Intracranial neuroenteric cyst

Case contributed by Dr Bruno Di Muzio

Presentation

Headache.

Patient Data

Age: 40
Gender: Male
CT

CT Brain (5 years ago)

Right frontal sharply demarcated cyst lesion creating smooth scalloping against the adjacent brain parenchyma, but no significant mass effect is noted. Although the lesion is favoured to be extra-axial, the parenchyma grey-matter surrounding the cyst is indistinct. There is no scalloping of the calvaria. 

CT

CT Brain (3 months ago)

The cystic lesion has increased in size when compared to the images from 5 years ago, and now causes compression of the lateral ventricle and early subfalcine herniation. 

CT

CT Brain (one month ago)

Right frontal approach craniotomy with overlying staples, soft tissue swelling subcutaneous emphysema. There is a prominent air-fluid level intraparenchymal blood at the anterolateral margin of the surgical bed. A small amount of blood poles at the dependent aspect of the right frontal fluid collection. There is 8 mm left which shift of the septum pellucidum. There is mild sulcal effacement of the right cerebral hemisphere. The basal cisterns are not effaced. The grey-white matter differentiation is preserved. 

MRI

MRI Brain

Right frontal craniotomy. A large cystic space that follows CSF signal on all sequences demonstrates similar dimensions when compared to the previous CT brain, although has distinctly enlarged when compared to the more distant CT brain from years ago. This lesion demonstrates a gently lobulated margin with some thin non-enhancing septations noted superiorly. A small volume of non-enhancing material lies dependently within. Inferiorly, this cystic lesion appears to abut white matter of the deep right frontal lobe with no intervening cortex. There is no associated enhancing components to this cystic structure. No communication with the right lateral ventricle. Increased size results in a progressive mass effect, with partial effacement of the right lateral ventricle as well as subfalcine herniation and mild right uncal herniation. No evidence of entrapment of the left lateral ventricle. Remote from this cystic lesion, the remainder of the intracranial structures have a normal appearance. The optic nerve sheaths are noted to be symmetrically mildly CSF distended. 

Case Discussion

Central nervous system neuroenteric cysts are congenital rare lesions that are more frequently seen involving the spine. Cases like this one, in a supratentorial location, are even rarer. On imaging, this case cannot be confidently distinguished from an arachnoid cyst, which is much more common. 

The patient underwent further surgical resection of the frontal cystic lesion.

MACROSCOPIC DESCRIPTION: 1. "R frontal nodule of cyst": One tan core tissue, 9mm long. A1. 2. "R frontal cyst wall": A flap of thin translucent tissue, 10x3mm in area and <1mm thick. The cyst wall is smooth on both sides. No mass lesions identified. A1.

MICROSCOPIC DESCRIPTION: 1&2. The two specimens show similar features and are described together. Examined at multiple levels, the sections show cyst wall lined with pseudostratified ciliated columnar epithelium with fine intracytoplasmic brown pigment, mixed with goblet cells and cells with PASD positive granules. No complete epithelial excrescences are present. The underlying connective tissue also shows brown pigment. No pituitary tissue is identified. The cells show patchy positive staining for CDX2 and SOX10, and are negative for EMA and Transthyretin. In the context of the intra-axial site of the frontal lobe cyst the features are most in keeping with the neuro-enteric cyst. No atypical features are identified.

DIAGNOSIS: 1&2. Right frontal cyst wall biopsy: Benign developmental cyst in keeping with neuro-enteric cyst.

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Case information

rID: 56939
Case created: 30th Nov 2017
Last edited: 8th Dec 2017
Inclusion in quiz mode: Excluded

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