Choroidal amelanotic mass with vermiform margins, vision loss, and extreme fatigue.

Purpose: To describe a case of a previously healthy middle-aged male with an atypical choroidal mass and fatigue.

Methods: Case report.

Results: A 56-year-old Caucasian male presented with decreased vision in the right eye (OD) and a choroidal amelanotic mass OD with subretinal fluid. He received bevacizumab (1.25 mg/0.05 mL) injections by multiple providers without improvement in vision and with tumor progression. Visual acuity was 20/400 OD and 20/25 left eye (OS). The anterior segment was unremarkable in both eyes (OU). He was referred with suspected diagnosis of choroidal melanoma, metastasis, or osteoma. On examination, a choroidal amelanotic tumor with vermiform margins measuring 12 mm in base and 1.8 mm in thickness was seen OD. Smaller satellite lesions were noted inferiorly. Fundus OS was normal. Infectious evaluation was unrevealing and angiotensin-converting enzyme, lysozyme, and chest x-ray were normal. Fine-needle aspiration biopsy was declined by the patient. Given the clinical suspicion of choroidal sarcoidosis with characteristic tumor color, vermiform margins, and presence of satellite lesions, oral corticosteroids 60 mg/day for 2 months were started, followed by sub-Tenons triamcinolone acetonide (40mg/1 mL) injection three months later and urgent rheumatologic evaluation. He self-discontinued the oral corticosteroids and the tumor progressed by the next follow-up. Oral corticosteroids were restarted at 80mg/day. 16 months after his presentation to us, he experienced fatigue with electrocardiogram findings of third-degree heart block from sarcoidosis. He received an intracardiac defibrillator and 80 mg/day of corticosteroids with plans to initiate methotrexate for stronger immunosuppression.

Conclusion: Understanding the numerous manifestations of ocular and systemic sarcoidosis is crucial. Choroidal sarcoidosis characteristically exhibits vermiform margins. Close follow-up and systemic monitoring is necessary in patients presenting with ocular signs of sarcoidosis.

Precis: The diagnosis of choroidal sarcoidosis can be challenging. Features including vermiform tumor margins, satellite lesions, and choroidal infiltration on OCT are suggestive of choroidal sarcoidosis. All patients with ocular sarcoidosis should be monitored for systemic involvement. In this case, the ocular manifestation guided the diagnosis and eventually the treatment of cardiac sarcoidosis.