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Case Reports
. 2017 Jan;3(1):11-16.
doi: 10.1159/000448144. Epub 2016 Sep 7.

Congenital Orbital Teratoma

Fernando Pellerano  1 , Elvis Guillermo  2 , Gloreley Garrido  2 , Pedro Berges  3
Affiliations
Case Reports

Congenital Orbital Teratoma

Fernando Pellerano et al. Ocul Oncol Pathol. 2017 Jan.

Abstract

We report a case of congenital orbital teratoma. A 3-day-old male, born at 39 weeks' gestation without relevant prenatal history, presented with a large vascularized proptotic mass distorting the left midface. Laboratory studies showed elevated serum alpha-fetoprotein (12,910 ng/ml). Computed tomography showed a multiloculated heterogeneous lesion composed of hypodense and hyperdense calcified areas encompassing the whole orbital cavity with expansion of the bony walls, as well as forward displacement and compression of the eyeball without extension to surrounding structures. Clinical, imaging and laboratory features were consistent with congenital orbital teratoma. Due to pronounced proptosis with exposure keratopathy and corneal perforation, no motility of the globe and no vision in the affected eye in a resource-limited setting, the patient underwent orbital exenteration. Histopathological examination confirmed the diagnosis of mature cystic teratoma. We describe the clinical course, radiographic and histopathological findings of this rare orbital tumor.

Keywords: Congenital teratoma; Orbital tumor; Proptosis in a neonate.

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Figures

Fig. 1
Fig. 1
a, b Frontal (a) and lateral (b) preoperative images of the left orbital mass showing eyelid retraction with chemosis, exposure keratopathy with corneal perforation, and anterior chamber inflammation with hypopyon. c Left nostril compression and downward displacement of the left angle of the mouth.
Fig. 2
Fig. 2
a, b Axial (a) and sagittal (b) CT scans showing a heterogeneous lesion composed of hypodense and hyperdense calcified areas encompassing the whole orbital cavity with expansion of the bony walls. c Multiple axial cuts showing forward displacement and compression of the eyeball.
Fig. 3
Fig. 3
a Macroscopic appearance of the resected specimen. b Low-magnification image of ectodermal component: keratinized stratified squamous epithelium with appendages. c High-magnification image of mesodermal component: bone and cartilage. d Low-magnification image of endodermal component: ciliated pseudostratified columnar epithelium with goblet cells resembling bronchial epithelium. b-d Hematoxylin and eosin: ×4 (b), ×40 (c) and ×10 (d).
Fig. 4
Fig. 4
Two months (a) and 12 months (b) after the procedure.
See this image and copyright information in PMC

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