Orbital Paraganglioma in Infancy: Does It Behave More Aggressively?

Abstract

Paragangliomas of the head and neck region are not only rare but also unique in that they are derived from the parasympathetic system and are nonsecretory. Orbit represents 1 of the least common sites for head and neck paragangliomas. Orbital paraganglioma remains predominantly a disease of adults, and it has never been reported in infancy earlier. Therefore it is not known if these tumors in infancy behave any differently than their adult counterparts. A 1-year-old boy presented with left-sided proptosis and restricted ocular movement for 3 months. On examination, the proptosis was nonaxial and nonreducible and the child could follow objects with the left eye with normal pupillary reactions bilaterally. On neuroimaging, a rounded enhancing mass was seen in the superomedial aspect of the left retrobulbar space closely abutting the optic nerve. The tumor was subtotally excised using a left fronto-orbital approach, leaving some tumor on the optic nerve. It was decided to closely observe the residual tumor in the follow-up period; however, the child developed recurrence with visual loss after 6 months of surgery. Orbital paraganglioma in infancy may be more aggressive than the adult counterparts. Although simple excision without adjuvant radiation may be tried in localized tumors with intact vision, recurrence tends to be high. Orbital exenteration with or without radiotherapy remains the only salvage option in such recurrences.