Malignant congenital orbital teratoma: Glioblastoma multiforme with primitive neuronal pattern and choroid plexus papilloma: A rare diagnosis at an unusual age.

Abstract

Childhood orbital teratomas are usually congenital lesions that are most often present at birth with progressive, massive unilateral proptosis. During the routine controls of 27-year-old woman between 26-27th weeks of pregnancy her fetal ultrasonography (USG) revealed a mass in the eye of the fetus, and termination was recommended. The family refused the termination option and in the 37th week of pregnancy, vaginal delivery is performed in an external medical center. The mass in the baby's eye was surgically removed. Microscopic examination revealed disorganized ocular tissues, adipose tissue, microcalcification, nerve plexuses as well as areas of neuronal nodules including hypercellular areas, palisatic necrosis, and microvascular proliferation. The immunprofile--patchy GFAP staining in the areas of cellular epithelioid and spindled cells that also show an focal and sparse expression p53 staining and a high proliferation rate in Ki67 staining--confirmed the hematoxylin-eosin (HE) impression of a teratoma with a component of glioblastoma (GBM). In this unique presentation of a malignant orbital teratoma with a GBM, we have identified three tumor components: (1) GBM component, (2) nodules of neuroglial tissue with mature neurons and BRAF mutation, and (3) papillary proliferation possibly representing a choroid plexus papilloma.