Clinical features of patients with retinocytoma

Objective: To analyze the clinical features and fundus performance of patients with retinocytoma. Methods: Retrospective case series study. Ten patients (12 eyes) diagnosed with retinocytoma were enrolled in Department of Ophthalmology of Eye & ENT Hospital of Fudan University from January 2006 to December 2019. Clinical data were reviewed, including gender, age, initial symptoms, tumor size and location, fundus manifestations and regression patterns. Results: There were 7 males and 3 females aged from 5 to 39 years, with a median age of 14 years. Eight patients had unilateral retinocytoma, while 2 patients had bilateral retinocytoma. The most common complaints were strabismus (4 patients) and blurred vision (3 patients). The average maximum base diameter of the tumor was 5.70 mm (2.69 to 8.69 mm), and the average thickness was 2.28 mm (0.61 to 3.76 mm). Tumors in 12 eyes were all of a single focus, 5 in the posterior pole, 5 in the midperiphery and 2 in the periphery. Ophthalmoscopic features of retinocytoma included a translucent retinal mass in 10 eyes, a calcification mass in 11 eyes, with well-defined retinal pigment epithelial alteration in 12 eyes and a zone of chorioretinal atrophy in 6 eyes. The regression patterns were type 1 in 2 eyes, type 2 in 1 eye and type 3 in 9 eyes. The gene test showed RB1 mutation in 1 patient. During the average follow-up of 28.7 months, all of the tumors were stable without malignant transformation. Conclusions: Retinocytoma is a rare benign retinal tumor with characteristic fundus manifestations. Patients with retinocytoma should be monitored closely. (Chin J Ophthalmol, 2021, 57: 526-530).