Grand RoundsGrand RoundsOctober 20, 2006October 20, 2006

Jeffrey D. Colburn, M.D., PGY-2Jeffrey D. Colburn, M.D., PGY-2

Vanderbilt Eye InstituteVanderbilt Eye Institute

The Case…The Case…• CC: Right eye swelling x 3 weeks

• HPI: 47 y.o. AA male, recently released from jail– “Pressure” in right temple region– Blurry vision, redness & tearing OD– Denies any injuries– Outside ophthalmologist and ENT treating for

sinusitis. Thyroid studies pending.

HistoryHistory• Past Ocular Hx

Negative

• Past Med/Surg HxNegative

• MedsLevaquin, Nasacort HQ,

Prednisone

• Allergies: NKDA

• Family HxAn aunt with Glaucoma

• Social Hx– “pretty good amount” EtOH– 60 pack yr smoking hx– Smokes crack cocaine– Occasional MJN

• ROS+ SOB, fatigue, weakness,

dizziness, HA’s, ~ 20 lbs wt loss, Right ear fullness, lightheadedness,

- Fever, chills, night sweats, URI symptoms,

ExamExam• VA: 20/60 OD, 20/25 OS• IOP: 26 OD, 21 OS • CVF: Full• Pupils: No anisocoria, no RAPD• Motility: OD -3 in upgaze/downgaze, -2 left/right gaze

• Hertel: 25 OD, 18 OS (123mm)• Color: 6/15 OD, 13/15 OS

• External: Proptosis, ptosis & lid edema OD, – No bruit heard over orbit

ExamExam• SLE (OS quiet)

LLL: lids edematous

Conj: arterialized injection, engorged fornix vein

K: arcus OU, otherwise clear

A/C: Deep & Quiet, no C/F

Iris: Intact

Lens: clear

Ant Vit: quiet

ExamExam• DFE:

Disks: sharp margins, good color OU

C/D: 0.6 OD, 0.4 OS

Macula: CWS temporally OD

Periphery: few chorio-retinal scars OS

Vitreous: clear OU

Vascular: mild tortuosity OU

Differential DiagnosisDifferential Diagnosis

Differential DiagnosisDifferential Diagnosis• Vascular

– C-C fistula (Low flow)– Arteriovenous malformation– Cavernous sinus thrombosis

• Neoplastic– Lymphoma/Leukemia– Cavernous sinus tumors– Orbital tumors– Metastatic tumors– Mucocele

• Infectious– Orbital cellulitis– Mucormycosis– Tuberculosis

• Trauma– Retrobulbar hemorrhage– Intraorbital foreign body

• Inflammatory/Infiltrative– Thyroid eye disease– Orbital inflammatory pseudotumor– Orbital vasculitis

• Wegener’s granulomatosis• Polyarteritis nodosa

– Intracranial sarcoidosis– Tolosa-Hunt syndrome

• Neurologic– Cranial nerve palsy

MRIMRI

• Admitted for WBC’s, hyperkalemia, alkalosis

• Labs unrevealing – (RPR, HIV, ANA, thyroid panel, blood cx)

• CXR: vertebral osteoblastic lesions, +lymphadenopathy, reticulonodular opacities

• CT – Diffuse pathologic lymphadenopathy

• Biopsies – poorly differentiated carcinoma

• GI Work-up / Tumor markers – Negative

Diagnosis:Diagnosis:Metastatic Carcinoma of Metastatic Carcinoma of

Unknown PrimaryUnknown Primary

Difficult CourseDifficult Course

• Progression clinically – High IOP despite lateral cantholysis, MMT

• Partial response to IV steroids

• Responded to XRT to right orbit

• Died 5 weeks after presentation– Had completed 1 round of chemotherapy

Metastatic carcinoma to the orbitMetastatic carcinoma to the orbit• Commonly develop symptoms abruptly

• Features:– Pronounced restriction– Eyelid swelling, ptosis, mass– Vision loss– Proptosis (less prominent than primary)– > 90% unilateral

• Often aggressive and poorly differentiated• Systemic prognosis is poor

• Shields, et al. (2001)– 100 malignant neoplasms: 91 carcinomas

• Breast – 53• Prostate – 12• Lung – 8• Unknown primary – 7

– Thirteen presented first with orbital mets

• A number of published reports of metastasis to the extraocular muscles

• Including:– Loes, Wesley, & Lavin (1996)– Lekse, Zhang, & Mawn (2003)

• Report of such diffuse involvement?

Carcinoma of Unknown PrimaryCarcinoma of Unknown Primary• 2 - 4% of cancer diagnoses• Median survival: 4 - 5 mos• Treatment options have been poor

– Toxicity– Efficacy

• New chemotherapy regimens hold promise– Greco, et al. (2000) – combination of paclitaxel,

carboplatin, etoposide with median survival of 11 mos

ReferencesReferences• Greco, FA, et al. Carcinoma of Unknown Primary Site: Long term

follow-up after treatment with paclitaxel, carboplatin, and etoposide. Cancer 2000;89:2655-60.

• Henderson, JW Orbital Tumors. W.B. Saunders Company, Philadephia, 1973. pp 474-494.

• Kanski, JJ. Clinical Ophthalmology: A systematic Approach. 5th ed. Butterworth Heinemann, New York, 2003.

• Lekse, JM, et al. Metastatic gastroesophageal junction adenocarcinoma to the extraocular muscles. Opthalmology 2003;110:318-321

• Shields, JA, Shields, CL, et al. Cancer Metastatic to the Orbit: The 2000 Robert M. Curts Lecture. Ophthal Plast Reconstr Surg 2001;17:346-354.