Spinal endodermal cyst undergoing malignant transformation and marked elevation of serum carbohydrate 19-9 level.

Endodermal cyst (EC) is a benign tumor that can arise along the craniospinal axis. Infrequently, ECs undergo malignant transformation. A 43-year-old man presented with numbness in the right arm, leg and occipitalgia. MRI revealed a multicystic, intradural extramedullary tumor at C2 with enhancement along the ventral surface of the spinal cord. Blood test showed an abnormal increase in serum carbohydrate antigen 19-9 (CA 19-9) level. Systemic positron emission tomography-computed tomography was normal. He underwent total tumor resection and was diagnosed with EC. He developed double vision, hearing loss, and swallowing difficulty on postoperative day 70. Cerebral MRI revealed marked extensions of leptomeningeal dissemination. The serum CA 19-9 level increased continuously and finally reached 1515.0 U/ml. He died of respiratory failure on day 108. An autopsy did not reveal abnormalities in the abdominal and chest organs. On microscopic examination, the post-mortem specimen revealed adenocarcinoma. Immunohistochemically, both the surgical and autopsy specimens were positive for CA 19-9. Spinal ECs may lead to malignant transformation with leptomeningeal dissemination that causes abnormal elevation of serum CA 19-9 levels.

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.

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.

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).

5-ALA fluorescence in intraparenchymal endodermal cysts.

In recent years, new indications have been suggested for 5-ALA, particularly for cystic lesions. We report the use of 5-ALA fluorescence in an intraparenchymal supratentorial endodermal cyst of a 52-year-old female presenting with headache, progressive right side hemiparesis and anomic aphasia. She underwent an image-guided 5-ALA-assisted left minicraniotomy for fenestration of the cystic lesion into the ventricular system. The capsule of the cyst was noted to fluoresce with 5-ALA. She recovered from the previous deficits and the cyst decreased in size. To the best of our knowledge, this is the first time 5-ALA fluorescence is reported in a case of endodermal cyst.

The diffusion-weighted imaging and 11-C-methionine positron emission tomography depiction of an endodermal cyst at the cervico-medullary junction.

A case of a 52-year-old male with left-sided neck pain, vertigo and gait instability is reported. A MRI scan revealed an intra-dural mass at the cervico-medullary junction, further characterised by diffusion-weighted imaging and 11-C-methionine positron emission tomography. Pathological diagnosis was endodermal cyst. The clinico-surgical relevance of the imaging findings is discussed.

A ventral brainstem neurenteric cyst - A case report and review of the pre-brainstem location.

Background: Neurenteric cysts are uncommon, benign endoderm-derived lesions that result from aberrant embryologic development of the notochord. They are typically located in the intradural extramedullary spinal cord and rarely located intracranially. Contrary to spinal-located cysts, intracranial cysts are rarer in the pediatric population. Clinically, they may present with symptoms of mass effect, or they can be incidentally discovered. Case Description: A 10-year-old healthy female child presented with recurrent headaches. The physical and neurological examination was unremarkable. Brain magnetic resonance imaging (MRI) showed a well-demarcated lesion anterior to the pontomedullary junction with striking T1 and T2/T2 fluid-attenuated inversion recovery high-signal intensity and a small rounded nodule within of low signal on T1, T2, and T2*. On initial conservative strategy with serial brain MRI, there was a progressive enlargement of the lesion with significant mass effect on the brainstem. The patient underwent a right retrosigmoid craniotomy, and the cyst wall was fenestrated and drained. Part of the cyst wall and the solid nodule were adherent to the brainstem and basilar artery and were not removed. The histologic findings were consistent with the diagnosis of a benign endodermal cyst. The postoperative period was uneventful. Conclusion: We report a successful surgical treatment of this rare congenital cyst located in the ventral brainstem. We present pre-and post-operative imaging findings, intraoperative microscopic images of the procedure, and a brief review of relevant clinical literature on the topic.

Neurenteric cyst of the dorsal craniocervical junction in an adult: A case report and operative video.

Background: Neurenteric cysts (NCs) are rarely located in the dorsal craniocervical junction (CCJ). Case Description: Here, we present a case and show the surgical video of a 24-year-old man with a history of neck pain, progressive hemiparesis, and difficulty swallowing. Radiological images revealed an intradural extramedullary lesion at the dorsal CCJ. Posterior approach with C1-C2 laminectomy for resection of the lesion was performed with significant improvement in symptoms postoperatively. Histopathological examination confirmed the diagnosis of NC. Conclusion: This case demonstrates a rare location of a NC in an adult patient. Complete excision of the cyst wall and its content is the recommended treatment option.

Supratentorial Neurenteric Cysts: Systematic Literature Review and Case Report.

BACKGROUND: Neurenteric cysts (NC) are uncommon congenital lesions with histopathologic properties derived from the gastrointestinal or respiratory tract. They are typically located in the intradural extramedullary compartment but rarely seen in the supratentorial region. The occurrence of supratentorial NCs (S-NC) presents an interesting quandary regarding their embryopathogenesis. METHODS: We present a case report and systematic literature review on S-NCs following PRISMA guidelines. RESULTS: A 57-year-old woman presented with a seizure and paresthesias of the face, hands, and feet. Magnetic resonance imaging showed a right temporo-occipital cystic lesion, which was managed with surgical resection. Histologically, the cyst was type A. The patient was without recurrence at 10 months. Including this case, 88 S-NCs have been reported in the literature. Common presenting symptoms are headaches and seizures. They were mostly treated with craniotomy, preferably with gross total resection, although subtotal resection may be necessary because of adhesions. Resection usually led to symptom improvement (61%). Malignant transformation was seen in 3%. Recurrence was seen in 17%, with a mean time to recurrence of 4.2 years, and was significantly more common after subtotal resection than gross total resection. CONCLUSIONS: If surgically resected, the cyst wall specimen should be sent for pathology review, because of the potential risk for malignancy. If conservatively managed, serial imaging is warranted to track for changes that may indicate transformation. The embryopathogenesis of these rare congenital lesions remains incompletely understood, but the most comprehensive theory involves enteric cell migration to the neuroectoderm during embryogenesis.

Pineal region neuroenteric cyst in a 13-year-old girl: A rare localization with postoperative recurrence and local dissemination.

Background: Neuroenteric cysts are rare cystic benign neoplasms of the central nervous system most often located in the spinal cord and rarely, intracranially. The nonspecific neuroimaging features make management planning potentially challenging. We present a case of a radiologically misdiagnosed neurenteric cyst with a complicated course. Case Description: A 13-year-old girl presented with a 6-month history of headache, tinnitus, and dizziness. Initial magnetic resonance images (MRIs) were indistinguishable from a pineal arachnoid cyst with aqueductal stenosis and hydrocephalus. Cyst fenestration was performed through an infratentorial supracerebellar approach. Histology revealed a neurenteric cyst. On day 10 postoperatively, she deteriorated with acute hydrocephalus and cyst enlargement. An external ventricular drain was inserted. She remained asymptomatic thereafter. At 1-year postoperative, the patient remains asymptomatic despite the MRI showing cyst enlargement and local dissemination in the form of multiple cystic lesions in the cerebellum along the operative corridor. The patient was managed conservatively considering adhesion noted intraoperatively. Conclusion: Neuroimaging features of brain cystic lesions may be nonspecific. Special attention should be awarded to posterior fossa and paramedian cystic lesions. Rarer lesions like neurenteric cysts should also be considered. When in doubt, we recommend using the following methods to prevent the free outflow of the cyst contents into the subarachnoid space: lining the cyst and operative corridor with cotton wool and puncture opening and suctioning of fluid. However, the "gold standard" remains surgical treatment with radical excision of the endodermal cyst capsule. It is necessary to preassess the possibility of total or subtotal resection.

Endodermal cyst: rare intraaxial brainstem location. Illustrative cases.

BACKGROUND: This report detailed an extremely rare location of an endodermal cyst. Such location of the tumor can pose radiological confusion and a therapeutic dilemma. However, when identified, it can be a pleasant surprise for the surgeon and provide the possibility of a symptom-free long life for the patient. OBSERVATIONS: This report discussed two young patients who presented with relatively short-duration reports of ataxia and diplopia. Investigations revealed intraaxial brainstem lesion. During surgery, thick, pus-like fluid was evacuated and part of a wall was resected. Histology revealed that the lesion was an endodermal cyst. Both patients are well and are lesion- and symptom-free for 24 and 72 months. LESSONS: Endodermal cysts are some of the few long-standing and benign intraaxial brainstem lesions.

Frontal Base Endodermal Cyst: A Case Report and Review of Literature.

BACKGROUND: Endodermal cyst (EC) is a rare congenital cyst of endodermal origin, but the pathogenesis of this entity remains uncertain. Supratentorial EC is particularly uncommon, but some cases have been reported. Here, we report a case of supratentorial EC that developed at the frontal base which indicates posttraumatic development rather than a congenital origin. CASE DESCRIPTION: A 65-year-old man who had a history of orbital bone fracture without rhinorrhea sustained in a traffic accident presented with gradually enlarging frontal-base cystic lesions. Multiple cystic lesions were removed via left frontal craniotomy. The cysts showed no communication with the frontal sinus. Histological examination identified EC. Postoperative course was uneventful and no recurrences have been identified as of 2 years later. CONCLUSIONS: According to reported cases, unlike ECs in other intracranial locations, frontal base ECs tend to present at advanced ages. The present case also presented with EC enlargement at an advanced age and two lesions located at the bone hiatus in the frontal base that were presumably caused by trauma. It is possible that sinus communication was repaired as the bone fracture was remodeled, and the remnant sinus epithelial tissues developed into ECs over time. This situation makes it reasonable to presume a posttraumatic rather than a congenital origin. In conclusion, as for frontal base ECs, contrary to the traditional theory, the developmental mechanisms may not necessarily be congenital.

Impact of the Extent of Surgical Resection on Patients With Intradural Extramedullary Bronchogenic Cysts: A Retrospective Institutional Experience and Review of the Literature.

Intradural extramedullary bronchogenic cysts (IEBC) are rare congenital cystic lesions. The clinical manifestations, radiological characteristics, especially the optimal treatment regimen are not well-understood. We retrospectively analyzed a series of patients with confirmed IEBC in Tangdu hospital and reviewed the published works to gain a comprehensive understanding of IEBC. In our institution, nine consecutive patients had pathologically confirmed IEBC between 2005 and 2018. We also identified 27 patients from previous studies. The most common presentations on magnetic resonance imaging (MRI) were hypointensity on T1-weighted images (T1WI), hyperintensity on T2-weighted images(T2WI), and no improvement on T1WI contrast-enhanced with gadolinium (94.4%). All patients in our center and the patients we reviewed received surgical resection; gross total resection (GTR) and partial resection (PR) were achieved in 20 (55.6%) and 16 (44.4%) patients, respectively. The symptom remission rate of patients who underwent GTR was 100%, which was similar to those who underwent PR (93.8%) (P = 0.457). The recurrence rate was 12.5% in the group who underwent PR and nil after GTR (P = 0.202). According to our current investigation, the surgical resection degree is irrelevant to the symptom remission rate. Therefore, we suggest that total resection should not be recommended for cases with tight adhesion. For patients with PR, longer follow-up will be necessary to determine the long-term outcome.

Saccular Limited Dorsal Myeloschisis with Spinal Cord Deviation out of the Spinal Canal to the Sac.

Saccular limited dorsal myeloschisis (LDM) is characterized by a fibroneural stalk linking the saccular skin lesion to the underlying spinal cord. Since untethering surgery during the early postnatal period is often indicated to prevent sac rupture, saccular LDM should be distinguished from myelomeningocele (MMC) during the perinatal period. We treated two patients with the spinal cord deviation from the spinal canal to the sac, which mimicked a prolapse of the neural placode into the MMC sac. In patient 1, pre- and postnatal magnetic resonance imaging (MRI) revealed that the spinal cord was strongly tethered to the thick stalk. During surgery, the dorsally bent cord and stalk were united, and the border between these two was determined with intraoperative neurophysiological mapping (IONM). In patient 2, the spinal cord was tethered to two slender stalks close to each other, which was visible with the combined use of sagittal and axial postnatal three-dimensional heavily T2-weighted imaging (3D-hT2WI). The preoperative MRI hallmark of saccular LDM is the visualization of a stalk that links the bending cord and sac. Complete untethering surgery to return the cord into the spinal canal and correct its dorsal bending is recommended.

Endodermal Cyst with a Non-enhancing Nodule in the Quadrigeminal Cistern Developed in an Octogenarian.

BACKGROUND: Intracranial endodermal cysts are congenital lesions that generally develop in the cerebellopontine angle and ventral brainstem of the posterior fossa, whereas endodermal cysts in the quadrigeminal cistern are very rare. We report a rare case of an endodermal cyst in the quadrigeminal cistern with a non-enhancing nodule that developed in patient over 80 years of age. CASE DESCRIPTION: An 85-year-old man presented to our hospital with progressing gait disturbance and urinary incontinence. Preoperative images showed a cystic mass lesion with a nodule in the quadrigeminal cistern and hydrocephalus. There was no enhanced portion in the lesion, and the intensity of the cyst on magnetic resonance imaging revealed a high protein concentration. Subtotal resection was performed due to the adhesion of the cyst to the brainstem. It was diagnosed as an endodermal cyst. The postoperative course was uneventful, and hydrocephalus improved. CONCLUSIONS: This is a rare case of an intracranial endodermal cyst in terms of location and age of onset compared with previous reports. This case demonstrates that endodermal cysts should be considered as a differential diagnosis for lesions in the quadrigeminal cistern with high protein concentration in the cyst and nodule representing chronic inflammation, regardless of enhancing effects.

Intracranial Neurenteric Cyst in a Patient with Acute Unilateral Thalamic Infarction.

Intracranial neurenteric cysts are rare congenital lesions that can be mistaken for other lesions. They may contain mucous or serous glands, smooth muscle, lymphoid tissue and components of connective tissue. We report a case of neurenteric cyst which was detected accidentally by diagnostic imaging in the context of a thalamus infarct in a 45-year-old woman. We also discuss the results of a brief literature review and the pathological findings, imaging spectrum, evaluation and management of neurenteric cysts and thalamic infarctions. LEARNING POINTS: Neurenteric cysts are a rare type of foregut duplication cyst that are classified as intracranial or spinal and are associated with central nervous system abnormalities.Neurenteric cysts are usually benign and slow-growing, so conservative treatment can be provided if there are no symptoms.Thalamic stroke may be caused by arterial or venous infarction.Cardioembolism is the most common cause of thalamus infarction; variations in blood supply to the thalamus should be investigated in affected patients.

Intracranial neurenteric cyst: A case report and differential diagnosis of intracranial cystic lesions.

Neurenteric cysts are uncommon, benign lesions that are rarely located intracranially. These cysts are likely due to aberrant embryologic development of the notochord. Clinically, neurenteric cysts may present with symptoms of mass effect, or they can be asymptomatic and incidentally discovered. Imaging features of neurenteric cysts have significant overlap with other intracranial cystic lesions, which can make diagnosis difficult. We present a case of a 35-year-old female with a histopathologically confirmed neurenteric cyst in the premedullary and left cerebellomedullary cistern, with associated symptoms of headache, dizziness, tinnitus, and dysphagia. The patient underwent surgical resection, with improvement in symptoms. We present a review of literature, and a discussion of typical features of multiple intracranial cystic lesions. We hope to promote accurate preoperative diagnosis, to allow for appropriate surgical technique to reduce the risk of recurrence.

Endodermal Cyst Arising from the Oculomotor Nerve: A Case Report Outlining the Novel Insertion of Cysto-subarachnoid Shunt for Refractory Disease.

BACKGROUND: Endodermal cysts of the oculomotor nerve are rare presentations. Only case reports are available to help guide clinicians with managing this rare entity. CASE DESCRIPTION: A 3-year-old boy presented with an acute on chronic left oculomotor nerve palsy due to a left interpeduncular cistern cyst found on magnetic resonance imaging. He underwent a left pterional craniotomy and fenestration of the histologically proven endodermal cyst and had initial improvement at the 2-month review. He subsequently developed clinical and radiologic evidence of recurrence and was treated surgically with a refenestration and insertion of a cysto-subarachnoid shunt through a trans-sylvian approach. At 6-month follow-up, there was complete resolution of the oculomotor nerve palsy with interval development of oculomotor synkinesis. CONCLUSIONS: Magnetic resonance imaging is an essential modality in the follow-up of these patients postoperatively in the setting of unchanged or deteriorated neurology. Fenestration of the cyst is appropriate first-line surgical management; however, a cysto-subarachnoid shunt is a safe consideration in recurrent, symptomatic cysts and provides sustained symptom resolution.

Endodermal Cysts of the Central Nervous System: Review of the Literature and a Case Report.

CONTEXT: Endodermal cysts are rare benign developmental cysts lined by mucin-secreting and/or ciliated, cuboidal, or columnar epithelium of probably endodermal origin. AIMS: Endodermal cysts are rarely intracranial, frequently located in the posterior fossa. Supratentorial location is the most infrequent and only few cases are reported in the literature, included our case. SETTINGS AND DESIGN: The authors report a case of intracranial supratentorial endodermal cyst with a review of the literature. SUBJECTS AND METHODS: A 40-year-old woman was admitted to our department because of progressive gait disorder for 3 months due to right brachial and crural motor deficit associated to right crural sensory disorder (tactile hypesthesia) and right Babinski response at neurological examination due to an endodermal cyst located in the left frontoparietal convexity. DISCUSSION: Total resection of endodermal cysts is recommended, despite their location and adhesion to the surrounding structures, due to its high risk of recurrence. Fenestration of the cystic content into the subarachnoid cistern may cause obstructive hydrocephalus or chemical meningism. RESULTS: Although rare, surgeons should be aware that these lesions must be differentiated clinically, radiologically, and histologically from other supratentorial cystic lesions.