Cancer-associated retinopathy as an initial presentation of gynecologic small-cell carcinoma

Abstract

A 56-year-old female presented with bilateral progressive blurred vision over 1 month. She has no known malignancy before her initial visit to our ophthalmologic clinic. Her best-corrected visual acuity decreased to hand motion from 30 cm in both eyes. Optical coherence tomography exhibited parafoveal thinning of outer retinal layers bilaterally. Fluorescein angiography and indocyanine green angiography disclosed hypofluorescent spots in late phase in both eyes. The suspicion of cancer-associated retinopathy (CAR) prompted us to investigate and refer for further systemic disease including occult malignancy. The patient was diagnosed with small-cell carcinoma of the endometrium or cervix, which is an extremely rare and aggressive neuroendocrine tumor. The patient was treated with oral prednisone with improved visual acuity. The patient expired from sepsis 2 months after her initial visit to our ophthalmologic clinic. In selected cases, CAR may present before the diagnosis of primary cancer. It is essential to recognize its ophthalmic manifestation for early discovery of primary malignancy.

Keywords: Autoimmune retinopathy; cancer-associated retinopathy; gynecologic cancer; paraneoplastic syndrome; small-cell carcinoma.

Figure 1

Vitreous opacity, attenuation of retinal arteries, and pallor disc were noted at initial presentation on fundus photography in both eyes

Figure 2

Generalized thinning of outer nuclear layer and diminish of parafoveal photoreceptor layer showed on optical coherence tomography (OCT). Hyper-reflective dots were also noted in the outer retinal layer within the perifoveal region on OCT (yellow arrows), corresponding to the hypoautofluorescent spots observed in fundus autofluorescence (FAF). Prominent vitreous opacities were observed in the FAF. (a) OCT of the right eye. (b) OCT of the left eye. (c) FAF of the right eye. (d) FAF of the left eye

Figure 3

Fluorescein angiography (left) and indocyanine green angiography (right) showed hypofluorescent spots in late phase in both eyes. Note that there was moderate vitreous opacity. (a) Right eye. (b) Left eye

Figure 4

Abdominal and pelvic computerized tomography scan disclosed endometrial or cervical cancer with extensive invasion (red arrow) and complicated right obstruction uropathy (yellow arrow)

Figure 5

(a) Atypical hyperchromatic cells with salt-and-pepper chromatin infiltrating in desmoplastic stroma (yellow arrows). (b) Marked tumor necrosis was seen on the right lower part on this slide