Choroidal Metastases

From EyeWiki



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.

Breast cancer is the most common cancer to metastasize to the choroid (47% total in men and women combined). In a study of 264 patients with uveal metastases from breast cancer, 62% of patients had unilateral metastasis at presentation. Most (99%) metastases were yellow in color, 77% had a plateau configuration, 89% were found in the posterior pole, and 64% showed related subretinal fluid. Patients with breast cancer metastatic to the uvea show survival rates of 65% at one year, 35% at three years, and 24% at 5 years.


Physical examination

Choroidal metastases are typically yellow in color (in 95% of lesions), have a plateau configuration, and are associated with subretinal fluid. Choroidal metastases are anatomically located between the macula and equator (80%), within the confines of the macula (12%) and anterior to the equator (8%). The mean tumor thickness is 3mm, but some solitary tumors can be much larger.

Although lesions are yellow in color in most cases, lesions may be orange (from renal cell, carcinoid, and thyroid) or brown (from skin melanoma).


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 (11%).


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 [6].

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 [7].

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 [8]. Color flow mapping shows that choroidal metastases tend to have hyper-vascularity, lack a ‘dominant vessel’ and typically havea ‘peripheral pattern’ blood flow [9].

Fine needle aspiration biopsy can be used to confirm the diagnosis of metastasis and aid in identifying the primary cancer. There are few risks associated with FNAB. In a study of 159 FNABs for suspected intraocular tumors, the most common complication was subretinal hemmorhage in 13% of patients. There were no cases of retinal detachments or tumor recurrence at the biopsy site or orbital recurrence. Adequate cytologic material was obtained in 88% of cases, with a diagnostic sensitivity of 100% and specificity of 98%.


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

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.

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 as the average life expectancy for these patients is less than one year.

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.

Bevacizumab, a potent monoclonal antibody that has also been employed for the treatment of ocular vaso-proliferative diseases, has been used in the treatment of choroidal metastases and has shown promising results [10].


Additional Resources


References

1. Shields CL, Shields JA, Gross N, Schwartz G, Lally S. Survey of 520 eyes with uveal metastases. Ophthalmology, 1997;104:1265-76.

2. 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.

3. 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.

4. 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.

5. BCSC Section 4: Ophthalmic Pathology and Intraocular Tumors. Chapter 20. 2010-2011.

6. 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.

7. 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.

8. 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.

9. 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.

10. Giuliari GP, Sadaka A. Uveal metastatic disease: Current and new treatment options (review). Oncol Rep. 2012 Mar;27(3):603-7.
  1. 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.