Intraparenchymal Supratentorial Neurenteric Cyst

Abstract

412Neurenteric cysts are congenital abnormalities usually found within the posterior mediastinum, 1 but can occur in the central nervous system (CNS). They are rare congenital cysts with histological features that indicate an endodermal origin. 2 The cysts are lined by single or multi-layered cuboidal or columnar epithelium. The epithelium may or may not be ciliated and goblet cells are occasionally seen. The epithelium resembles that which lines the respiratory or gastrointestinal tract. 2,3 Central nervous system neurenteric cysts are most commonly found ventral to the spinal cord in the intradural extramedullary space at the level of the cervical or upper thoracic spine. 4 Lumbosacral neurenteric cysts have also been reported. [5][6][7] The majority of intracranial neurenteric cysts occur in the posterior f o s s a 8 , 9 or at the craniocervical junction. 1 0 , 11 S u p r a t e n t o r i a l neurenteric cysts are very rare and none of the cysts in those cases reported to date have been located within the brain parenchyma. 3,[12][13][14][15][16][17][18][19] We report a case of a 35-year-old female who underwent successful excision of an intraaxial right frontal lobe neurenteric cyst. To our knowledge, this is the first case report of A B S T R A C T: B a c k g r o u n d : Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin. In this report we present the unique case of a supratentorial neurenteric cyst that is contained entirely within the brain parenchyma. M e t h o d s : Apatient presented with an intraparenchymal cystic lesion that was subsequently identified as a neurenteric cyst. This lesion is reviewed in light of the available literature. Case report: A 3 5 -y e a r-old female presented with a one year history of progressive headaches and seizure-like episodes. Her examination revealed no deficits. Magnetic resonance imaging showed a 4 cm x 4 cm x 4 cm cystic lesion within the parenchyma of the right frontal lobe. A right frontal craniotomy and complete excision of the cystic lesion was performed. Pathologic examination confirmed that it was a neurenteric cyst. Postoperatively the patient's symptoms improved. C o n c l u s i o n : Review of the literature revealed this to be the first case of a surgically excised, pathologically confirmed supratentorial neurenteric cyst, contained entirely within the brain parenchyma.RÉSUMÉ: Kyste neurentérique supratentoriel intraparenchymateux. Introduction: Les kystes neurentériques sont des kystes congénitaux du système nerveux central qu'on pense être d'origine endodermique. Nous présentons un cas unique de kyste neurentérique supratentoriel situé entièrement dans le parenchyme cérébral. Méthodes: Une patiente a consulté pour un kyste intraparenchymateux qui a été éventuellement identifié comme étant un kyste neurentérique. Nous faisons une révision de la littérature concernant cette pathologie. Cas clinique: Une femme âgée de 35 ans a consulté pour des céphalées progressives depuis un an et des crises é...

Mucinous adenocarcinoma arising from supratentorial intramedullary neuroenteric cyst with broncho‐pulmonary differentiation

“…1 However, supratentorial intramedullary occurrence as in our case is quite rare. 1,16 Stratified columnar epithelium of bronchial type with CK5-positive basal cells as in our case is frequently seen in neuroenteric cysts, but expression of TTF-1 is not so frequent. 17 TTF-1 is a marker common to distal airway epithelial cells, including bronchiolar Clara cells and alveolar pneumocytes.…”

“…Neuroenteric cysts are rare intradural cysts lined by the epithelial cells with immunophenotype analogous to endoderm cells, and most frequently occur in the spinal canal . However, supratentorial intramedullary occurrence as in our case is quite rare . Stratified columnar epithelium of bronchial type with CK5‐positive basal cells as in our case is frequently seen in neuroenteric cysts, but expression of TTF‐1 is not so frequent .…”

Intracranial neuroenteric cysts: A concise review including an illustrative patient

“…Multiple NC in both the supratentorial and infratentorial compartments have been documented rarely. 1,3,29,108,109 Midline-located NC were described in 63% of patients and 29% were located laterally, with reports of 11 (8%) patients not providing sufficient details on cyst location. Although NC are most commonly located in the midline, 61% of those located laterally were found on the left side and 39% on the right side.…”

Neuroenteric cyst of the left lateral ventricle: case report and review of the literature

“…In the reported cases of intracranial NCs, NCs mainly developed along the midline of the posterior cranial fossa, anterior to the brainstem or in the fourth ventricle [8,12,16]. In exceptional cases, multiple NCs in both the supratentorial and the infratentorial compartments have been documented [2,11,13,17,18]. There are 3 cases showed NCs in the supratentorial region [19][20][21], and 2 of these cases reported intramedullary lesions located in the cerebral hemisphere [19,20].…”

“…Clinical symptoms and signs of supratentorial NCs were greatly related to the increased intracranial pressure which led to the symptoms of headache, nausea and vomiting. Focal and generalized seizures with focal motor and sensory deficits were also observed in some NC patients [4,18,25,31,32]. Although the imaging characteristics and location of the cystic lesion might suggest the presence of the NC, they were not specific [5,15].…”