Choroidal Melanoma


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  • Left eye ??Cotton wool spot versus metastases to retina appearing as white, noncohesive lesions distributed in a perivascular location.
  • Right eye 17 secs, 19 secs, 21 secs, 23.6 secs
  • Choroidal Malignant Melanoma
  • Choroidal tumor composed of bone, leakage of choroidal neovascularization(1/3) Choroidal Osteoma
  • Vascular tumor Choroidal Hemangioma
  • Scleral thickening, t sign Posterior scleritis
  • Kaplan-Meier survival estimates analyzed probability of death as a function of time from initial examination
  • 50% survival rate at 2 year follow up.
  • Stop adrenal androgen to estrogen
  • Left eye ??Cotton wool spot versus metastases to retina appearing as white, noncohesive lesions distributed in a perivascular location.
  • Choroidal Melanoma

    1. 1. Michele Todman, MD November 3, 2009 Grand Rounds
    2. 2. History <ul><li>CC: Sudden loss of inferior field of vision of my right eye </li></ul><ul><li>HPI: 57 y/o female </li></ul><ul><li>-2 day h/o inferior altitudinal field loss OD </li></ul><ul><li>- Noticed when closing OS to apply make-up </li></ul><ul><li>PMH: HTN </li></ul><ul><li>PSH: None </li></ul><ul><li>Meds: ASA, HCTZ </li></ul><ul><li>SH: 1PPD smoker x 30 years </li></ul><ul><li>FH: Mother died at 82 y/o of metastatic cancer, primary unknown </li></ul>
    3. 3. Examination <ul><li>VA: 20/50 OD; 20/20 OS </li></ul><ul><li>Pupils: Trace APD OD </li></ul><ul><li>Motility: Full OU </li></ul><ul><li>Confrontation VF: Inferior field defect OD; WNL OS </li></ul><ul><li>SLE: 1+NSC OU </li></ul>
    4. 4. Fundus Photography
    5. 5. Fundus Photography
    6. 6. FA 23 sec 34 sec 1:30 1:32 3.51 4.13
    7. 7. Ultrasound A-scan: Medium to high internal reflectivity B-scan: Acoustic solidarity
    8. 8. Differential Diagnosis?
    9. 9. A-scan: Low internal reflectivity with reduction in amplitude. High amplitude Spike = break in Bruch’s membrane. B-scan: Solid mass with mushroom shape
    10. 10. A-scan: High internal reflectivity B-scan: Highly reflective plate-like lesion with orbital shadowing beyond lesion FA can help distinguish if CNV
    11. 11. A-scan: High internal reflectivity B-scan: Solid elevated mass
    12. 12. B-scan: Thickening of the retina, choroid and sclera with fluid beneath Tenon’s capsule and squaring of optic nerve shadow
    13. 13. A-scan: Medium to high internal reflectivity B-scan: Acoustic solidarity
    14. 14. Working Diagnosis <ul><li>Is this choroidal mass with exudative detachment a primary lesion or a metastatic lesion to the choroid? </li></ul><ul><li>Need thorough systemic evaluation </li></ul><ul><ul><li>PCP contacted </li></ul></ul><ul><ul><li>1 wk prior pt presented with blurred vision OD & SOB </li></ul></ul><ul><ul><li>Found to have 13cm x 15cm left breast mass </li></ul></ul><ul><ul><li>Lab Work : Liver function tests wnl </li></ul></ul><ul><ul><li>CT Chest : Breast mass, lung nodules, liver lesions </li></ul></ul><ul><ul><li>Breast Biopsy : Infiltrating ductal carcinoma, ER/PR Negative </li></ul></ul><ul><ul><li>MRI brain : Frontal, occipital, and cerebellar lesions </li></ul></ul><ul><ul><li>PET Scan : Breast mass, lung, bone, adrenal, hepatic uptake </li></ul></ul>
    15. 15. <ul><li>Stage 4 Metastatic Breast Cancer </li></ul>
    16. 16. Incidence of ocular metastasis <ul><li>-1872: first description of a metastatic tumor to the eye in a patient with carcinoma </li></ul><ul><li>-Since then realization that ocular mets are not that rare </li></ul><ul><li>-Bloch and Gartner (1971) 10% patients with autopsy proven carcinoma had ocular mets </li></ul><ul><li>- Most common intraocular malignancy- choroidal metastasis </li></ul><ul><li>-Hard to gauge exact incidence as patients often are asymptomatic or present with advanced CA and eye exam never performed </li></ul>Demirci, Shields C, et al. Uveal Metastasis from breast Cancer in 264 Patients. Ophthalmology 2003 264-271 Shields C, Shields J, et al. Survey of 520 Eyes with Uveal Metastases. Ophthalmology 1997 1265-1273
    17. 17. Epidemiology <ul><li>For American women lifetime risk breast cancer- one in eight (12.5%) & lifetime risk death from breast cancer is 3.4% </li></ul><ul><li>Breast Cancer is the most common malignancy to metastasize to the uvea </li></ul><ul><li>Mean age at ocular diagnosis is 58 years, most cases (40-70 yrs) </li></ul><ul><li>At time of ocular diagnosis 66% reported a history of a primary cancer and 34% had no history of cancer </li></ul>
    18. 18. Primary Sites of Choroidal Mets Breast 1% Kidney <1% Others 4% Skin 1% Skin 4% GI 2% Prostate 6% Others 4% Kidney 6% Unknown 12% GI 9% Lung 12% Unknown 29% Breast 68% Lung 40% Females (N=287) Males (N=137)
    19. 19. Breast Cancer Metastases <ul><li>Most Common Sites: </li></ul><ul><li>Lung (71%) </li></ul><ul><li>Bone (71%) </li></ul><ul><li>Lymph nodes (67%) </li></ul><ul><li>Liver (62%) </li></ul><ul><li>Pleura (50%) </li></ul><ul><li>Ocular (9%-37%) </li></ul>
    20. 20. Clinical Features Uveal Mets <ul><li>Appearance: </li></ul><ul><ul><li>99% choroidal mets from breast cancer were yellow in color </li></ul></ul><ul><ul><li>77% plateau-shaped </li></ul></ul><ul><ul><li>65% a/w subretinal fluid </li></ul></ul><ul><ul><li>Exudation and hemorrhage rare </li></ul></ul><ul><li>Symptoms: </li></ul><ul><li> - 75% Blurred vision </li></ul><ul><li> - 6% Floaters </li></ul><ul><li> - 5% Photopsias </li></ul><ul><li> - 7% Asymptomatic </li></ul><ul><li>Location: </li></ul><ul><ul><li>89% Posterior to equator </li></ul></ul><ul><ul><li>62% Unilateral and 38% bilateral to Uvea </li></ul></ul><ul><li>Met Sites: </li></ul><ul><ul><li>85% to Choroid, 3% to Iris, <1% to Ciliary body </li></ul></ul><ul><ul><li>Other: 5% Optic nerve; <1% Conjunctiva, Orbit, Retina, Adnexa </li></ul></ul>
    21. 21. Prognosis <ul><li>Average patient survival: 8-9 months after ocular diagnosis </li></ul><ul><li>Primary tumor type determines prognosis </li></ul><ul><li>Metastatic malignant melanoma: worst prognosis 1-2 months </li></ul><ul><li>Metastatic breast carcinoma: prognosis of 12 months </li></ul>
    22. 22. Survival Rates for Uveal Mets from Breast CA
    23. 23. Treatment Choroidal Mets <ul><li>Plaque Radiotherapy </li></ul><ul><ul><li>Systemically healthy patients with solitary uveal metastasis or those with metastasis that failed other treatment </li></ul></ul><ul><li>External beam radiation </li></ul><ul><li>Chemotherapy & hormone therapy </li></ul><ul><ul><li>Used to manage disseminated systemic disease </li></ul></ul><ul><ul><li>Aromatase inhibitors - Estrogen receptor positive breast cancer responded to aromatase inhibitors similar to ERBT with no radiation side effects </li></ul></ul><ul><li>Observation </li></ul><ul><ul><li>Uveal metastases that are inactive or regressed </li></ul></ul><ul><ul><li>Asymptomatic tumors </li></ul></ul>Manquez et al. Management of Choroidal metastases from breast carcinomas using aromatase inhibitors, Current Opinion in Ophthalmology 2006, 251-256
    24. 24. Survival Rates + Brain Mets <ul><li>Not treated with Whole Brain Radiation Treatment (WBRT) or treated with corticosteroids alone: 1-2 months </li></ul><ul><li>Median survival is increased to 3-6 months with WBRT & corticosteroids </li></ul><ul><li>WBRT prevents any further deterioration of neurologic function </li></ul>
    25. 25. Back to our patient <ul><li>Patient was started on Decadron </li></ul><ul><li>Radiation-Oncology->Palliative Radiation to whole brain (WBRT) recommended (18 days) </li></ul><ul><li>Systemic chemotherapy to follow </li></ul>
    26. 26. Before Radiation Therapy After completion of 18 day course of radiation therapy but prior to chemotherapy Mass slightly smaller with regularly arranged pigment clumping on tumor surface
    27. 27. Before radiation therapy After Radiation Therapy
    28. 28. 1 Month follow-up s/p Radiation <ul><li>Va 20/50 OD, 20/20 OS </li></ul><ul><li>Pupils: Trace APD OD </li></ul><ul><li>Motility: Full ou </li></ul><ul><li>Confrontation VF: Inferior field defect OD persists </li></ul>
    29. 29. Visual Prognosis Poor <ul><li>Age > 55 years </li></ul><ul><li>Choroidal tumor base diameter > 15mm </li></ul><ul><li>Pre-radiotherapy visual acuity 20/40 or worse </li></ul>
    30. 30. Before radiation therapy After radiation therapy