Presentation
Long-lasting nasal obstruction and sinus pain.
Patient Data
The tumor is visible in the left nasal cavity with the destruction of the nasal septum and small amount of lesion in the right nasal cavity, the expansion to the left bulla ethmoidalis and the left ethmoid air cells are noticed. The left maxillary sinus contains only a very small amount of tumor but is filled with fluid as the ostiomeatal complex is obstructed. The secondary sinusitis is noticed in the frontal sinuses and the sphenoid sinus. It showed on T1W contrast-enhanced and T2W sequences parallel lines of high and low signal intensity (convoluted cerebriform pattern). The mass avidly enhanced following contrast administration.
Atrophy of the cerebellar hemispheres and the brainstem (mainly pons and medulla oblongata) with a prominence of the prepontine cistern and fourth ventricle. Visible thinning of both middle cerebellar peduncles. The "hot cross bun sign" is present in the pons on T2-weighted images. Widened subarachnoid spaces and sulci in both cerebral hemispheres.
Case Discussion
Features of the tumor are characteristic of the inverted papilloma. The tumor was surgically excised.
Histology
Polypoid tissue covered with columnar epithelium with goblet cells, which showed inversion into the underlying connective tissue stroma.
Diagnosis: inverted papilloma
Discussion
Atrophy of cerebellum and brainstem, with hot cross bun sign of the pons raises the possibility of multiple system atrophy (MSA-C) although other neurodegenerative diseases can have a similar appearance.
Patient's symptoms connected with the MSA-C from the medical history and clinical examination: ataxia and dysarthria. Unfortunately, the patient hasn't had any further exams in our hospital.