Table 3 Cases of paraneoplastic Acute Exudative Polymorphous Vitelliform Maculopathy (pAEPVM)
Article | ICI | Age (y), gender | Previous/concurrent/sequential therapy | Cancer | Symptoms | Initial BCVA | Time to onset | Antitumor efficacy of ICI | Other IRAE | Treatment | ICI discontinued | Ophthalmological outcome | Recurrence after rechallenge | Follow-up |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Lambert et al. (2021) [21] | Pembrolizumab | 54, F | NS | Metastatic vaginal mucosal melanoma | OU: blurry vision OS: yellow spot in the central visual field | OU 20/20 | 4 cycles | Partial remission | Immune-related thyroiditis, sarcoid-like syndrome, grade 1 pneumonitis | / | Y | Total resolution of the subretinal fluid, vitelliform deposits persisted | No rechallenge | 4 months |
Kemels et al. (2020) [22] | Nivolumab | 74, M | P: surgery S: Ipilimumab | Metastatic melanoma of the rectum | Visual loss | OD 20/25 OS 20/32 | 3 weeks | Metastatic progression | NS | PO CSa | Y | BCVA stable, mild decrease of subretinal fluid and stable subretinal material | No rechallenge | 4 months (died) |
Kemels et al. (2020) [22] | Nivolumab | 51, F | / | Metastatic vulvovaginal mucosal melanoma | Decreased vision and light flashes | OD 20/32 OS 20/20 | 1 month | Progression Clinical significant reduction of metastatic lesions on CT scan 4 m after re-administration | Sarcoid-like granulomatous reaction | SC + IVI CSb | Y, after 6 cycles | No improvement | Rechallenge after 2 months with surgical resection of primary tumor → significant reduction of the SRF OU 20/25 | 10 months |
Miyamoto et al. (2020) [23] | Nivolumab | 73, M | / | Malignant nasal melanoma | Metamorphopsia OU | OD 20/20 OS 20/16 | 2 months | NS | NS | / | N | Fundoscopy worsened | No discontinuation | 3 months, died 5 months after initial presentation |
Miyakubo et al. (2019) [24] | Ipilimumab | 78, M | P: surgery, chemotherapy; nivolumab x47 | Metastatic cutaneous melanoma | VF impairment | OU 16/20 | 2 cycles | NS | NS | / | N | SRF initially increased and then decreased VF, BCVA stable | No discontinuation | > 3 months |
Sandhu et al. (2019) [25] | Pembrolizumab | 55, F | P: Dabrafenib, Trametinib, ipilimumab C: Vemurafenib, Dabrafenib | Metastatic cutaneous melanoma | OU: blurred vision | Initially normal, OU 20/40 3 weeks after the introduction of vemurafenib | 5 days after starting Vemurafenib 3 months after starting Pembrolizumab | NS | NS | Difluprednate 4dd, dorzolamide 2dd | Vemurafenib discontinued | Gradual resolution of SRF OCT: normalization of the central retinal architecture with only a small foveal elevation above residual hyperreflective material OD 20/20 OS 20/25 | No discontinuation | 4 months (died 5 months after presentation) |
Lincoff et al. (2016) [26] | Ipilimumab | 65, M | / | Metastatic melanoma of gallbladder | Missing characters to the left of fixation for seconds at a time while reading | OD 20/25 OS 20/20 | AEPVM before introduction of Ipilimumab | No signs of recurrence | NS | Ipilimumab and surgery | N | Symptoms slowly improved | No discontinuation | > 1 year |
Crews et al. (2015) [27] | Ipilimumab | 46, M | Surgery, radiotherapy | Metastatic cutaneous melanoma | Blurred vision and photophobia | 20/100 OU | 3 cycles | NS | NS | Elevated liver transaminases | IV CSd | OD 20/60 OS 20/40 at 1 week follow-up OCT: OD resolution of SRF, OS improvement of SRF | Y | No rechallenge |
Mantopoulos et al. (2015) [28] | Ipilimumab | Early 70s, F | P: surgery | Acral lentiginous melanoma | OU: decreased vision, mild photophobia, ocular tenderness on palpation | OU 20/40 | 28 weeks | Progression | NS | Temozolomide and topical CS After 1 month: PO CSc | Y | OU 20/25 Resolution of SRF OCT: hyperreflective subretinal material | No rechallenge | > 6 months |