Adenoid cystic carcinoma presenting as a retro-orbital mas
Adenoid Cystic Carcinoma (ACC) accounts for 4.8% of primary orbital neoplasms and commonly arises super laterally from the main lacrimal gland, and rarely may arise in the medial aspect of the orbit from the lacrimal apparatus. Primary ACC of the orbit from an extralacrimal region is quite rare. Published in the journal Ophthalmology Case Reports, the authors report a unique presentation of an ACC, presenting as an orbital apex mass.

A 38 years old female with African ancestry presented to our clinic with complaints of insidious onset, intermittent in nature, gradually worsening frontal region headache of 2 years duration, proptosis of 1 year duration associated with recent onset of double vision since 2 months, and restricted lateral eye movement since 2 months.

She gave no history of decreased vision, vomiting, seizures, or any symptoms suggestive of peripheral motor or sensory deficits. On clinical examination she was found to have proptosis, mild chemosis, limited right lateral ocular motility and diplopia. She was evaluated with MRI brain (Figure 1a and 1b), which showed altered signal intensity lesion in the right orbit in extra clonal region appearing isointense in T1W and hypointense in T2W images in the temporal region of the orbit seen separately from the lateral rectus muscle and lacrimal gland.

She underwent supraorbital craniotomy and decompression of the lesion. Histopathological examination was confirmative of ACC (Figure 2a and 2b). On IHC, P63 Highlights the Albumin Cells Whereas C-KIT Highlights the luminal cells.

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