Malignant non‐teratoid medulloepithelioma of ciliary body with retinoblastic differentiation: A case report and review of literature

Abstract

We present a 6-year-old child with intraocular and extraocular mass and high intraocular pressure. The tumor mass involved a disorganized anterior segment and extended through the medial cornea and sclera. A preliminary diagnosis of retinoblastoma with extraocular extension was made. An exenteration of the left globe and orbital tissue was performed. Histological examination showed that the lesion, which occupied the posterior chamber, involved the ciliary body, extending into the iris, sclera and cornea, projecting beyond the cornea anteriorly and extending to the retina posteriorly. The tumor cells were diffusely immunoreactive to vimentin, neuron specific enolase and CD 138. The medulloepithelioma cells were focally positive to cytokeratin (AE1/AE3), cytokeratin 18, CD56,CD57, S100, HMB-45 and bcl2 while areas of retinoblastic differentiation showed diffuse immunoreactivity to synaptophysin, neurofilament and CD138 with focal immunoreactivity to calretinin. All tumor cells showed no immunoreactivity to cytokeratin 7, cytokeratin 20, epithelial membrane antigen, carcinoembryonic antigen, desmin, GFAP, and chromogranin. Nuclear staining for P53 was seen in 80% of tumor cells. The ki-67 index was 90%. The tumor was described as malignant intraocular non-teratoid medulloepithelioma with retinoblastic differentiation arising from the ciliary body. Tumor satellites were seen in the adjacent periocular soft tissue. The treatment involved exenteration of the left globe and orbital tissue with secondary skin graft following chemotherapy. The patient is well and has no recurrence after 1 year of treatment. We report that medulloepithelioma can present as a case of infantile glaucoma, can show signs of intraocular calcifications and can show retinoblastic differentiation.

Intraocular Medulloepithelioma

“…[22][23][24][25][26][27][28][29][30] Limited and conflicting results have been reported for chromogranin, synaptophysin, glial fibrillary acid protein, S100 protein, and HMB-45. 12,[22][23][24][25][26][27][28][29][30] Positive immunohistochemistry for pancytokeratins and cytokeratin (CK)18, with no reactivity for CK7, CK20, and epithelial membrane antigen, has been described and awaits confirmation. 23 Fine-needle aspiration biopsy will probably have a greater role in diagnosing medulloepithelioma in the future.…”

“…12,[22][23][24][25][26][27][28][29][30] Positive immunohistochemistry for pancytokeratins and cytokeratin (CK)18, with no reactivity for CK7, CK20, and epithelial membrane antigen, has been described and awaits confirmation. 23 Fine-needle aspiration biopsy will probably have a greater role in diagnosing medulloepithelioma in the future. 26,28-30 While cords of medullary epithelium may be diagnostic of medulloepithelioma, the absence of malignant neuroblasts, or cells with sarcomatous features, does not exclude the possibility of malignancy.…”

Pediatric choroidal melanoma in a 13-year-old girl—a clinical masquerade

“…Staining patterns suggestive of melanoma include HMB-45, Melan-A, S-100, and NKI-C3. 6 In our case, although clinical findings were initially suggestive of an intraocular tumor, the history of trauma was difficult to ignore. Intraoperative findings were suggestive of medulloepithelioma.…”

Rapidly growing primary malignant teratoid medulloepithelioma of the ciliary body in a gelding

“…Medulloepitheliomas most often have a ciliary origin, but the optic nerve and retina are other potential structures that may be at the origin of an intraocular medulloepithelioma (Al-Salam and others 2008). Furthermore, retinoblastic differentiation of meduloepithelioma has been reported previously (Al-Salam and others 2008). In current veterinary literature, the differentiation between the two conditions is confusing.…”