Choroidal Metastases
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Disease Entity
The uvea is the most common site for ocular metastasis. Within the uvea, 88% of metastases are to the choroid, followed by metastases to the iris (9%) and ciliary body (2%). This large difference is thought due to the distribution of blood supply, which heavily favors the choroid as compared to the iris or ciliary body.
Shields and associates published a comprehensive survey of uveal metastases in 520 eyes of 420 patients with a total of 950 uveal metastases. The most common primary cancer sites for uveal metastasis in males were lung (40%), gastrointestinal (9%), kidney (8%), and other. Rarely, serous retinal detachment is the first manifestation of aggressive malignancies, such as testicular cancer.[1] The primary site was unknown at the time of presentation in 29% of males. In females, the most common sites included breast (68%), lung (12%), and other (4%). The primary site was unknown at the time of presentation in 12% of females.
In 2018, Shields and associates published features of uveal metastases in 1,111 patients. The primary tumor originated in the breast (n = 416, 37%), lung (n = 295, 27%), kidney (n = 46, 4%), GI tract (n = 40, 4%), cutaneous melanoma (n = 27, 2%), lung carcinoid (n = 24, 2%), prostate (n = 23, 2%), thyroid (n = 15, 1%), pancreas (n = 8, 1%), and other sites (n = 40, 3%). The primary tumor site was unknown in 177 (16%). Regarding choroidal metastases (n = 1213), mean basal diameter was 9.5 mm and mean thickness was 3.2 mm, and tumor color was yellow (86%), orange (8%), or brown (4%), subretinal fluid was present (72%), and ultrasound confirmed echodensity (80%). Overall, the primary cancer was detected before (67%) or after (18%) the uveal tumor. Those with predominant detection before uveal metastasis include breast (94%), kidney (96%), GI tract (85%), cutaneous melanoma (96%), and thyroid (93%). Those with late detection after the uveal metastasis include primaries of lung carcinoma (47%), lung carcinoid (33%), and pancreatic cancer (37%). Overall Kaplan–Meier survival was 57% at 1 year, 42% at 2 years, 32% at 3 years, 29% at 4 years, and 24% at 5 years, with mean survival time of 17.2 months. At 5 years, survival with primary tumor in the breast (25%), prostate (31%), and cutaneous melanoma (33%) was better than lung (13%), GI tract (13%), and thyroid (12%), whereas lung carcinoid displayed the most favorable 5-year survival (92%). By comparison, relative to breast cancer, 5-year survival was significantly worse with lung cancer (P < 0.001), GI cancer (P = 0.002), and pancreatic cancer (P = 0.005), and significantly better with lung carcinoid (P = 0.005).
Diagnosis
Physical examination
Choroidal metastases are typically yellow in color, have a plateau configuration, and are associated with subretinal fluid. They can be solitary and unilateral or multiple and bilateral. Ultrasound can play a key role in making the diagnosis of a large choroidal metastasis as the internal reflectivity is variable (high and intermediate) compared to melanoma in which the internal reflectivity is low.
Symptoms
Patients with choroidal metastases present to ophthalmologists with visual symptoms almost in 90% of cases. Asymptomatic metastases are commonly detected in the fellow eye. Patients most often present with blurred vision (70%), flashes and floaters (12%), and pain (7%). It is not uncommon for metastases to be found in asymptomatic patients.
Diagnostic procedures
Indocyanine green angiography shows a blockage of the background staining and a patchy staining of the tumor surface. Intratumoral vessels could not be detected using ICG-A [2].
Optical coherence tomography may reveal a pattern of hyperintense irregular spots in the context of the photoreceptor layer and in the retinal pigment epithelium, subretinal fluid, and marked irregularity of the retinal pigment epithelium with thickening and gross undulation [3].
Ultrasound, fine needle aspiration biopsy (FNAB) and fundus autofluorescence are all used to aid in the diagnosis of choroidal metastases.
Ultrasound can be very useful in diagnosis. A-scan will typically show moderate to high internal reflectivity. B-scan will identify an echogenic choroidal mass and may show secondary retinal detachment. Choroidal metastases are characterized by a significantly lower height-to-base ratio than melanomas, whereas reflectivity is significantly higher in metastases [4]. Color flow mapping shows that choroidal metastases tend to have hyper-vascularity, lack a ‘dominant vessel’ and typically havea ‘peripheral pattern’ blood flow [5].
Differential diagnosis
- Choroidal Amelanotic Melanoma
- Choroidal Amelanotic Nevus
- Posterior Scleritis
- Choroidal Hemangioma
- Choroidal Granuloma
- Choroidal Osteoma
- Posterior Uveal Effusion Syndrome
- VKH
- Central Serous Retinopathy
- Infectious lesions
- Organized subretinal hemorrhage
- Solitary idiopathic choroiditis
Management
Regarding treatment of choroidal metastases, it is important to first assess the patient’s systemic status. This can be done with a thorough history, physical exam, laboratory evaluation, and imaging studies.
It is also important to assess the condition of the opposite eye and examine for multifocality of tumors.
General treatment
Treatment for choroidal metastases include systemic chemotherapy and/or hormonal therapy as well as plaque radiotherapy, external beam radiation therapy (EBRT), and/or photodynamic therapy.
Treatment involves a collaborative effort between the ocular oncologist, radiation oncologist, and medical oncologist. Sometimes, it can be difficult to convince the medical oncologist that the patient has metastatic disease. If the patient does not have known metastatic disease then a PET CT is often warranted to evaluate for metastasis elsewhere in the body. If no other lesions are found then a biopsy may be requested from the oncologist. If other metastases are found then sometimes chemotherapy alone can treat the choroidal metastasis with complete resolution.
Plaque radiotherapy is usually reserved for solitary metastases. This modality offers precise, controlled radiation delivery to the eye. Furthermore, it is a quick treatment modality, requiring only 3 to 4 days of treatment, as compared with EBRT, which may require 3 to 4 weeks of therapy. Treatment duration is an important consideration in uveal metastases and must be weighed with life expectancy.
Several considerations should be taken into account when designing plaque radiotherapy and radiation exposure time. These include the size and thickness of the metastasis, the distance of the lesion from the optic nerve, and the distance of the lesion from the foveola. Complications from plaque radiotherapy are similar to those of external beam and other radiation therapies, including radiation retinopathy, papillopathy, and cataract. These side effects are uncommon, especially given the short life expectancy of many patients.
External beam radiation works well for bilateral and multifocal disease and can diminish the exudative detachment often associated with these lesions and thereby improve vision.
Photodynamic therapy (PDT) can also be used to treat small metastases in the posterior choroid. When associated with SRF and decreased vision, PDT often improves vision after treatment.
References
- ↑ Phelps, P. O., et al. "Metastatic mixed germ-cell neoplasm presenting as retinal detachment." Journal of clinical oncology: official journal of the American Society of Clinical Oncology 30.13 (2012): e133.
- ↑ Krause L, Bechrakis NE, Kreusel KM, Servetopoulou F, Heinrich S, Foerster MH. Indocyanine green angiography in choroid metastases. Ophthalmologe. 2002 Aug;99(8):617-9.
- ↑ Iuliano L, Scotti F, Gagliardi M, Bianchi I, Pierro L. SD-OCT Patterns of the Different Stages of Choroidal Metastases. Ophthalmic Surgery, Lasers and Imaging Retina.2012;43:E30-E34.
- ↑ Sobottka B, Schlote T, Krumpaszky HG, Kreissig I. Choroidal metastases and choroidal melanomas: comparison of ultrasonographic findings. British Journal of Ophthalmology.03/1998;82(2):159-61.
- ↑ Neudorfer M, Waisbourd M, Anteby I, Liran A, Goldenberg D, Barak A, Kessler A. Color flow mapping: a non-invasive tool for characterizing and differentiating between uveal melanomas and choroidal metastases. Oncology reports 2011;25;91-96.
- Shields CL, Shields JA, Gross N, Schwartz G, Lally S. Survey of 520 eyes with uveal metastases. Ophthalmology, 1997;104:1265-76.
- Shields CL, Shields JA et al. Uveal Metastasis: Clinical Features and Survival Outcome of 2214 Tumors in 1111 Patients Based on Primary Tumor Origin. Middle East African Journal of Ophthalmology 2018 Apr-Jun;25(2):81-90.
- Demirci H, Shields CL, Chao A-N, Shields JA. Uveal Metastasis from Breast Cancer in 264 Patients. American Journal of Ophthalmology 2003;136,2:264-71.
- Shields JA, Shields CL, Ehya H, Eagle RC, Jr, DePotter P. Fine needle aspiration biopsy of suspected intraocular tumors. The 1992 Urwick Lecture. Ophthalmology 1993;100:1677-1684.
- Shields CL, Shields JA, De Potter P, Quarante M, Friere J, Brady LW, Barrett J. Plaque radiotherapy for the management of uveal metastasis. Arch Ophthalmology 1997;115:203-9.
- BCSC Section 4: Ophthalmic Pathology and Intraocular Tumors. Chapter 20. 2010-2011.
