Abstract:A 53-year-old male presented with a rare enterogenous cyst in the fourth ventricle associated with repeated ventriculoperitoneal shunt malfunction. Surgical excision of the cyst resolved the shunt problems. Electron microscopy findings of the surgical sample were microvilli covered with electron dense coating materials, basement membrane, and several intercellular junctional devices , suggest ing the cyst was derived from the endodermal structure. The viscous and gelatinous contents of the cyst might be respon… Show more
“…Los quistes endodérmicos intracraneales constituyen una entidad patológica muy poco frecuente que ha sido descrita preferentemente en la fosa posterior 7,9,[11][12][13]17,19,[22][23][24][25][26]28 , siendo excepcionales los casos descritos a nivel supratentorial.…”
Endodermal cysts (EC) of the central nervous system are very uncommon lesions predominantly located in the spinal canal. Although rare, intracranial EC have been mainly described in the posterior fossa, with the supratentorial location considered exceptional. Apart from the low frequency of these lesions, their pathoembriology still remais unknown. We report a patient with a huge frontal EC and review the literature. A 62-year-old man presented with abnormal behaviour, disorientation and decreased level of consciousness after moderate head injury. Initial cranial CT scan revealed a large cyst in the left frontal region with marked midline shift. Emergency puncture and decompression of the cyst demonstrated a milky fluid with high protein levels. Cranial MRI after patient improvement confirmed the existence of the cystic lesion with less mass effect. Delayed surgery was performed with craniotomy and total removal of the cyst. Pathological examination confirmed the presence of a typical EC. Patient made a complete recovery on follow-up with no recurrence on postoperative MRIs. Differential diagnosis of EC based on radiological data is quite difficult. As aggresive behaviour of this condition has been described following incomplete resections, the treatment of choice is a radical removal of the cyst in one or two stages depending on patient clinical condition.
“…Los quistes endodérmicos intracraneales constituyen una entidad patológica muy poco frecuente que ha sido descrita preferentemente en la fosa posterior 7,9,[11][12][13]17,19,[22][23][24][25][26]28 , siendo excepcionales los casos descritos a nivel supratentorial.…”
Endodermal cysts (EC) of the central nervous system are very uncommon lesions predominantly located in the spinal canal. Although rare, intracranial EC have been mainly described in the posterior fossa, with the supratentorial location considered exceptional. Apart from the low frequency of these lesions, their pathoembriology still remais unknown. We report a patient with a huge frontal EC and review the literature. A 62-year-old man presented with abnormal behaviour, disorientation and decreased level of consciousness after moderate head injury. Initial cranial CT scan revealed a large cyst in the left frontal region with marked midline shift. Emergency puncture and decompression of the cyst demonstrated a milky fluid with high protein levels. Cranial MRI after patient improvement confirmed the existence of the cystic lesion with less mass effect. Delayed surgery was performed with craniotomy and total removal of the cyst. Pathological examination confirmed the presence of a typical EC. Patient made a complete recovery on follow-up with no recurrence on postoperative MRIs. Differential diagnosis of EC based on radiological data is quite difficult. As aggresive behaviour of this condition has been described following incomplete resections, the treatment of choice is a radical removal of the cyst in one or two stages depending on patient clinical condition.
“…They are rare at the craniovertebral junction, with only 28 reported cases [1,22,8,[37][38][39][40][41][42][43][44][46][47][48][50][51][52][53][54][55][56][57]. Supratentorial NCs are rare, and 25% of the cysts in those cases reported to date have been located within the brain parenchyma [2,28,[58][59][60][61][62][63][64][65][66][67][68][69][70][71][72][73].…”
Intracranial neurenteric cysts are uncommon and occur usually in the posterior fossa. We report one case of a neurenteric cyst that was situated in the 4th ventricle. Total surgical removal was performed. One hundred cases having been published in the English language literature. We report on the imaging features of the tumor on several modalities as well as its histopathology. We further review the literature regarding this rare benign tumor entity.
“…From 1952 to 2011, 140 cases of histologically confirmed intracranial NE cysts have been reported: among the 140 intracranial NE cysts, two most frequently found locations were the posterior fossa (47 cases), especially cerebellopontine angle and the craniocervical junctional area (61 cases), while only two of them were found in the fourth ventricle [1312]. Nevertheless, two among 140 is not a portion that is small enough to be readily dismissed.…”
Section: Discussionmentioning
confidence: 99%
“…Neurenteric cyst (NE cyst) is an intracranial benign cystic lesion composed of epithelial lining of endodermal origin [1]. Though its exact etiology is still uncertain, it is widely believed to originate from incomplete dissolution of the neurenteric canal in embryonic stage.…”
This report presents a case of fourth ventricle neurenteric cyst (NE cyst) mimicking hemangioblastoma, which developed in a 50-year-old woman. A tiny enhancing mural portion of the fourth ventricle in MRI suggested that the cyst was hemangioblastoma, but pathological evidence showed that the cyst was in fact NE cyst in the fourth ventricle. In order to make proper decision on to what extent of surgical resection should be done, considering every possibility in differential diagnosis might be helpful. This case reports an unusual pathology in 4th ventricle, considering the patient's age, and demonstrates that a rarer disease may share radiological features of a common disease.
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