Introduction
Uveal melanoma accounts for 3-5% of all melanomas in our body and is the most common intraocular neoplasm. It arises from melanocytes of the iris (3–5%), ciliary body (5–8%) and choroid (85–90%) [1–3].
It most often affects patients in the 6th-8th decade of life. The prognosis depends on the tumor’s location, size, and histological subtype. Uveal melanomas are usually single, one-sided tumors [1–3].
In this paper, we present a rare case of cooccurrence of ciliary body melanoma and choroidal tumor in one eye and a choroidal tumor in the other eye.
Case report
An 82-year-old patient reported to the ophthalmology department in October 2017 complaining of a limited visual field in his left eye. Visual acuity with spectacle correction in the right eye was 0.9, and in the left eye, 0.4. Intraocular pressure was 18 mmHg in each eye.
Indirect ophthalmoscopy in the left eye revealed a choroidal tumor peripherally from the nose. An incipient cataract was found in the right eye. Ultrasonography (USG) of the eyeballs was performed, which confirmed a limited choroidal lesion with high internal echogenicity in the left eye. The patient was referred to the Ophthalmic Oncology Department with a suspicion of uveal melanoma to expand the diagnosis and implement an appropriate form of therapy.
The above diagnosis was confirmed. One month later, the patient underwent a 125I brachytherapy procedure. In the following months, he remained under our observation.
In February 2018, due to the features of radiation retinopathy with elevated intraocular pressure (left eye = 27.0 mm Hg), panretinal laser coagulation of the left eye was implemented. During several months of observation, features of tumor regression were noted on ultrasound examination.
Due to the deteriorating visual acuity of the right eye (0.4), the patient was referred for cataract surgery. In November 2018. phacoemulsification surgery was performed. Visual acuity in the right eye at discharge was 0.7 and light perception in the left eye.
During a follow-up visit a month later, a tumor was observed to be spreading beyond the left eyeball with evidence of metastatic deposits observed on the corneal endothelium (Fig. 1). The patient was referred to the Ophthalmic Oncology Center for urgent consultation to exclude local recurrence.
After the consultation, the patient was qualified for left eyeball enucleation surgery. This procedure was performed in January 2020. The histopathological examination revealed a mixed type of melanoma tumor of the choroid and ciliary body of the left eye. The patient remained under our observation.
Six months later, the visual acuity in the right eye was 0.8. Intraocular pressure was 9 mm Hg. The anterior and posterior segments of the right eye were within normal limits for his age norm. Topical levofloxacin was prescribed 4 times daily to the left orbit to manage a persistent purulent discharge secondary to the enucleation.
Meanwhile, the oncologist’s check-up revealed numerous metastases to the liver, presenting a guarded prognosis without any further treatment options.
In January 2021, the patient reported to our Ophthalmology Clinic with a significant deterioration of visual acuity in the right eye (0.2 cc +1.5 dsph); intraocular pressure was 8 mm Hg. Indirect ophthalmoscopy revealed a choroidal tumor with subretinal fluid. We performed swept source-optical coherence tomography (SS-OCT), which confirmed the presence of a limited, domed choroidal tumor with subretinal neurosensory fluid (Fig. 2). An ultrasound scan showed a tumor with high internal echogenicity within the macula (Fig. 3). The patient was referred to the Ophthalmic Oncology Department for urgent consultation. Even before the scheduled appointment, the patient died.
Discussion
Our case report concerns bilateral uveal melanoma which occurs with frequency of about 0.18%.
Bilateral uveal melanoma presented by us is rare and occurs with a frequency of about 0.18% [4]. Although histopathological diagnosis for the left eye tumor is not available to confirm the diagnosis of a melanoma, the ultrasound studies support this diagnosis. No predisposition to its occurrence has been found in terms of gender, race, mean age at diagnosis, location of the lesion, or presence of distant metastases [4].
Bilateral localization may suggest a genetic predisposition. BAP1 (BRCA-associated protein 1) tumor predisposing syndrome mutation has been identified recently as germline cancer syndrome, which can lead to several cancers, including bilateral uveal melanoma, and follows an autosomal-dominant inheritance pattern [5, 6]. According to Rai et al., analysis of 174 cases of patients with germline BAP1 mutation revealed the most common tumours to be uveal melanoma (31%), mesothelioma (22%), cutaneous melanoma (13%), renal cell carcinoma (10%), breast carcinoma (10%), atypical Spitz tumour (18%) and others [6, 7].
The infiltration of the optic nerve promotes the formation of distant metastases. They are found to occur in 15% at 5 years, 25% at 10 years, and 36% at 20 years [6]. The most common location for them is the liver, affecting 40–60% of patients. In addition, metastases occur in the lungs, bones, skin, and brain [8–11].
The average survival after diagnosis of liver metastasis does not exceed 2–11 months [12–15]. For single metastases, surgical treatment is used, while the primary treatment for multiple metastatic foci is chemotherapy with the use of dacarbazine [16].
The differential diagnosis of choroidal tumors includes primarily choroidal pigmented nevi, age-related macular degeneration, choroidal hemangioma, hemorrhagic choroidal detachment, vortex veins distension, subretinal hemorrhage, retinal pigment epithelial hyperplasia, choroidal ossification, toxoplasmic choroiditis, melanocytoma, and retinal astrocytoma [17]. Ultrasonography is extremely helpful in diagnosing and monitoring clinical changes of ocular tumors as the internal reflectivity or the presence of the so-called choroidal incision is pathognomonic, as discussed in the SOCT study [17].
The location of bilateral choroidal melanoma should always be taken into account. It becomes crucial to carefully and regularly examine both eyes when uveal melanoma is found in one eye. The examination result may determine the type of treatment undertaken and, thus, the prognosis for long-term survival. Shields et al. also suggest that all patients with bilateral uveal melanoma and those with history of mesothelioma, cutaneous melanoma, renal cell carcinoma and other cancers should be tested for germline BAP1 tumor predisposition syndrome. Patients identified as positive should have relatives tested and should maintain routine screening for related cancers [6].
Conflict of interest
No conflict of interest.
Funding
No financial support.