Retinoblastoma in an older child with secondary glaucoma as the first clinical presenting symptom: A case report

Abstract

Background: Retinoblastoma (Rb) is primarily found in infants or young children. The most common primary presenting sign of Rb is leukocoria. Rb is very rare in children who are 10 years old or older. Timely and correct diagnosis as well as proper treatment are the key factors affecting the prognosis of Rb.

Case summary: A 10-year-old girl with symptoms of vision loss, redness, swelling and pain in the right eye for 2 mo was admitted to our Department of Ophthalmology. The visual acuity of the right eye was graded as hand movement. The intraocular pressure of the eye was 46.9 mmHg. No substantial space-occupying lesion or characteristic calcified plaque was found in the eye. The patient underwent anterior chamber irrigation under general anesthesia on the same day of admission, and 2 mL of irrigation solution was saved for pathological examination. Histopathological examination of the anterior chamber fluid revealed cancer cells. A diagnosis of Rb with masquerade syndrome was made. The patient underwent enucleation followed by 6 rounds of systematic chemotherapy. A follow-up examination almost 9 years later found no relapse of Rb.

Conclusion: For older pediatric patients who have secondary glaucoma and uveitis symptoms without a clear cause of the disease and have no space-occupying lesion found by imaging examination, aqueous humor or vitreous humor examination is recommended for timely and correct diagnosis and appropriate treatment.

Keywords: Case report; Masquerade syndrome; Older children; Retinoblastoma; Secondary glaucoma; Uveitis.

Figure 1

Image of the anterior chamber in the right eye on Day 1 after hospitalization. The arrow shows corneal edema with haze. The depth of the anterior chamber was normal. Mutton-fat KP: ++ (arrow); anterior chamber cells: +++ (asterisk).

Figure 2

Color image of the fundus of the right eye. The vitreous body was opaque, the C/D ratio of the optic disk was 0.55, retinal edema with bluish color, and the superior temporal retina was pale with occluded, line-like vessels.

Figure 3

B-ultrasound image of the right eye. The images show the opacity of the vitreous body (arrow) and retinal edema (asterisk).

Figure 4

Image of ultrasound biomicroscopy examination of the right eye. The result suggested multiple ciliary body cysts (arrow).

Figure 5

Computerized tomography image from axial view and coronal view. No intraocular, space-occupying lesions were found.

Figure 6

Image of the anterior segment of the right eye on Day 1 after the anterior chamber irrigation procedure. The cornea was clear. The depth of the anterior chamber was normal. KP: +; AR: +.

Figure 7

Color image of the fundus of the right eye after the anterior chamber irrigation procedure. The image showed vitreous opacity, retinal edema with bluish color, and sporadic bleeding in the macular and subretinal area.

Figure 8

H&E staining of the enucleated eye. Arrow: Tumor mass.