Intracranial neuroenteric cyst

Discussion:

Central nervous system neurenteric 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, they usually have non-CSF signal characteristics on MRI. In this instance, the MRI appearances are following resection, and therefore cannot be taken as typical. 

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

Histology

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.

Final diagnosis: 

Benign developmental cyst in keeping with a neurenteric cyst.

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