A case of presumed choroidal metastasis from carcinoid tumor treated by photodynamic therapy with verteporfin
A 75-year-old woman who noticed floaters in her left eye for 2 weeks was referred to Tokyo Medical University Hospital in September 2010. She had a history of abdominal surgery for colon cancer (well-differentiated adenocarcinoma) in 2007. At presentation, her best-corrected decimal visual acuity was 0.6 oculus dexter (OD) and 0.9 oculus sinister (OS). Fundus examination revealed an orange-yellow mass that was 8 disc diameter (DD) in size with no measurable height at the inferotemporal side of the fundus OD, and a yellow-white mass 12 DD in size and 4.8 mm in height at the superior side of the fundus OS ( and ). Fluorescein angiography of the left eye showed hyperfluorescence inside the mass from early to late phase ( and ). Serum levels of carcinoembryonic antigen, cancer antigen 19-9, and cancer antigen 125 were within normal limits. A gallium-67 citrate scintigraphy scan showed no obvious abnormal uptake in the whole body including the eyes. Single-photon emission computed tomography with N-isopropyl-p-[123I] iodoamphetamine, which is known to be useful for the diagnosis of uveal melanoma, demonstrated no abnormal uptake in the eyes.\nIn September 2011, the masses at the inferotemporal side OD and at the superior and inferior sides OS had enlarged, but visual acuity was preserved in both eyes. On both T1 and T2-weighted magnetic resonance images of the left orbit, iso-dense regions relative to the cerebral white matter were found consistent with the masses. A systemic workup to search for extraocular primary lesions revealed multiple nodules in the subcutaneous tissues of bilateral breasts on ultrasonography and neoplastic lesions in the right bronchial tubes on chest computed tomography. Histopathology of the tumor from a breast biopsy revealed foci of oval and spindle-shaped cells in alveolar and palisading arrangement (), and that from a lung biopsy showed oval hypertrophic nuclei and proliferation of atypical epithelial cells in rosette-like arrangement. Immunohistochemical staining was positive for CD56, chromogranin A, and synaptophysin. These imaging and histological findings were consistent with breast and lung carcinoid tumors.\nFrom the clinical findings, general systemic workup, and clinical course, this case was presumptively diagnosed as choroidal metastasis from breast or lung carcinoid tumor. Fifteen months after the initial examination, the masses in both eyes expanded further. The mass at the inferotemporal side of the right eye was 20 DD in size and 3.5 mm in height. The mass at the superior side of left eye was 28 DD in size and 14.9 mm in height, and the mass at the inferior side was 7 DD in size and 5.5 mm in height. The left optic disc was invisible because of the enlarged mass, and best-corrected visual acuity was reduced to hand motion ( and ). An indocyanine green angiogram of the right eye showed choroidal vessel staining inside the mass from the early phase, and a mixture of hyper- and hypofluorescence in the late phase ( and ).\nExternal-beam radiotherapy of 40 Gy was used to treat the tumor in the left eye. Although the tumor in the right eye showed a tendency toward rapid expansion and required aggressive treatment to preserve vision, the tumor was still small and we had reservations about using external-beam radiotherapy due to the risk of adverse effects such as radiation retinopathy. Consequently, PDT was performed on her right eye after obtaining informed consent.\nStandard PDT was conducted with intravenous infusion of verteporfin at a dose of 6 mg/m2 body surface area over 10 minutes. Treatment parameters were 689 nm at 50 J (600 mW)/cm2 for an exposure time of 83 seconds. The diameter of each spot was 6,500 μm, and three spots with overlapping margin were administered. The tumor at the inferotemporal side was reduced to 7 DD and no measurable height after treatment. Later, a new mass 4 DD in size appeared at the inferonasal side and remained unchanged. The tumor at the superior side of the left eye was reduced after radiotherapy and the optic disc became visible ( and ). Best-corrected decimal visual acuity was 0.8 OD and 50 cm hand movement OS at 14 months after PDT (30 months after the initial visit) and the patient’s general condition was stable.