PMC7680916_01
Enhanced CT (A-C) and MRI (D-F) imaging. Significant abnormal findings of left kidney and tumor thrombus of vena cava were noted (arrow).
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August 2018. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) scan detected a mass in his left kidney, with Mayo level IV inferior vena cava tumor thrombus ( Figure 1 ). A grayish-yellow mass with 11 cm x 7 cm in size with papillary arrangement, irregular nucleus, and large nucleoli and renal venous tumor thrombus formation was identified ( Figure 1 ). Histology showed was high-grade RCC, papillary type with vena cava tumor thrombus ( Supplementary Figure 1 ). IHC indicated that PD-L1 Tumor Proportion Score (TPS) was 25% ( Supplementary Figure 1 ). His tumor sections were stained with anti-FH and anti-2-succinocysteine (2SC) antibodies to confirm the diagnosis ( Supplementary Figure 1 ). RCC in the first line setting, subsequent treatment with this was given from June 2019 but dosing of pembrolizumab was 100 mg every 3 weeks due to economic considerations. Response evaluation after one dose of pembrolizumab showed a partial response, with shrinkage of hepatic and right adrenal lesions, and response persisted for 5 months ( Supplementary Figure 1 ).
(A, B) Bilateral ultrasound images showing posterior retinal detachment in both eyes. (C, D) Fundus photographs showing large areas of depigmentation in retina. (E-H) Fluorescein fundus angiography (FFA) reveals early hypofluorescence of left (E) and right (G) side and late hyperfluorescence of left (F) and right (H) side in the optic disc area. (I, J) Optical coherence tomography of the left (I) and right (J) eye shows pockets of subretinal fluid.
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B-ultrasound after 1 month confirmed progression of disease ( Supplementary Figure 2 ). Based on the results of KEYNOTE-426 which demonstrated axitinib in combination with pembrolizumab conferred a survival benefit for patients with advanced Large areas of depigmentation were observed in retina, and local pigmentation were noticed ( Figure 2 ).
Time course of images of the presentation in fundus examination (A, B) and optical coherence tomography (C, D) at 3-week follow-up to fundus examination (E, F) and optical coherence tomography (G, H) at 6-week follow-up. (I, J) Eye MRI shows bilateral retinal detachment of optic nerve.
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Abdominal enhanced CT in May 2019 suggested metastatic lesions in the liver and right adrenal gland ( Figure 3 ). Follow-ups of OCT is shown in  Figure 3 . Eye MRI at January 2020 also confirmed a thickening of posterior walls ( Figure 3 ). At follow-up in February 2020, progressive disease in his adrenal, hepatic and bones were observed ( Supplementary Figure 3 ).