Plaque brachytherapy in unresectable medulloepithelioma with anterior chamber seeding and review of literature

Payal Naresh Shah; Mahesh Shanmugam Palanivelu

doi:10.4103/ijo.IJO_2148_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 380-383

Plaque brachytherapy in unresectable medulloepithelioma with anterior chamber seeding and review of literature

Payal Naresh Shah, Mahesh Shanmugam Palanivelu
Vitreo-Retina and Ocular Oncology Services, Sankara Eye Hospital, Bangalore, Karnataka, India

Date of Submission	27-Aug-2022
Date of Acceptance	30-Jan-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Mahesh Shanmugam Palanivelu
Vitreo-Retina and Ocular Oncology Services, Sankara Eye Hospital, Kundalahalli Gate, Bangalore, Karnataka
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2148_22

Abstract

Medulloepithelioma is a rare nonhereditary tumor seen most often in children, usually in the first decade. In this paper, we present a case of extensive unresectable medulloepithelioma with neovascular glaucoma and anterior chamber seeding, which was confirmed histopathologically by performing biopsy and successfully managed using plaque brachytherapy along with a review of literature on brachytherapy in medulloepithelioma. The unique features in our case are extensive tumor, with anterior chamber seeding and neovascular glaucoma which have been successfully managed with brachytherapy. Also, the placement of brachytherapy plaque over the corneal surface has not been described previously for managing extensive medulloepithelioma. So far, brachytherapy has been tried in only three cases with concomitant neovascular glaucoma, of which two cases required subsequent enucleation.

Keywords: Brachytherapy, histopathology, medulloepithelioma

How to cite this article:
Shah PN, Palanivelu MS. Plaque brachytherapy in unresectable medulloepithelioma with anterior chamber seeding and review of literature. Indian J Ophthalmol Case Rep 2023;3:380-3

How to cite this URL:
Shah PN, Palanivelu MS. Plaque brachytherapy in unresectable medulloepithelioma with anterior chamber seeding and review of literature. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 3];3:380-3. Available from: https://www.ijoreports.in/text.asp?2023/3/2/380/374939

Medulloepithelioma is a rare nonhereditary tumor seen most often in children, usually in the first decade. However rarely, the tumor can also occur in adults. Due to its origin from nonpigmented ciliary epithelium, the tumor appears whitish or grayish white in color, although rarely it can also arise from pigmented ciliary epithelium.^[1] It is classified into nonteratoid and teratoid types which can either be benign or malignant.^[2] Broughton and Zimmermann described identification of malignant medulloepithelioma based on the presence of retinoblastoma-like elements with or without rosettes, sarcoma-like elements, pleomorphism with high mitotic index, and invasion into adjacent structures like uvea, lens, sclera, cornea, optic nerve, or orbit.^[3] Adults are more likely to present with malignant tumor.^[4] The most common site is at the ciliary body and iris, followed by retina or optic nerve head.^[5]

Management options in early or small tumors (<3–4 clock hours) are cryotherapy or plaque brachytherapy or partial lamellar sclerouvectomy. Local tumor recurrence is reported to be as high as 50–80% after local resection eventually requiring enucleation.^[3],[6] In large or extensive tumors with neovascular glaucoma, enucleation is the treatment of choice.^[7] Very rarely, metastasis and tumor-related deaths have been reported due to extraocular extension and involvement of the central nervous system.^[3],[6]

In this paper, we present a case of extensive unresectable medulloepithelioma with neovascular glaucoma and anterior chamber seeding, which was successfully managed using plaque brachytherapy along with a review of literature on brachytherapy in medulloepithelioma.

Case Report

A 4-year-old girl presented to us with her father complaining of left eye (LE) redness for one week. On examination, her best-corrected visual acuity was 6/6 in the right eye (RE) and 6/36 in the LE. On anterior segment examination, RE was unremarkable. LE showed circumcorneal congestion, neovascularization of the iris (NVI) in 3 clock hours (over the involved tumor, around the pupillary margin inferiorly and temporally), whitish gelatinous cystic lesion arising from the ciliary body on the temporal side pushing the lens nasally, lens showing cataractous changes with a temporal notch, temporal stretching of zonules with cysts over zonules and retrolental membrane. Multiple cysts of varying sizes, some partly retroiridal and extending into the anterior chamber and a few free floating in the anterior chamber, were noted [Figure 1]. Fundus examination in the RE was normal. LE fundus showed a healthy disc with 0.4 cup disc ratio, normal foveal reflex, and attached retina. The extreme periphery of the LE fundus could not be seen due to the retrolental membrane and ciliary body mass. There was no positive ocular history in the family.

Figure 1: Anterior segment photographs showing temporal cystic tumor (white arrows) and anteriorly free-floating seeds (white arrows) with Koeppe lens in-situ (a) and intraoperatively during biopsy (b)

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A differential diagnosis of medulloepithelioma or diffuse anterior retinoblastoma was made, and the child was posted for examination under anesthesia. Intraocular pressure (IOP) was noted to be 15 mm of Hg in the RE and 38 mm of Hg in the LE with Tonopen^TM (Reichert, NY, USA). Gonioscopy in the LE showed 360-degree angle closure. An ultrasound biomicroscopy of the LE showed a cystic mass involving nearly 7 clock hours of the ciliary body. A biopsy was planned and anterior chamber (AC) entry from inferonasal site (7 o clock) and superotemporal site (1 o clock) were made via limbal route; multiple cysts of 3–5 mm size were collected and sent for histopathological examination (HPE) in formalin solution, and the child was started on brinzolamide and timolol eye drops.

HPE revealed pseudostratified primitive neuroepithelial cells with fibrillary background suggestive of low-grade benign nonteratoid type of medulloepithelioma [Figure 2]. A Ruthenium-106 plaque brachytherapy was planned with an apex dose of 5000 cGy to the tumor, calculated for an apex height of 3 mm. The plaque was in-situ for 20 h. The plaque was placed temporally over the ciliary body and covering almost 90% of the corneal surface [Figure 3]. One-month postbrachytherapy, the tumor regressed completely. Due to persistently raised IOP (39 mm of Hg) despite topical antiglaucoma medication, a 270-degree transcleral cyclophotocoagulation was done.

Figure 2: HPE showing pseudostratified primitive neuroepithelial cells with fibrillary background suggestive of low-grade benign nonteratoid type of medulloepithelioma

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Figure 3: Ru-106 plaque (white arrow) brachytherapy placed over the corneal surface

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After six months, the tumor was under control and NVI regressed. Due to the nasally subluxated notched and cataractous lens, a pars plana lensectomy was performed. [Figure 4] shows a regressed tumor. At one-year follow-up, her eye was stable with aphakia, no tumor residue, and stable fundus.

Figure 4: Regressed tumor with notched cataractous lens

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Discussion

In a child presenting with unilateral neovascular glaucoma with a normal fundus, medulloepithelioma needs to be strongly suspected. Medulloepithelioma can also cause congenital lens notch or lens coloboma or lens subluxation due to sparse or weak zonules as noted in our case. Other ocular associations of medulloepithelioma are cataract, glaucoma, free-floating cysts in aqueous or vitreous, retrolental neoplastic membrane, and persistent hyperplastic primary vitreous and retinal detachment. Rarely, it can also cause extraocular extension or metastatic spread to parotid glands, cervical lymph nodes, and facial soft tissues.^[8],[9]

So far, there are very few case reports/series published on brachytherapy in the management of medulloepithelioma. [Table 1] shows the review of literature published so far on brachytherapy in medulloepithelioma. All the cases reported were confirmed prior by fine needle aspiration biopsy. The unique features in our case are extensive tumor, with anterior chamber seeding and neovascular glaucoma, which have been successfully managed with brachytherapy. Also, the placement of brachytherapy plaque over the corneal surface has not been described previously. So far, brachytherapy has been tried in only three cases with concomitant neovascular glaucoma, of which two cases required subsequent enucleation.

Table 1: Review of literature on brachytherapy in medulloepithelioma

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Of late, intracameral and intravitreal melphalan have also been tried in medulloepithelioma with neovascular glaucoma with successful outcomes.^[17] Combined intracameral (2.25 μg/0.15 ml) and intravitreal melphalan (30 μg/0.15 ml) injections can be given every 7–10 days, similar to treatment of aqueous and vitreous seeding in retinoblastoma.

While medulloepithelioma is usually intraocular, a delay in diagnosis can lead to extrascleral extension and metastases. Malignant tumors, despite undergoing enucleation and exenteration, can develop local recurrence or metastasize to cervical or parotid lymph nodes or even intracranial extension.^[18],[19] The role of adjuvant and neoadjuvant chemotherapy (vincristine and carboplatin) in metastatic ocular medulloepithelioma has also been reported.^[20]

Conclusion

Medulloepithelioma is a rare tumor which may require histopathological confirmation before treatment. Plaque brachytherapy can be a successful treatment modality in the management of these tumors.

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.

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