Immature Orbital Teratoma With Intracranial Extension

Abstract

We present the case of a 20-day-old boy brought to the Head and Neck Surgery Department at Instituto Nacional de Cancerologı´a in Bogota, Colombia, with the chief complaint of an enormous, protruding mass of the right eye (Fig. 1A). Magnetic resonance revealed an ocular globe replaced by a calcified, multilocular, mainly cystic mass causing total loss of the anatomic pattern and expansion of the osseous walls and complete displacement of the eyeball, without extension to neighboring structures (Fig. 1B). At 28-day old he underwent surgical excision of the tumor (Fig. 1C). The histopathologic analysis revealed an immature orbital teratoma (Fig. 1D and E). In 2011, Polito et al2 stated that approximately 70 patients have been reported. Orbital teratomas usually present in otherwise healthy children with unilateral congenital proptosis. Epidemiologically, our patient differs in some aspects. The literature shows a 2:1 female-to-male ratio and a slight preponderance for the left orbit.3 We present a male patient with a right eye orbital teratoma. Gnanaraj et al4 outlined the objectives for their management: to save the eye, allow for normal orbitofacial development, and to maintain cosmesis. Preservation of the globe is not possible when the eye is disorganized, when the nerve engulfs the optic nerve or when there is extreme proptosis with exposure keropathy, as in the patient that presented to our institution. Regarding treatment options, it is clear that surgical resection of the mass has been the method of choice since 1863. In our patient, total removal of the tumor was not possible, as it was documented in both postoperatively imaging and histopathological reports. Therefore, concomitant radiotherapy was necessary. This situation puts our patient in a lifelong follow-up program and it includes regular clinical and radiographic evaluation of the growth and development of the face and orbital complex. The large ocular defect created by the surgical resection of the tumor calls for prosthetic rehabilitation.