Presentation
At birth, a large mass was seen behind the left globe which resulted in protrusion and decreased acuity in the left eye.
Patient Data
There is a large left orbital mass which displays areas of fat attenuation and fully formed long bones. The left globe is along the ventral margin of the mass, and the optic nerve sheath is markedly thinned. There is no evidence of dehiscence of the left frontal calvarium.
There is a large expansile left orbital mass that measures 5.1 x 4.6 x 5.1 cm (TRV, CC, AP) without intracranial extension. The mass demonstrates predominantly T1 hyperintense signal with areas of interspersed linear T1 hypointense signal. There are foci of mineralization as well as fully formed long bones within the mass.
The globe is displaced ventrally and is flattened along its posterior margin. The left optic nerve courses within the superior and medial aspect of the left orbit, and is hypoplastic when compared to the right. The extraocular muscles are not identified with certainty. The lacrimal gland is also not well delineated. The left orbital roof is intact, without evidence of dehiscence.
The right orbit and globe is within normal limits.
Case Discussion
This is a pathologically proven teratoma of the orbit. It has characteristic imaging features (fat and calcification). Histopathologic examination of the tumor revealed orderly arranged mature ectodermal elements (squamous epithelium with adnexal structures), mature mesodermal elements (adipose tissue, cartilage, and bone), and endodermal elements (developing small intestine). The latter of which was seen on hematoxylin and eosin-stained slides as intestine with notable areas of mucosa, submucosa, muscularis propria, and serosa. The mucosa showed multifocal villous architecture with Goblet cells, scattered Paneth cells, and enteric ganglion cells. No immature tissue or malignancy was identified.
Co-authors:
Travis Bevington, MD
Gaetano Pastena, MD
Joseph Giampa, DO
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