1. A
Figure 1. (A) Left, RetCam fundus photograph of child 1 showing a right multilobulated tumor with overlying serous retinal detachment (RD) and subretinal seeding. Middle left, Histopathological section under low magnification through the optic nerve demonstrating extra-scleral and post-lamina cribrosa invasion (arrows), but not to the optic nerve resection margin (pT4). Middle right, High magnification of trans-scleral and extra-scleral invasion (arrow). Right, High magnification showing retrolaminar invasion (arrow). Whole-body MRI (WBMRI), lumbar puncture (LP) and bilateral bone marrow aspirate (BMA) were negative for malignancy. He underwent six cycles of vincristine, etoposide, carboplatin (VEC) and cyclophosphamide, followed by orbital irradiation.
(B) Left, RetCam fundus photograph of child 2 demonstrating a large inferior tumor with overlying RD, vitreous and subretinal seeds. The optic nerve was obscured. Middle and right, Histopathological sections under low and intermediate magnification showing massive choroidal invasion (asterisk) beyond the confines of the retinal pigment epithelium (arrow), with no evidence of scleral invasion (pT3a). There was prelaminar optic nerve invasion. LP and BMA were negative for malignancy and she received four cycles of VEC.
(C) Left, RetCam fundus image of child 3 demonstrating a large tumor with associated RD, subretinal and focal vitreous seeding. There was no visualization of the optic nerve and the macula was involved. Initial review was consistent with low-risk histopathology. One year later the child presented with fever and pain, and WBMRI identified a paraspinal tumor. Molecular analysis confirmed metastasis (Racher 2016). Bilateral BMA were involved with tumor cells. MRI showed no orbital or intracranial disease and LP was negative. Internal review of the ocular pathology, including further choroidal sections, showed an area of massive choroidal invasion (pT3a). The child received 6 cycles of VEC and cyclosporine, followed by autologous bone marrow transplant and focal irradiation. He was diagnosed with dural-based metastases 1 year later. Despite radiotherapy, the child died 18 months after presentation with metastases.
(D) Left, RetCam fundus photograph of child 4 demonstrating a large tumor obscuring the nerve and macula, with associated hemorrhage, RD and diffuse vitreous seeding. There was no anterior segment extension evident on ultrasound biomicroscopy. Histopathology review initially demonstrated 2.2 mm of choroidal invasion, but was confirmed to be massive following external review (pT3a). The child received systemic adjuvant chemotherapy. B * C C D