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A 65 year old woman with visual changes
Digital Journal of Ophthalmology 2005
Volume 11, Number 14
July 28, 2005
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Burton Goldstein, M.D. | University of South Floida Eye Institute
David Eichenbaum | Ophthalmic Consultants of Boston/Tufts New England Eye Center
Diagnosis and Discussion
Diagnosis and Discussion

“Carcinoid” is a term used to describe any type of tumor arising from the diffuse neuroendocrine cells of the amine precursor uptake and decarboxylation system, and it comprises less than 1% of all pulmonary and colorectal cancers . It is typically slow to metastasize and is of relatively low malignant potential .

The presenting symptom in our patient was new photopsias. Although the most common presentation in choroidal metastases is painless loss of vision, several other presenting complaints including floaters, field defects, and ocular pain have been documented5. The appearance of the tumor, a dome-shaped mass with an orange color, was characteristic for carcinoid. This color is unique in contrast to the creamy-yellow hue found in most uveal metastases7. The lesion was a solitary monocular metastasis, the most typical presentation of metastatic choroidal carcinoid.

Our diagnosis of metastatic carcinoid was made without a choroidal biopsy. In cases where the clinical appearance of the intraocular tumor, ultrasonography, fluorescein angiography, and a history of histopathologically confirmed systemic carcinoid tumor are strongly suggestive of the diagnosis, biopsy has been obviated7. In addition, our work-up of the patient revealed the characteristic, yet non-specific fluorescein angiography pattern typical for metastases , which is prominent hyperfluoresence in late phases of the angiogram, consistent with the prominent vascularity of metastases. Ultrasonographic findings of intermediate reflectivity were consistent with metastatic neoplasms .

In a recent review of reported cases of carcinoid metastatic to the uvea, Fan and associates found that the bronchus was the site of the primary tumor in 24 of 28 cases. Although the gastrointestinal tract is the location for over 90% of carcinoid tumors diagnosed, those that metastasize to the choroid are most frequently found in the bronchus7,8, whereas gastrointestinal carcinoid tumors most frequently metastasize to the orbit . The interval between detection of the primary carcinoid and the diagnosis of uveal metastases has been reported as being between 0 and 9 years with an average of 4.1 years .

Treatment for metastatic choroidal carcinoid has not been standardized. Due to the rarity of this diagnosis and the interval between the detection of new cases, a powerful prospective therapeutic trial would be very difficult to produce. For decades, the most commonly employed therapy for patients with systemic malignancies metastatic to the choroid has been external beam radiation therapy, ranging between 20Gy to 50Gy fractionated , , . The assumption that radiation would effectively treat ocular carcinoid may be flawed, however, since external beam radiation has incomplete success for primary carcinoid tumors , . Despite the literature that carcinoid tumors are generally radioresistant, there are reports from groups that have observed these tumors effectively treated by both external beam radiation and plaque brachytherapy7, . There is an equal paucity of information regarding metastatic ocular carcinoid response to chemotherapy; however, there is one case report of cisplatin and etoposide being effective treatment . Our patient had a remarkably good response to external beam radiation treatment of her primary bronchogenic carcinoid. This result early in her initial course suggested that she would achieve the observed, good response of her choroidal lesion following palliative radiation. Although radiation treatment of carcinoid metastatic to the choroid has mixed results in the literature, we are encouraged by our patient’s tumor reduction after treatment.

Early detection and treatment of ocular carcinoid is important due to the comparatively favorable prognosis of patients with carcinoid metastatic to the choroid. Orbital and intraocular metastases are generally associated with a grave prognosis, and the interval between surgical intervention and death is usually measured in months4. However, there are several cases of choroidal carcinoid with prolonged survival, and the prognosis for patients with this rare choroidal metastasis can be measured in years. The indolent course of systemic carcinoid and extended survival observed in some cases of ocular carcinoid justify aggressive intervention to improve prognosis. Harbour et al has observed a mean survival of 34 months from ocular diagnosis7, and Fan et al observed 4 study patients surviving over 7 years after choroidal metastases were diagnosed . Such encouraging reports, the documented radioresponsiveness of our patient’s specific tumor, and the rapid and remarkable response of the ocular metastasis we have observed allow us an above-average prognosis in this case.