatric cases reported. These cases were studied in regard to clinical presentation, laboratory findings, histology of the lesion, and neurological follow-up. Although rare, enterogenous cysts should be considered where there is evidence of spinal cord compression.
(Am J Dis Child 132:906-908, 1978)
Nineteen epithelial cysts in the central nervous system including six colloid cysts of the third ventricle, seven Rathke's cleft cysts in the sella, two enterogenous cysts in the posterior fossa, two epithelial cysts in the spinal canal and two neuroectodermal cysts in the cerebrum were examined immunohistochemically for expression of intermediate filament proteins-simple type, stratified type and skin type cytokeratins and GFAP. Colloid cysts of the third ventricle, Rathke's cleft cysts in the sella and epithelial cysts in the spinal canal expressed complex type cytokeratins while enterogenous cysts and neuro-ectodermal cysts showed only simple type cytokeratins. In addition, Rathke's cleft cysts expressed GFAP in occasional lining cells. The characteristic composition and distribution of cytokeratins in various kinds of epithelial cysts in the central nervous system are demonstrated and discussed with regard to their origins.
The case of a large ventral cyst of the posterior fossa of probable ectodermal origin in a 33-year-old patient is presented. Intracranial epithelial cysts are rare, and their histogenesis is a subject of controversy. Previous reports have hypothesized either a neuroepithelial or endodermal origin for the majority of these cysts. This case is unusual because it is the first case, to our knowledge, where melanocytes and melanin have been demonstrated within the cyst wall. These findings suggest an ectodermal origin for this cyst.
A case of a benign epithelial cyst in the posterior cranial fossa is described. It had the unique histological feature of a double-layered cuboidal epithelial lining. Detailed immunohistochemical and electron microscopic studies supported an endodermal origin. The differential diagnosis and the histogenesis of epithelial cysts in the central nervous system are discussed.
: A case of an enterogenous cyst located in the cerebellopontine angle cistern is presented. These cysts have usually been found in the spinal canal, and their intracranial occurrence is exceptional. In the present case, the cyst was lined histologically by a single epithelial layer of cuboidal cells with some glandular structures resembling the fundic glands of the stomach. The unusual location of the cyst and its histological features are discussed. (Neurosurgery 28:462-466, 1991) Copyright (C) by the Congress of Neurological Surgeons
A case of a 29-year-old woman with a cystic lesion in the right cerebellopontine angle, who had three bouts of chemical meningitis in 1 year, is reported. The lesion proved to be a delicate, thin-walled cyst, in part with a squamous lining and in part with a respiratory epithelial (tall, columnar, ciliated, mucus-secreting) lining. Various possibilities to explain this unusual epithelium are explored.
A four-year-old child with an enterogenous cyst located lateral to the brainstem is reported. The endodermal origin of the cyst was histologically and immunohistochemically established. The unusual localization of the cyst as well as its destructive biological behavior as a result of inflammation is discussed.
Immunohistochemical characterization of 14 cases of intracranial cysts was performed. Among these 14 cases, five different types of cysts were represented; Rathke's cleft cyst (4 cases), neurenteric cyst (2 cases), colloid cyst (1 case), choroidal epithelial cyst (2 cases) and arachnoid cyst (5 cases). Immunohistochemical evaluation utilized antibodies to glial fibrillary acidic protein (GFAP), S-100 protein, prealbumin, carcinoembryonic antigen (CEA), and epithelial membrane antigen (EMA). GFAP-positive cells were detected in 1 Rathke's cleft cyst and in 1 choroidal epithelial cyst. S-100-positive cells were detected in 2 Rathke's cleft cysts, in 2 neurenteric cysts, in 1 colloid cyst, and in 2 choroidal epithelial cysts. Prealbumin-positive cells were detected only in the 2 choroidal epithelial cysts. CEA-positive cells were detected in 1 Rathke's cleft cyst and in 2 neurenteric cysts. EMA-positive cells were detected in all cases. Immunohistochemical study of prealbumin and S-100 protein is useful for correct diagnosis of choroidal epithelial cyst and study of CEA is useful for diagnosis of neurenteric cyst. The arachnoid cyst is negative for immunoreactivity to GFAP, S-100, prealbumin, and CEA; this can be helpful in distinguishing this type of cyst from single epithelial cysts, a task that is sometimes difficult with only light microscopy.
The clinical and radiological features of eight patients, five male and three female; with an enterogenous cyst of the central nervous system are reported. The cysts were all anteriorly placed, intradural or extramedullary in situation and their sites ranged from the brain stem to the conus, with a predilection for the lower cervical and upper thoracic spinal cord. Pain was a universal presenting symptom of the intraspinal cases with a varying degree of motor deficit. Three patients, two of whom also had congenital anomalies of the axial skeleton, showed localised expansion of the spinal canal. The site and size of all the spinal cysts were demonstrated by myelography. Computed tomography of the spine with intrathecal contrast medium was performed on two patients and shown to be of particular value in demonstrating recurrence. Three patients on long-term follow-up had a recurrence of the cyst at the original site. Our findings are discussed with reference to the previous literature.
Spinal intradural cysts Handbook of Clinical Neurology
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An epithelial cyst in the cerebello-pontine angle.
- Leung SY
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Posterior fossa epithelial cyst of ectodermal origin
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Respiratory epithelial cyst of the cerebellopontine angle
- Muller
Enterogenous cyst of the cerebellopontine angle cistern: Case report
- Umezu
Epithelial cysts in the central nervous system
- Uematsu