Pigmented ciliary body medulloepithelioma in a newborn infant.

Ciliary body medulloepithelioma is a nonpigmented tumor which presents during the first decade of life. It is rarely seen in newborn infants. We report a pigmented medulloepithelioma in a newborn infant, which showed a rapid growth. Ciliary body medulloepithelioma should be considered in the differential diagnosis in a newborn infant presenting with leukocoria.

Introduction

Intraocular medulloepithelioma is a rare intraocular tumor arising from the nonpigmented cells of the ciliary epithelium.[1] This tumor most frequently arises from the ciliary body but can also arise from the retina, iris, and optic nerve.[12] We report a rare case of pigmented ciliary body medulloepithelioma in a newborn child, which showed rapid growth.

Case Report

A 15-day-old male child was brought to the hospital by his parents who had noticed a white reflex in the right eye of 4-day duration. On anterior segment examination, the right eye showed a vascularized whitish-yellow mass at the pupil extending behind the iris temporally [Figure 1a]. Posterior pole and the rest of the retina looked normal [Figure 1b]. The left eye was normal. B-scan examination of the right eye showed a moderate to high reflective mass temporally arising from the ciliary body with an attachment to the retina [Figure 1c]. Magnetic resonance imaging showed an enhancing lesion in the posterior aspect of the ciliary body projecting into the vitreous. A differential diagnosis of persistent hyperplastic posterior vitreous or medulloepithelioma was made.

Figure 1

(a) RetCam photograph of anterior segment of the right eye showing fibrovascular whitish-yellow mass behind the lens, temporally (white arrow). (b) RetCam photograph of posterior segment of the right eye showing the fibrous mass temporally (white arrow) with normal posterior pole. (c) B-scan of the right eye showing a moderate to high reflective mass (black arrow) with a thin stalk attached to the retina (white arrow). (d) Histopathology slide of enucleated specimen with ×40 magnification and hematoxylin and eosin staining showing necrotic and apoptotic cells (black arrow) with pigmentation (white arrow)

Trans-scleral incision biopsy was performed under general anesthesia. Histopathology showed mild ciliary hyperplasia with no evidence of malignancy. At 1-month follow-up, there was an increase in the size of the mass, and after explaining poor visual prognosis to the family, the eye was enucleated. Histopathology report showed primitive neuroepithelial cells with mitosis and necrosis arranged in cords and rosettes with areas of pigmentation [Figure 1d]. The final diagnosis of malignant nonteratoid pigmented medulloepithelioma was made. Systemic examination by an oncologist was normal. The patient is now on regular follow-up and has a healthy socket with no evidence of recurrence.

Discussion

Medulloepitheliomas can be histopathologically classified into teratoid and nonteratoid varieties and into benign and malignant varieties. Although there is no universally accepted criterion for determining the malignancy of the tumor, Zimmerman and Broughton standards are generally considered as a reasonable measure of malignancy of the tumor.[3] The features are sheets of poorly differentiated neuroblastic cells, sarcomatous areas, greater pleomorphism or mitotic activity, and extraocular extension. Biopsy specimen of this case did not show any of the above features of malignancy. The probable reason for the benign appearance could have been the possibility that the sample taken for biopsy was not representative of the entire mass.

Treatment options for ciliary body medulloepithelioma include cryotherapy for very small anterior tumors or recurrences, local excision of small tumors, brachytherapy of small-medium tumors, external beam radiotherapy, and enucleation.[3] Considering the size of the tumor and its rapid growth, we preferred enucleation. In this case, the medulloepithelioma was pigmented on histopathology. This is an extremely rare occurrence as most of these tumors are amelanotic. There are only few cases of pigmented medulloepitheliomas reported previously.[4] However, pigmented ciliary body medulloepithelioma has never been reported in a newborn. These patients should also be carefully followed for the development of pleuropulmonary blastoma.[5]

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Conflicts of interest

There are no conflicts of interest.