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1/22. Enterogenous cyst of the posterior fossa.

    We report the case of a 13-year-old boy who presented with deafness due to a posterior fossa cystic lesion which was surgically excised. Histological examination showed it to be an enterogenous cyst. These extremely rare lesions seldom occur within the neural axis.
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2/22. Extradural endodermal cyst of posterior fossa: case report, review of the literature, and embryogenesis.

    OBJECTIVE AND IMPORTANCE: Posterior fossa endodermal cysts are rare. They are located in the midline, in ventral or ventrolateral locations, or intrinsic to the neural axis. Accordingly, various theories of embryogenesis have been proposed. We report the first case of an extradural, dorsolaterally situated endodermal cyst. CLINICAL PRESENTATION: An adult male patient presented with a short history of headache and cerebellar ataxia. neuroimaging revealed an extra-axial cystic posterior fossa mass. INTERVENTION: An entirely extradural cyst was found and was totally excised. immunohistochemistry confirmed the diagnosis of endodermal cyst. CONCLUSION: The extradural, dorsal location of the endodermal cyst suggests gaps at the cranial end of the notochord causing ectodermal-endodermal adhesions during early gastrulation and the persistence of endodermal remnants in the dorsal mesenchyme of the blastemal cranium. The literature is reviewed, and proposed theories of embryogenesis are discussed.
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3/22. galactorrhea as the sole presenting symptom of a posterior third ventricular epidermoid cyst.

    BACKGROUND: brain tumors that are remote from the sellar and parasellar region rarely produce galactorrhea. CASE DESCRIPTION: galactorrhea was the sole presenting symptom in a patient with a posterior third ventricular epidermoid cyst. On investigation, her serum prolactin level was mildly elevated. After radical excision of the tumour, the spontaneous galactorrhea stopped and the serum prolactin level decreased. CONCLUSION: Because of the clinical and biochemical response seen after surgery, we speculate that the galactorrhea was caused by the presence of the tumor. We postulate that the pressure exerted by the tumor on the diencephalic structures was probably the cause of galactorrhea in our patient.
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4/22. siblings with development delay, mild spasticity and subcortical cysts: a further leukoencephalopathy?

    We present a family with mild neurological symptoms and intra-cerebral subcortical cysts on magnetic resonance imaging (MRI). Common clinical features are microcephaly, learning difficulties, spasticity, dyspraxia and restricted movements of the neck and shoulder. The family has features in common with vacuolating leukoencephalopathy of van de Knaap and Olivier and may represent a new variant.
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keywords = leukoencephalopathy
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5/22. Non-progressive leukoencephalopathy with bilateral temporal cysts.

    We report two cases of a peculiar leukoencephalopathy with temporal cysts. Both patients have a non-progressive neurological disorder with mental retardation, microcephaly and sensorineural deafness although clinical differences between them may reflect a different aetiology. The metabolic disorders with white matter involvement and the recently described leukoencephalopathies (Van Der Knaap disease, 'vanishing white matter disease') were excluded based on clinical, biologic and imaging findings. cytomegalovirus infection is a likely possibility in the first case although the magnetic resonance imaging picture is only partially similar to previously reported cases. Our patients are strikingly similar to the patients reported by Deonna et al. and Olivier et al. We discuss the clinical and imaging findings in our patients and the differential diagnosis considering the known disorders of the white matter in childhood.
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keywords = leukoencephalopathy
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6/22. 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.
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7/22. Intra-sellar salivary gland-like pleomorphic adenoma arising within the wall of a Rathke's cleft cyst.

    Salivary gland rests occur in the posterior lobe of the pituitary gland near or often communicating with the Rathke's cleft or its cystic subdivisions, and are usually incidental autopsy findings. They are attributed to the oropharyngeal development of the Rathke's pouch and may rarely give rise to salivary gland-like tumors in the sella. We present a pleomorphic adenoma, a rare tumor of the sellar region, that has not been previously recognized in association with Rathke's cleft cyst. It occurred in a 44-year-old man who presented with hypopituitarism and reduced vision. magnetic resonance imaging showed a sellar mass with suprasellar extension which was totally removed. It consisted of segments of a cyst wall lined by focally ciliated columnar of cuboid epithelium containing goblet cells. An eosinophilic granular material with cholesterol clefts represented the contents of the cyst. Within its wall there was a tumor with ductal structures and non-ductal varied cellular components including hypercellular areas of spindle and ovoid cells forming interlacing fascicles. Individual cells appeared to float in abundant mucinous material. The appearances were those of a salivary gland pleomorphic adenoma arising within the wall of a Rathke's cleft cyst. The myoepithelial nature of non-ductal tumor cells was confirmed with immunocytochemistry. The existence of seromucous glands communicating with the Rathke's cleft remnants, explains the concomitant occurrence of the tumor and the cyst. This rare neoplasm from salivary gland rest should be considered in the differential diagnosis of unusual sellar and suprasellar tumors.
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8/22. Infratentorial supracerebellar approach to the colloid cysts of the third ventricle.

    OBJECTIVE: The transcallosal and the frontal transcortical approaches are the most widely used methods in surgery of third ventricle colloid cysts. However, these approaches require traction of the frontal lobe and dissection of the corpus callosum or corticotomy and involve some postoperative consequences. The rationale of the proposed method is to remove the colloid cyst by the infratentorial supracerebellar approach and the posterior wall of the third ventricle without dissection of any neural structures. methods: Five patients with a colloid cyst of the third ventricle were operated on by the proposed method. The first patient presented with several months' history of symptoms that included increased intracranial pressure and right-sided cerebellar signs, caused by a metastatic tumor of the right cerebellar hemisphere. The other four patients had symptoms including intracranial hypertension for an extended period of time without any other neurological deficits. In all patients, magnetic resonance imaging revealed a colloid cyst of the third ventricle without hydrocephalus. TECHNIQUE: With the infratentorial supracerebellar approach, the arachnoid of the quadrigeminal cistern is dissected. The pineal body is separated and displaced from the internal vein medially, and the posterior velum interpositum is opened. Perforation of the inferior layer of the tela choroidea just above the suprapineal recess allows opening of the third ventricle cavity. A foraminal region is exposed after a slight lateral displacement of medial surfaces of the thalamus along the third ventricle roof. CONCLUSION: The proposed approach through the infratentorial supracerebellar space and the posterior wall of the third ventricle may be used for removal of colloid cysts, especially in patients in whom the lateral ventricles are not enlarged.
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9/22. 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.
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ranking = 0.2
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10/22. Pathogenesis and diagnostic pitfalls of ventricular diverticula: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: Ventricular diverticula are local herniations of the ventricular system occurring in association with severe, long-standing hydrocephalus. Variability in the extent and location of these ventricular pouches may produce misleading radiological conformations and raise diagnostic challenges. In this report, we discuss the pathogenesis and differential diagnosis of ventricular diverticula on the basis of a case illustration. CLINICAL PRESENTATION: We report the case of a 38-year-old woman presenting with hydrocephalus secondary to an obstructive lesion of the third ventricle. A large, dilated structure compressing the brainstem was present in the posterior fossa. INTERVENTION: The posterior fossa lesion was identified as an atrial diverticulum by injection of contrast medium into the right lateral ventricle, which led to immediate enhancement of the infratentorial cavity. Ventricular drainage and removal of the third ventricular lesion, a colloid cyst, allowed progressive collapse of the diverticulum and symptom resolution. CONCLUSION: This report discusses the pathogenesis of ventricular diverticula and demonstrates that ventriculography can be used to differentiate atrial diverticula from other posterior fossa lesions when time-consuming multiplanar magnetic resonance imaging cannot be afforded on an emergency basis.
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ranking = 0.6
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