Analysis of clinical and pathological features of ciliary body medulloepithelioma

Abstract

AIM: To analyze and summarize the clinical and pathological features of ciliary body medulloepithelioma. METHODS: The clinical and pathological data of 11 patients (11 eyes) who were diagnosed with ciliary body medulloepithelioma at Beijing Tongren Hospital, Capital Medical University, from 2007 to 2021 were retrospectively analyzed. RESULTS: The initial symptoms of 11 patients included vision loss (6 eyes), atrophia bulbi (1 eye), proptosis (2 eyes), and leukocoria (2 eyes). Most patients suffered with corneal opacity, anterior chamber flare and hyphema. Iris neovascularization and synechia, complicated cataract, and secondary glaucoma occurred in several cases. Three patients even had lens subluxation and retinal detachment. B-scan ultrasonography showed vitreous opacity and a medium-high uneven echo mass in the eyeball. Ultrasound biomicroscopy examination showed a spherical or hemispherical ciliary body mass with uneven internal echoes and irregular cystic spaces. All of the 11 patients were diagnosed with malignant ciliary body medulloepithelioma by pathological evidence. In this study, 6 patients had enucleation (2 patients had systemic chemotherapy after surgery), and the other 5 patients had local tumor resection (1 patient had plaque radiotherapy after surgery). CONCLUSION: Ciliary body medulloepithelioma is a rare intraocular tumor and may be easily confused with retinoblastoma. Analyzing the clinical and pathological features of ciliary body medulloepithelioma is useful to further understand ciliary body medulloepithelioma, and can make an accurate diagnosis and better treatment.

The development and the genetic diseases of the ciliary body

“…Medulloepithelioma of the ciliary body originates from the nonpigmented ciliary epithelium, and primarily develops in children ( He et al., 2023 ). Ciliary body tumours are pinkish-white and may appear as chalky calcified opacities when located in the ciliary body through slit-lamp microscopic examination ( Rehman et al., 2021 ).…”

Uveitis masquerade syndromes: An approach to diagnosis

“…There is often a delay in establishing the diagnosis as there may be free-floating tumour cells in the anterior chamber with aqueous flare, posterior synechiae, cataract as well as vitreous opacities on B-scan ultrasonography, which add to diagnostic confusion or impair visualisation of the tumour. 58,59 The diagnosis is usually made by clinical or ultrasonographic identification of the mass, and then biopsy. 57 The presence of multiple cysts within a ciliary body mass along with a retrolental neoplastic cyclitic membrane on ultrasound biomicrosopy may offer useful diagnostic clues.…”

“…Patients usually present with leucokoria, loss of vision or pain and have a ciliary body mass often with associated neovascularization of the iris, cataract and cyclitic membrane. There is often a delay in establishing the diagnosis as there may be free‐floating tumour cells in the anterior chamber with aqueous flare, posterior synechiae, cataract as well as vitreous opacities on B‐scan ultrasonography, which add to diagnostic confusion or impair visualisation of the tumour 58,59 . The diagnosis is usually made by clinical or ultrasonographic identification of the mass, and then biopsy 57 .…”