Retrolental vascular membrane in a case of ciliary body medulloepithelioma: Clinicopathologic correlation

Luca Bongiovanni De Miranda Gonçalves; Joao Carlos De Miranda Gonçalves; Maria Cristina Martins; Miguel N Burnier

doi:10.4103/PAJO.PAJO_21_19

CASE REPORT

Year : 2019 | Volume : 1 | Issue : 1 | Page : 15

Retrolental vascular membrane in a case of ciliary body medulloepithelioma: Clinicopathologic correlation

Luca Bongiovanni De Miranda Gonçalves¹, Joao Carlos De Miranda Gonçalves², Maria Cristina Martins², Miguel N Burnier³
¹ Santa Marcelina Medical School, São Paulo, Brazil
² Department of Ophthalmology, Federal University of São Paulo, Brazil
³ Department of Ophthalmology, McGill University, Montreal, Canada

Date of Submission	15-Sep-2019
Date of Acceptance	16-Oct-2019
Date of Web Publication	06-Nov-2019

Correspondence Address:
Dr. Joao Carlos De Miranda Gonçalves
Avenida Doutor Altino Arantes 16 4, Vila Clementino, São Paulo
Brazil

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/PAJO.PAJO_21_19

Abstract

A unilateral nonteratoid malignant ciliary body medulloepithelioma presented in a 13-year-old girl as a leukocoria with an extense vascularized retrolental membrane in her right eye. In this description, clinical documentation, posterior segment ultrasound, and ultrasound biomicroscopy were obtained, and an anatomopathological correlation was made by showing rosettes in some histological cuts stained by haematoxylin and eosin obtained from the mass and the membrane.

Keywords: Ciliary body, medulloepithelioma, ocular neoplasm

How to cite this article:
De Miranda Gonçalves LB, De Miranda Gonçalves JC, Martins MC, Burnier MN. Retrolental vascular membrane in a case of ciliary body medulloepithelioma: Clinicopathologic correlation. Pan Am J Ophthalmol 2019;1:15

How to cite this URL:
De Miranda Gonçalves LB, De Miranda Gonçalves JC, Martins MC, Burnier MN. Retrolental vascular membrane in a case of ciliary body medulloepithelioma: Clinicopathologic correlation. Pan Am J Ophthalmol [serial online] 2019 [cited 2023 Mar 23];1:15. Available from: https://www.thepajo.org/text.asp?2019/1/1/15/270448

Introduction

Medulloepithelioma is a rare congenital embryonic intraocular tumor in children originated from the primitive neuroectoderm, the structure responsible for the formation of the nonpigmented epithelium of the ciliary body. This neoplasm most commonly involves the ciliary body but in rare cases can affect the retina, optic disc, optic nerve, or iris.^[1]

The purpose of this case description is to report a clinical presentation of a ciliary body medulloepithelioma with an extense neovascular retrolental membrane and leukocoria in a 13-year-old girl.

case Report

A 13-year-old white female was evaluated at São Paulo Federal University (UNIFESP) due to pain, decreased vision, and presence of a white pupillary lesion in her right eye. She attested having a normal bilateral previous eye examination 1 year prior. She did not present with any complaints about her left eye. Her ophthalmological examination revealed corrected visual acuity of hand motions in the right eye and 20/20 in her left eye. Pupillary reflexes were normal. Extraocular muscle functions were preserved. At biomicroscopy, an extense and vascular retrolenticular lesion in the right eye was observed in an inverted sea-fan shape, apparently being originated from superior temporal toward inferior nasal, attested by the fluorescein angiogram [Figure 1]. Both lenses were preserved with a bilateral opened angle at gonioscopy. Indirect ophthalmoscopy was normal in the left eye but impaired by opacities in the right eye. A posterior ultrasound scan and an ultrasound biomicroscopy showed a peripheral cystic lesion located superior temporal of the right eye, without signs of infiltration or calcification [Figure 2]. Clinical and laboratory evaluation showed no abnormalities.

Figure 1: Clinical appearance of vascular retrolental membrane (color and black-white after fluorescein injection)

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Figure 2: B-scan and ultrasound biomicroscopy showing a solid iridociliary mass

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Enucleation was indicated due to continuous ocular pain, and anatomopathological examination was performed showing at the macroscopic examination, a shadow at the superior ciliary body region that served of guidance to the vertical eye dissection. Anterior segment was preserved, as well as the retina. At the microscopic level, cornea, anterior chamber, and iris were spared, but the angle was partially closed and the crystalline lens was slightly dislocated inferiorly. Ciliary body was fibrotic and partially disarranged. It was also observed in some histological slides that the presence of cell proliferation was organized in bands and forming rosettes [Figure 3]. The same cellular proliferation was also observed in the fibrovascular membrane originated from the ciliary body and directed toward the crystalline lens and pars plana. This tumor was limited to the ciliary body and pars plana in the 20 studied cuts. No workup of the tumor with immunohistochemistry was performed.

Figure 3: Hematoxylin and eosin stain of tubules of proliferating ciliary epithelium (rosettes)

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Discussion

Intraocular medulloepithelioma is a rare congenital tumor originated mostly from the nonpigmented ciliary epithelium of the pars plicata. In most of the cases, it is diagnosed in thefirst decade of life, it is unilateral without predominance for gender or race, and it occurs much less frequently than retinoblastoma.^[2] Establishing the diagnosis is not an easy task. In many cases, this is accomplished only following enucleation and anatomopathological analysis.^[2]

This case illustrates a malignant nonteratoid variant due to the absence of cartilage, glial tissue, and skeletal muscle. In the present case, we ruled out retinoblastoma due to the lack of calcification in the ultrasound and the age of presentation at 13 years of age (most cases of retinoblastoma present before 6 years of age). Intratumoral cartilage in teratoid medulloepithelioma can present calcification as retinoblastoma does, posing another challenge to the differential diagnosis. Unlike medulloepithelioma, retinoblastoma can appear bilaterally, be multifocal, and present a positive family history.^[3] The presence of rosettes, in this case, favors the diagnosis of medulloepithelioma but does not exclude differentiated retinoblastoma. The lack of an immunohistochemistry workup could have been an obstacle to reach a diagnosis. The nonteratoid component is typically positive for vimentin and enolase. Other immunohistochemical markers are currently under study for distinguishing a neuroepithelial tissue from a heteroplastic one.^[3]

There was no serologic evidence for toxocariasis or another possible granulomatous source. The patient denies contact with dogs. In the literature, one can realize that medulloepitheliomas can occasionally show a wide array of presenting features. Pigmented tumors were already described with deeply pigmented areas (clinical and histopathologically) in a malignant teratoid case.^[4]

A neoplastic cyclitic membrane extending to the retrolental area can be also characteristic of medulloepithelioma, and it is very useful to distinguish from retinoblastoma and Coats disease.^[3] As in the present case, a retrolental vascularized membrane was found in an inverted sea-fan shape, apparently being originated superior temporal [Figure 1]. On fluorescein angiography, we had a fast filling of big vessels coming from the ciliary body across the hyaloid face, whereas in total retinal detachments from Coats or retinoblastoma, the vessels would travel from the central funnel toward the ciliary body region. Coats disease presents with unilateral retinal vascular telangiectasia with exudative retinal detachment, but there is no ciliary body involvement.^[3] Membrane in medullepithelioma is a layer of neoplastic tissue that uses the hyaloid as a support to grow slowly, producing collagen and therefore becoming opaque, and then resembling anterior persistent hyperplastic primary vitreous (PHPV).^[3]

Such a membrane has been described previously in 51% in a large series of cases from a teratoid mass ^[5],[6] and can grow along the posterior lens capsule and the anterior hyaloid, resembling sometimes cases of anterior PHPV, but in PHPV, it occurs shortly after birth, when the remnant vascular stalk is seen attached to the back of the lens, but no longer extends back to the optic nerve. This form is typically associated with cataract, glaucoma, and retrolenticular membrane, which was not seen in this reported case. About 20% of eyes with medulloepithelioma also have histologic evidence of PHPV.^[7] The most frequent presenting signs in a large series ^[5] were poor vision, pain, mass in iris or ciliary body, and leukocoria – all of which were also present in this described case.

Delayed diagnosis in cases of the ciliary body, medulloepithelioma is common and mismanagement can occur.^[5] Mass enlargement will produce secondary effects such as unilateral glaucoma, visible pupillary mass, cataract with or without subluxation, metastasis, and even death. Our patient presented to us with ocular pain, decreased vision, and presence of a white pupillary lesion in her right eye. Due to the continuous pain, enucleation was performed and diagnosis was finally done. There was a good life prognosis in this patient due to the absence of extraocular extension signs or methastasis.^[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

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