If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
Correspondence to: Chia-Kai Chu, MD, Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch, 700 University Blvd, Galveston, TX 77550;
Carcinoid tumours are derived from highly differentiated neuroendocrine cells that often produce biogenic amines or polypeptide hormones. They arise from various primary sites along the gastrointestinal, bronchopulmonary, and urogenital tracts and metastasize to the lymph node, liver, or bone, and only rarely to the eye and orbit. Uveal metastasis is the most common intraocular malignancy and mostly arises from breast cancer in women and lung cancer in men. Uveal metastasis from carcinoid tumours constitutes only 2.2% of all uveal metastasis.
Treatment of uveal metastases has varied based on individual case and has included external beam radiotherapy, plaque radiotherapy, proton beam irradiation, argon laser photocoagulation, and local resection.
Case Report
A 28-year-old male presented with photopsias and micropsia in his left eye for the past 2 months. His medical history was significant for well-differentiated carcinoid tumour of the lung with metastasis to the brain, liver, spine, and eyes, as found on imaging. He was referred to ophthalmology for further examination.
Visual acuity was 20/20 OD and 20/30 OS. Fundus examination on the right showed multiple diffuse choroidal lesions in the periphery with a large choroidal infiltrate inferiorly (Fig. 1A). The left eye demonstrated macular thickening with 2 large choroidal infiltrates located superotemporally and superonasally, along with diffuse smaller choroidal infiltrates in the periphery (Fig. 1B). Intravenous fluorescein angiogram (IVFA) showed staining of the lesions with no vascular changes or leakage (Fig. 1C, D). Optical coherence tomography (OCT) revealed subretinal fluid in the left eye macula from the superotemporal lesion (Fig. 1F). B-scan of the left eye performed on the visually significant superotemporal lesion showed a high internal reflectivity measuring 10.07 mm in longer basal diameter and height of 4.45 mm. His findings were consistent with metastatic disease.
Fig. 1Findings at presentation. (A) Fundus photograph of the right eye with multiple deep diffuse choroidal infiltrates. (B) Fundus photograph of the left eye with multiple deep diffuse choroidal infiltrates with 2 large choroidal infiltrates, 1 superotemporally and the other superonasally. (C) Right eye and (D) left eye fluorescein angiogram demonstrating hyperfluorescence of the tumours in the late stage. (E) Right eye and (F) left eye optical coherence tomography macula showing subretinal fluid in the left eye.
Given that the patient was only symptomatic with regard to vision, he proceeded with palliative radiotherapy and deferred somatostatin analog therapy until there was evidence of systemic progression. Treatment was initiated to both globes using opposed lateral fields to a dose of 30 Gy in 12 fractions, below the threshold for radiation retinopathy, before his first eye examination.
Afterwards, the patient’s vision worsened to 20/200 with tumour enlargement and increased subretinal fluid. Given that there was no improvement with palliative radiotherapy, the patient agreed to undergo photodynamic therapy (PDT) of the superotemporal lesion of the left eye.
Standard-fluence PDT was conducted, and intravenous verteporfin (6 mg/m2) was infused over 10 minutes. Treatment parameters were 689-nm diode laser at an intensity of 600 mW/cm2 delivered at a 5.3-mm spot size for 83 seconds. After 2 months, his vision improved to 20/80 with regression of the subretinal fluid and lesion, now measuring 6.56 mm in longer diameter by 3.32 mm in height (Fig. 2). At 10 months, his vision improved to 20/60 with resolution of the subfoveal fluid on OCT (Fig. 3). The patient’s general condition remained stable.
Fig. 2Findings before and after photodynamic therapy (PDT). (A) Fundus photograph of the left eye before PDT and (B) 2 months after PDT demonstrating reduction in size. (C) Optical coherence tomography macula of the left eye before and (D) 2 months after PDT with reduction in subretinal fluid.
Fig. 3Findings 10 months after photodynamic therapy. Optical coherence tomography of the left eye demonstrates further improvement of subretinal fluid and resolution of subfoveal fluid.
PDT mediates tumour destruction through multiple mechanisms: direct destruction of tumour cells, damage to endothelial cells leading to tumour infarction, and activation of immune response against tumour cells.
PDT also avoids longer-duration treatment courses, hospitalization, interventional surgery, radiation retinopathy, and systemic effects from chemotherapy. PDT was performed in 8 patients with choroidal metastasis and provided excellent control of the tumour with stabilization or improvement of visual acuity.
However, it is unclear which tumour characteristics are amenable to PDT. To our knowledge, there are only 2 published cases of choroidal metastasis by a carcinoid tumour treated with PDT.
In the first case, reported by Harbour, the carcinoid tumour was primarily from the lung and demonstrated marked intratumoural vascularity on IVFA; thus, PDT was elected after failing chemotherapy and radiation treatments.
The tumour regressed by 33% in basal area and 25% in height, the exudative retinal detachment resolved, and visual acuity improved from 20/60 to 20/40. In our case, a similar pulmonary carcinoid tumour of comparable height (4.45 mm vs 4.8 mm) demonstrated staining on IVFA but no intratumoural vascularity. Nonetheless, the tumour responded favourably to PDT after failing radiotherapy with a comparable reduction as reported, with a 35% decrease in basal diameter and 25% in height.
In the second case, reported by Kawakami et al., the carcinoid was primarily from the breast or lung. IVFA demonstrated staining of the tumour with no intratumoural vascularity. The tumour was treated with PDT without prior treatment with chemotherapy or radiation therapy. There was a marked regression in tumour height, from 3.5 mm to unmeasurable 14 months after PDT. The patient’s vision was reduced from 20/80 to 20/125.
It is possible that, in our patient, PDT was not as effective in reducing tumour height because of tumour thickness.
In conclusion, PDT is a good treatment option for choroidal metastatic carcinoid tumours. It leads to reduction of tumour size, resolution of subretinal fluid caused by the tumour, and marked improvement in vision. In addition, it seems to work as a primary treatment modality or after failed radiation alone or failed radiation and systemic chemotherapy. Furthermore, it works on choroidal metastatic carcinoid tumours whether the tumour demonstrates intratumoural vascularity or not on IVFA. However, thicker tumours have a favourable response that may be limited by thickness. Further clinical trials with a larger sample size might be helpful to validate the efficacy of PDT in treatment of choroidal metastatic carcinoid tumours.
Disclosure
The authors have no proprietary or commercial interest in any materials discussed in this article.
References
Harbour J.W.
De Potter P.
Shields C.L.
Shields J.A.
Uveal metastasis from carcinoid tumor: clinical observations in nine cases.
30584-7&id=gr1.jpg){kind=link}
30584-7&id=gr2.jpg){kind=link}
30584-7&id=gr3.jpg){kind=link}