Orbital Pleomorphic Liposarcoma in an Eight-Year-Old Boy

Abstract

Adult-type sarcomas are, as the name indicates, rare tumors in the pediatric population. Although soft tissue sarcomas as a group are not uncommon diagnoses, nonrhabdomyosarcoma soft tissue sarcomas are much rarer and encompass a wide range of diagnoses. A few of these tumors are commonly found in the adult population and are thus referred to as adult-type sarcomas. We present a case of a pleomorphic liposarcoma, an adult-type sarcoma, arising as a primary tumor in the orbit of an 8-year-old boy. The histologic analysis revealed bizarre tumor giant cells and definitive lipoblastic differentiation. The atypical cells were positive for S100, and negative for CD34, desmin, MyoD1, and myogenin. This is a high-grade sarcoma, very rarely encountered in the pediatric population. We present the histologic findings of this unusual pediatric sarcoma and review the literature.