Aggressive, recurrent diffuse orbital Lipoblastomatosis - a rare and challenging encounter

Lipoblastomatosis is a rare primitive embryonal adipose tissue tumor seen in the early pediatric age group. It is diffusely infiltrative but typically benign in nature. The standard of care for the management of these tumors is complete surgical extirpation. Incomplete excision is reported to have a higher incidence of recurrence. It is an extremely uncommon tumor in the orbit and only a few cases have been previously reported in the ophthalmic literature. Herein, the authors present the case of a diffuse orbital lipoblastomatosis in a young girl that displayed very aggressive behaviour with relentless recurrence even after a radical surgical approach with adjuvant therapy. Apart from documenting the rarity of this tumor in the orbit, this report also explores the challenges in making a definitive diagnosis and planning the management in cases that are aggressive and recurrent in nature.