Primitive Myxoid Mesenchymal Tumor of Infancy in the Orbit: A New Location for a Rare Tumor

Primitive myxoid mesenchymal tumor of infancy is a rare subtype of sarcoma. With the advent of relevant immunohistochemical and genetic analysis, it was defined by Alaggio et al. in a 2006 case series. Since then, 25 further cases are described in the literature-arising variably from the neck, chest, scalp, abdomen, back, or limbs. Here it is described for the first time arising in the orbit, confirmed by identification of BCOR immunopositivity with internal tandem duplication. All specialties involved in the management of orbital masses should be aware of the subtypes of sarcomas found in the orbit as approaches to their management may change depending on the diagnosis. As more cases are identified, a better understanding of this tumor's clinical behavior and appropriate management can be established.