Choroidal Melanoma

Michele Todman, MD Michele Todman, MD

November 3, 2009November 3, 2009

Grand Rounds

History

CC: Sudden loss of inferior field of vision of my right eyeHPI: 57 y/o female -2 day h/o inferior altitudinal field loss OD- Noticed when closing OS to apply make-upPMH: HTNPSH: NoneMeds: ASA, HCTZSH: 1PPD smoker x 30 yearsFH: Mother died at 82 y/o of metastatic cancer, primary

unknown

Examination

VA: 20/50 OD; 20/20 OS

Pupils: Trace APD OD

Motility: Full OU

Confrontation VF: Inferior field defect OD; WNL OS

SLE: 1+NSC OU

Fundus Photography

Fundus Photography

FA

23 sec 34 sec 1:30

1:32 3.51 4.13

Ultrasound

A-scan: Medium to high internal reflectivityB-scan: Acoustic solidarity

Differential Diagnosis?

A-scan: Low internal reflectivity withreduction in amplitude. High amplitudeSpike = break in Bruch’s membrane.

B-scan: Solid mass with mushroom shape

A-scan: High internal reflectivity

B-scan: Highly reflective plate-like lesion with orbital shadowing beyond lesion

FA can help distinguish if CNV

A-scan: High internal reflectivity

B-scan: Solid elevated mass

B-scan: Thickening of the retina, choroid and sclera with fluid beneath Tenon’s capsule and squaring of optic nerve shadow

A-scan: Medium to high internal reflectivityB-scan: Acoustic solidarity

Working Diagnosis

• Is this choroidal mass with exudative detachment a primary lesion or a metastatic lesion to the choroid?

• Need thorough systemic evaluation– PCP contacted– 1 wk prior pt presented with blurred vision OD & SOB– Found to have 13cm x 15cm left breast mass– Lab Work: Liver function tests wnl– CT Chest: Breast mass, lung nodules, liver lesions– Breast Biopsy: Infiltrating ductal carcinoma, ER/PR Negative– MRI brain: Frontal, occipital, and cerebellar lesions– PET Scan: Breast mass, lung, bone, adrenal, hepatic uptake

Stage 4 Metastatic Breast Cancer

Incidence of ocular metastasis

-1872: first description of a metastatic tumor to the eye in a patient with carcinoma

-Since then realization that ocular mets are not that rare

-Bloch and Gartner (1971) 10% patients with autopsy proven carcinoma had ocular mets

- Most common intraocular malignancy- choroidal metastasis

-Hard to gauge exact incidence as patients often are asymptomatic or present with advanced CA and eye exam never performed

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

Epidemiology

• For American women lifetime risk breast cancer- one in eight (12.5%) & lifetime risk death from breast cancer is 3.4%

• Breast Cancer is the most common malignancy to metastasize to the uvea

• Mean age at ocular diagnosis is 58 years, most cases (40-70 yrs)

• At time of ocular diagnosis 66% reported a history of a primary cancer and 34% had no history of cancer

Primary Sites of Choroidal Mets

Males (N=137) Females (N=287)

Lung 40% Breast 68%

Unknown 29% Lung 12%

GI 9% Unknown 12%

Kidney 6% Others 4%

Prostate 6% GI 2%

Skin 4% Skin 1%

Others 4% Kidney <1%

Breast 1%

Breast Cancer Metastases

Most Common Sites:

• Lung (71%)

• Bone (71%)

• Lymph nodes (67%)

• Liver (62%)

• Pleura (50%)

• Ocular (9%-37%)

Clinical Features Uveal MetsAppearance:

– 99% choroidal mets from breast cancer were yellow in color– 77% plateau-shaped– 65% a/w subretinal fluid– Exudation and hemorrhage rare

Symptoms: - 75% Blurred vision - 6% Floaters - 5% Photopsias - 7% Asymptomatic

Location:– 89% Posterior to equator– 62% Unilateral and 38% bilateral to Uvea

Met Sites:– 85% to Choroid, 3% to Iris, <1% to Ciliary body– Other: 5% Optic nerve; <1% Conjunctiva, Orbit, Retina, Adnexa

Prognosis

• Average patient survival: 8-9 months after ocular diagnosis

• Primary tumor type determines prognosis• Metastatic malignant melanoma: worst

prognosis 1-2 months• Metastatic breast carcinoma: prognosis of

12 months

Survival Rates for Uveal Mets from Breast CA

Treatment Choroidal Mets• Plaque Radiotherapy

– Systemically healthy patients with solitary uveal metastasis or those with metastasis that failed other treatment

• External beam radiation • Chemotherapy & hormone therapy

– Used to manage disseminated systemic disease – Aromatase inhibitors - Estrogen receptor positive breast cancer

responded to aromatase inhibitors similar to ERBT with no radiation side effects

• Observation– Uveal metastases that are inactive or regressed– Asymptomatic tumors

Manquez et al. Management of Choroidal metastases from breast carcinomas using aromatase inhibitors, Current Opinion in Ophthalmology 2006, 251-256

Survival Rates + Brain Mets

• Not treated with Whole Brain Radiation Treatment (WBRT) or treated with corticosteroids alone: 1-2 months

• Median survival is increased to 3-6 months with WBRT & corticosteroids

• WBRT prevents any further deterioration of neurologic function

Back to our patient

• Patient was started on Decadron

• Radiation-Oncology->Palliative Radiation to whole brain (WBRT) recommended (18 days)

• Systemic chemotherapy to follow

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

Before radiation therapy

After Radiation Therapy

1 Month follow-up s/p Radiation

• Va 20/50 OD, 20/20 OS

• Pupils: Trace APD OD

• Motility: Full ou

• Confrontation VF: Inferior field defect OD persists

Visual Prognosis Poor

• Age > 55 years

• Choroidal tumor base diameter > 15mm

• Pre-radiotherapy visual acuity 20/40 or worse

Before radiation therapy After radiation therapy