Ciliary Body Medulloepithelioma

Adenoma of the non-pigmented ciliary epithelium in a 13-year old child

“…Медуллоэпителиома, будучи наиболее частым морфологическим вариантом среди всех опухолей НПЦЭ врожденного генеза, возникает преиму-щественно в детском возрасте [4]. Приобретенные опухоли НПЦЭ, в отличие от врожденных вари-антов, развиваются из полностью дифферен-цированного цилиарного эпителия и встреча-ются у людей более старшего возраста [3,[5][6][7][8][9][10].…”

Commentary on the Masquerades of a Childhood Ciliary Body Medulloepithelioma: A Case of Chronic Uveitis, Cataract, and Secondary Glaucoma

“…Glaucoma is also a common early clinical feature [9] as a result of the tumor's close proximity to the anterior chamber angle as well as its secondary inflammatory and ischemic effects. The frequent association between the presence of cyclitic membranes and ciliary body medulloepithelioma has been described in the literature [3]. It is thought that these features could represent local migration of the tumor tissue into the anterior vitreous space.…”

“…The clinical picture is typical of a nonhereditary slow growing locally invasive tumor in a child of a median age of 2-5 years old [2]. This condition is uncommon in adulthood [3]. Histopathological studies have classified ciliary body medulloepitheliomas as benign or malignant in nature, with or without teratoid features.…”

“…Three case series in the literature have highlighted that approximately 50-63% were nonteratoid, while 38-50% had teratoid features [2,4,5]. Hyaline cartilage was most frequently seen in the teratoid group [3]. In both groups, there were a higher proportion of malignant cases compared to benign ones.…”

“…In our case, the tumor only became clinically apparent over a year after presentation and measured 11 × 8 mm on ultrasound examination ( Figure 2). As such, the early clinical picture is often one that masquerades as the pathology of other ocular structures that are in close proximity to the tumor, with up to 88% of misdiagnosed cases in a reported series [3]. The lens is very frequently involved and the child can present with a myriad of lens-related pathology such as a lens opacity [7][8][9], lens notching (referred to as a "lens coloboma") [4,6,7] and ectopia lentis [7,10,11].…”