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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.