Neurenteric cyst of the anterior cranial fossa: case report and literature review

Intracranial neurenteric cysts are rare congenital lesions that typically occur in the posterior fossa. We report a case of a 70-year-old gentleman presenting with gait disturbance, found to have a neurenteric cyst primarily arising from and expanding the sella turcica. A review of the literature revealed 27 reports of supratentorial neurenteric cysts. Clinical presentation, radiological characteristics, treatment, prognosis and embryological origin are discussed. Intracranial neurenteric cysts should be included in the differential with any well-demarcated cystic lesion without enhancement on magnetic resonance imaging (MRI). Complete surgical excision is the treatment of choice, with good prognosis.     

Intramedullary neurenteric cyst without an associated malformation. Case report

The authors report a case of an intramedullary neurenteric cyst without any associated dysraphic lesion. Unlike extramedullary intradural forms, this type of malformation remains rare. Our observation is one of the first to have been evaluated by magnetic resonance imaging. Theories concerning the embryogenesis of neurenteric cysts as well as their clinical characteristics and surgical treatment are discussed.     

Dermoid cyst: A rare intramedullary inclusion cyst

Intramedullary dermoid cysts are rare tumors, especially those not associated with spinal dysraphism. Only six cases have been reported in the literature. Of these, only two cases have had magnetic resonance imaging studies. We report a case of an 18-year-old female patient, who presented with progressive weakness of both the lower limbs and wasting of both the upper limbs. Magnetic resonance imaging (MRI) showed an intramedullary lesion extending from C3 to D2 with peripheral enhancement on contrast. Decompression of the cystic contents with partial removal of cyst wall was done. Hair with oily cholesterol and keratin debris was encountered. Histopathology confirmed the diagnosis of dermoid cyst. This case adds to the previous reported cases of the rare and uncommon intramedullary space occupying lesions of the spinal cord.     

A recurrent intradural cervical neurenteric cyst operated on using an anterior approach: a case report

The neurenteric cyst is an uncommon congenital lesion. In most reported cases, it has been operated on via a posterior approach using a laminectomy, despite the fact that the cyst is usually located ventral to the spinal cord. Reports have shown that early postoperative results have been good with the posterior approach, but very few studies of the long-term postoperative recurrence of neurenteric cysts have been conducted. Here, we report on a case of recurrent neurenteric cyst that was operated on using an anterior approach.A 42-year-old woman presented with a cervical neurenteric cyst that had recurred eight years after its partial removal via a posterior approach. The patient complained of pain on the lateral side of her upper arms, and an magnetic resonance imaging showed that the recurrent cyst was located ventral to the spinal cord and compressed the cord dorsally at the C4-6 level.The patient was operated on via an anterior approach using a vertebrotomy at the lower half of C5 and the upper half of C6. The cyst was attached to the spinal cord firmly and was subtotally removed, with the thickest portion adhering to the cord not being removed. The caudal end of the cyst was observed with the assistance of a rigid endoscope.A neurenteric cyst may recur after partial removal, and the patient's condition may deteriorate during postoperative follow-up. The anterior surgical approach provides good visualization and facilitates safe removal of the lesion.     

Neurenteric cyst: surgery and diagnostic imaging

Neurenteric cysts are rare, with fewer than 30 cases noted in the literature. We report the case of a newborn infant with respiratory distress caused by a large neurenteric cyst that was identified by prenatal ultrasound. Treatment consisted of excision of the mass through a right posterolateral thoracotomy. The cyst adhered to the spine at the level of the first thoracic vertebra and communicated with the jejunum through a posterior diaphragmatic defect. Postoperative studies with magnetic resonance imaging (MRI) and computed tomography (CT) disclosed an anterior meningocele and tethering of the spinal column. This is the second reported case of a neurenteric cyst demonstrated by prenatal ultrasound. The presence of an intrathoracic cyst associated with spinal abnormalities is characteristic of this anomaly. With imaging techniques such as MRI and CT, we may detect residual intraspinal disease associated with neurenteric cysts.     

Intramedullary epidermoid cysts of the spinal cord. Case report.

Epidermoid cysts are tumors familiar to neurosurgeons, but intramedullary epidermoid cysts are rare. The authors report the case of a 6-year-old girl presenting with progressive paraparesis. A midthoracic intramedullary mass was revealed on myelography and magnetic resonance (MR) imaging and confirmed as an intramedullary epidermoid cyst at surgery, at which time the cyst was removed. This is the fourth report documenting a purely intramedullary epidermoid cyst occurring in a child. The pathology and etiology, epidemiology, clinical features, radiology (including MR image characteristics), and surgical treatment of such rare intramedullary benign tumors are discussed. Magnetic resonance imaging reduces the delay in diagnosis of spinal cord tumors but should be guided by clinical judgment.     

Lateral supratentorial endodermal cyst: case report and review of literature

OBJECTIVE AND IMPORTANCE: 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. CLINICAL PRESENTATION: 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. INTERVENTION: 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. CONCLUSION: 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.     

Contrast magnetic resonance imaging diagnosis of periaortic bronchogenic cyst

A 39-year-old woman who had suffered from intermittent left back pain for 7 months was diagnosed as having a tumor in the posterior mediastinum to the left of the seventh thoracic vertebra. It was attached to the descending aorta and to a rib. Preoperative contrast magnetic resonance imaging strongly suggested that the tumor was a cystic lesion. We resected the tumor, and it was revealed to be a bronchogenic cyst. After 7 months' follow-up, the patient is asymptomatic and without complications. Bronchogenic cysts are rare developmental abnormalities of the primitive foregut and seldom arise in the posterior mediastinum, especially in a periaortic position. The preoperative diagnosis is difficult because of the diverse nature of a cyst's contents. We were able to predict preoperatively the nature of the tumor and its relation to other structures. Contrast magnetic resonance imaging is helpful in elucidating these cysts.     

Spinal neurenteric cyst in association with syringomyelia: case report.

A case of spinal neurenteric cyst in association with Klippel-Feil deformity, spinal lipoma, and syringomyelia is presented. Neuroradiological evaluation was performed by means of magnetic resonance imaging of the spine and a computed tomography myelograph. The diagnosis of neurenteric cyst was established by histological and immunohistochemical studies. The pathogenesis of syringomyelia associated with an extramedullary mass lesion is discussed.     

Huge intramedullar neurenteric cyst with intrathoracic development in a 1 month-old boy: excision though the anterior approach. A case report and review of the literature

Spinal neurenteric cyst is a rare congenital lesion that may occur either alone or in the context of a complex malformative disorder. Our case is unusual because of its rare intramedullary location, the association with an important intrathoracic development, and the age of the child at presentation (1 month). An anterior approach through a right-sided lateral thoracotomy was performed for a total resection of the intrathoracic part and a subtotal resection for the intramedullar portion. During 2 years of follow-up, the child presented no neurological deficit and post-operative magnetic resonance imaging found a small residue fixed on the anterior spinal cord without progression.     

Adjacent epidermoid cyst and primary central nervous system lymphoma: case report

BACKGROUND: Except for cases with radiotherapy or phacomatosis, multiple primary brain tumors of different histologic types are rare. The authors report the association of an epidermoid cyst and a primary CNS lymphoma. CASE DESCRIPTION: A 66-year-old man with a 20-year history of gait disturbance was admitted because of recently worsening symptoms. Magnetic resonance imaging showed an abnormal mass in the posterior fossa. The mass lesion had the appearance of a cystic tumor with a large mural nodule. Gross total removal of the tumor was performed. Histologic examination disclosed the cystic portion to be an epidermoid, whereas the nodular portion exhibited the histologic and immunohistochemical features of a malignant lymphoma. The lymphoma cells were shown to harbor EBV by in situ hybridization. CONCLUSIONS: To our knowledge, this is the first report of the coexistence of an epidermoid cyst and primary CNS lymphoma. When the lesions are adjacent, a definite preoperative diagnosis can be difficult. Epstein-Barr virus-associated CNS lymphoma is rare among the immunologically normal population. The possible pathogenesis for the coexistence of these 2 tumors is discussed.     

Advances in diagnosis and treatment of spinal hemangioblastomas

Spinal hemangioblastoma usually leads to large intramedullary cysts. Until recently clinical and conventional radiological examination was often not able to detect single or multiple tumor niduses at the wall of a secondary syringomyelia cyst. Thus during surgical exploration the tumor not infrequently was missed, leading to misinterpretation of the pathological entity as syringomyelia, spinal gliosis or low grade spinal astrocytoma. This often resulted in explorative biopsy or decompressive laminectomy. Clinical deterioration postoperatively was not uncommon due to the remaining tumor and increasing spinal cord enlargement. Now magnetic resonance imaging allows the exact preoperative localization of the medullary lesion apart from the accompanying cysts and facilitates differential diagnosis of primary syringomyelia or other spinal cord neoplasms. Microsurgical techniques and laser-assisted resection have improved the outcome. We report on eight patients with spinal hemangioblastoma treated in our clinic since the installation of magnetic resonance imaging and laser-assisted microneurosurgery.     

Prenatal diagnosis of mediastinal neurentric cyst with an intraspinal component

Mediastinal neurenteric cysts are the least common types of the bronchopulmonary foregut malformations, and their antenatal diagnosis is rare. We report a case of mediastinal neurenteric cyst diagnosed on antenatal ultrasonography at 28 weeks' gestation. A small intraspinal component and vertebral segmentation anomalies were also noted. The diagnosis was confirmed on postnatal magnetic resonance imaging and at the time of operation.     

Spinal hydatid cyst mimicking arachnoid cyst on magnetic resonance imaging

BACKGROUND: Primary spinal hydatid cysts are uncommon and account for 1% of all cases of hydatid disease. Echinococcus granulosus is most often responsible for the cyst hydatid. Intradural, extramedullary involvement is rare. When the cysts do not demonstrate typical magnetic resonance imaging findings, the differential diagnosis is more complex. METHOD: Case report. FINDINGS: An isolated primary hydatid cyst of the spine in a 35-year-old man that appeared to be an arachnoid cyst on preoperative radiographic examination. CONCLUSION: Hydatid cysts that lack the typical radiographic appearance may be mistaken for arachnoid cysts. Misdiagnosis has serious implications for surgical intervention and long-term care.     

Unique Imaging Features of Spinal Neurenteric Cyst

A 50-year-old male presented with acutely progressed paraplegia. His magnetic resonance imaging demonstrated two well-demarcated components with opposite signals in one cystic lesion between the T1- and T2-weighted images at the T1 spine level. The patient showed immediately improved neurological symptoms after surgical intervention and the histopathological exam was compatible with a neurenteric cyst. On operation, two different viscous drainages from the cyst were confirmed. A unique similarity of image findings was found from a review of the pertinent literature. The common findings of spinal neurenteric cyst include an isointense or mildly hyperintense signal relative to cerebrospinal fluid for both T1- and T2-weighted images. However, albeit rarer, the signals of some part of the cyst could change into brightly hyperintensity on T1-weighted images and hypointensity on T2-weighted images due to the differing sedimentation of the more viscous contents in the cyst.     

Microsurgical Excision of the Craniocervical Neurenteric Cysts by the Far-Lateral Transcondylar Approach: Case Report and Review of the Literature

Neurenteric cysts in the anterior craniocervical junction (CCJ) region can be found in extremely rare cases. We report one case with craniocervical neurenteric cyst that was excised by the far-lateral transcondylar (FLT) approach. A 43-year-old man presented with a history of recurrent episodes of mild neck pain and dysesthesia in his bilateral hands of 2 years'' duration with rapid deterioration 3 weeks prior to admission. Magnetic resonance imaging (MRI) of the CCJ region revealed a well-defined intradural cystic lesion located ventral from the pontomedullary junction to C1 vertebra with medulla and C1 cord compression. This patient underwent total excision of the lesion via the FLT approach without any postoperative neurological deficits, and the histopathologic diagnosis was neurenteric cyst. Follow-up MRI has revealed no evidence of recurrence. The clinical features, imaging studies, and surgical approach options involved in resecting craniocervical neurenteric cysts are discussed, along with a review of the literature.     

Prenatal diagnosis of a large, cervical, intraspinal, neurenteric cyst and postnatal outcome

Neurenteric cysts are rare congenital anomalies derived from the notochord and located in front of the spinal column, mostly at the cervical level. They consist of an intraspinal cystic component that is connected to a mediastinal or thoracic cyst. A case of a cervical, intraspinal, neurenteric cyst, diagnosed at 28 weeks of gestation and operated on at 3 and half months after birth, is reported. Herein, we discuss important features of this case including its rare occurrence, intrauterine diagnosis, and early surgical intervention. In conclusion, the nature of prenatal intradural cysts should be fully evaluated and differentiated between neurenteric cyst and other types of cysts with fetal ultrasonography. Therefore, it is essential to be aware of this potential cyst in fetal ultrasonography in utero. Neurenteric cyst should be considered in the differential diagnosis of cervical intradural cystic lesions. In infants, successful surgery provides a cure in patients with cervical neurenteric cysts.     

Neurenteric cyst of the posterior fossa. Case report and review of the literature

Intracranial neurenteric cysts are rare entities. The term is currently used to describe epithelial cysts that are lined with a presumed endodermal-derived epithelium and are mostly located in the posterior fossa.Preoperative diagnosis is often difficult because of their clinical presentation, which may resemble a subarachnoid hemorrhage, and the radiological aspect, which can mimic vascular pathologies.We describe a posterior fossa neurenteric cyst in a 27-year-old woman, who presented with sudden headache as the only symptom and who was addressed to our hospital for subarachnoid hemorrhage. Diagnostic angiography was negative and MRI revealed a prepontine cystic lesion. The patient underwent a posterolateral approach on the right side, with subtotal resection of the lesion.We discuss the embryologic, diagnostic and therapeutic aspects of these cysts and review the literature.     

Non-neoplastic cystic and cystic-like lesions of the pancreas: may mimic pancreatic cystic neoplasms

BACKGROUND: Cystic lesions of the pancreas consist of a broad range of pathological entities. With the exception of the pancreatic pseudocyst, these are usually caused by pancreatic cystic neoplasms. Non-neoplastic pancreatic cystic and cystic-like lesions are extremely rare. In the present article, the surgical experience with these unusual entities over a 14-year period is reported. METHODS: Between 1991 and 2004, all patients who underwent surgical exploration for a cystic lesion of the pancreas were retrospectively reviewed. Patients with a pancreatic pseudocyst were excluded. There were 106 patients of whom 8 (7.5%) had a final pathological diagnosis consistent with a non-neoplastic pancreatic cystic or cystic-like lesion, including 3 patients with a benign epithelial cyst, 2 with a pancreatic abscess (one tuberculous and one foreign body), 2 with mucous retention cysts and 1 with a mucinous non-neoplastic cyst. These eight patients are the focus of this study. RESULTS: There were six female and two male patients with a median age of 61.5 years (range, 41-71 years). All the patients were of Asian origin including seven Chinese and one Indian. Four of the patients were asymptomatic and their pancreatic cysts were discovered incidentally on radiological imaging for other indications. All the patients underwent preoperative radiological investigations, including ultrasonography, computed tomography or magnetic resonance imaging, which showed a cystic lesion of the pancreas. Three patients, all of whom were symptomatic, were diagnosed preoperatively with a malignant cystic neoplasm on the basis of radiological imaging. Two patients were eventually found to have a pancreatic abscess, one tuberculous and the other, secondary to foreign body perforation. The third patient was found on final histology to have chronic pancreatitis with retention cysts. The remaining five patients had a preoperative diagnosis of an indeterminate cyst; on pathological examination, they were found to have a benign epithelial (congenital) cyst (n = 3), retention cyst (n = 1) and mucinous non-neoplastic cyst (n = 1). At a median follow up of 20 months (range, 3-34 months), none of the patients had any evidence of recurrent disease. CONCLUSION: Non-neoplastic cystic and cystic-like lesions of the pancreas are rare causes of pancreatic cystic lesions that are generally benign and do not require surgery when asymptomatic. However, despite advances in diagnostic investigations such as endoscopic ultrasound with fluid aspirate and magnetic resonance imaging, the preoperative diagnosis remains unreliable. Hence, the challenge for all clinicians is to recognize these lesions preoperatively and to avoid 'unnecessary' surgery.     

Endodermal cyst of the quadrigeminal cistern: case report.

BACKGROUND: Endodermal cysts usually develop in the subdural space in the anterior spinal cord and rarely occur inside the cranium. Most intracranial endodermal cysts develop in the posterior fossa. We report the first case of an endodermal cyst in the quadrigeminal cistern. CASE DESCRIPTION: The patient was a 71-year-old man who suffered from gait disturbance for 6 months. Although head computed tomography (CT) scanning 4 years previously did not show any cystic lesion, CT and magnetic resonance imaging (MRI) on admission showed a cystic lesion extending from the quadrigeminal cistern to the right ambient cistern. The cyst was subtotally removed via a suboccipital transtentorial approach. The cyst wall consisted of a layer of columnar epithelium and connective tissue. Based on the results of immunostaining, it was diagnosed as an endodermal cyst. CONCLUSIONS: It is possible that the increase of secretion from the cells lining the cyst may have caused a difference in osmotic pressure between the cerebrospinal fluid and the cyst contents, leading to rapid enlargement of the cyst. An endodermal cyst should be removed as completely as possible because its cells have the ability to grow and produce secretions.