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Purpose : To report a rare case of pigmented epithelioid melanocytoma of the orbit in a 6 year-old girl.
Methods : A 6 year-old girl had a history of slowly growing noticeable mass in the right upper eyelid since 1 year. Right upper eyelid was droopy and slightly swollen with no history of redness nor accompanying pain. Visual acuity of right eye was 6/20 with hypotropia and slight proptosis to inferior. There was soft, smooth-surfaced, painless palpable mass at the superotemporal region without noticeable skin changes. Other ocular examination of the right eye and left eye was within normal limit. Orbital CT scan showed a distinct, homogenous, enhancing contrast mass at the superotemporal region extending from anterior to 2/3 of the globe.
Results : Complete tumor excision was done through extended lid crease lateral orbitotomy. Histopathology examination revealed features of round/oval nucleus epitheloid cells and heavily pigmented cytoplasm suggestive of pigmented epithelioid melanocytoma. Three month follow up after surgery there was no sign of mass recurrency. The right eye was no longer protruded and back in orthophoric position, with some improvement in visual acuity
Conclusion : Although extremely rare, pigmented epitheloid melanocytoma of the orbit should be considered in the diagnosis of heavily pigmented lesion other than malignant melanoma.
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