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Grand Rounds October 20, 2006 Jeffrey D. Colburn, M.D., PGY-2 Vanderbilt Eye Institute 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. History • Past Ocular Hx Negative • Past Med/Surg Hx Negative • Meds Levaquin, Nasacort HQ, Prednisone • Allergies: NKDA • Family Hx An 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, Exam • • • • • 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 Exam • SLE (OS quiet) LLL: Conj: K: A/C: Iris: Lens: Ant Vit: lids edematous arterialized injection, engorged fornix vein arcus OU, otherwise clear Deep & Quiet, no C/F Intact clear quiet Exam • DFE: Disks: C/D: Macula: Periphery: Vitreous: Vascular: sharp margins, good color OU 0.6 OD, 0.4 OS CWS temporally OD few chorio-retinal scars OS clear OU mild tortuosity OU Differential Diagnosis Differential 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 MRI • 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: Metastatic Carcinoma of Unknown Primary Difficult 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 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 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 References • • • • • 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.