Submitted by Mohit Shukla and Halyna Kuzyshyn
Published October 6, 2019
A 60-year-old African American female presented with a 2-week history of right eye pain, “bulging”, redness, and blurry vision (Figure A). She had a 4-year history of biopsy proven pulmonary sarcoidosis and history of breast cancer status post mastectomy, chemotherapy, and radiation, currently in remission. Laboratory studies revealed an elevated ESR: 41 (0 - 20 mm/hr); elevated CRP: 1.30 (<0.50 mg/dl); normal ACE and LDH levels; thyroid profile; calcium levels; negative screen for ANCA and negative RPR/FTA testing for syphilis. A CT scan of her head and orbits showed right pre-septal, periorbital swelling, enlargement of the right lateral rectus/ superior rectus/ levator palpebrae complex, and fullness of retro-orbital soft tissues adjacent to the optic nerve sheath complex (Figure B & C). CT chest showed improved mediastinal lymphadenopathy as compared to CT from 2 years ago and no other lesions were identified.
The patient was started on high dose intravenous glucocorticoids (1mg/kg) for working diagnosis of ocular sarcoidosis. The eye symptoms failed to resolve even after 1 week of high dose steroid use. For this reason, an anterior orbital biopsy was performed, which showed metastatic disease of breast origin (Figure D&E). She underwent radiotherapy treatment with complete resolution of the eye proptosis.
Image A - Right eye after the biopsy
Image B: CT Orbits with IV contrast showing enlargement of right lateral rectus, superior rectus, levator palpebrae complex as well as prominence of lacrimal gland fossa
Image C: CT Orbits with IV contrast showing enlargement of right lateral rectus, superior rectus, levator palpebrae complex as well as prominence of lacrimal gland fossa
Image D: H&E stain under 400x of right anterior orbit biopsy showing striations of skeletal muscle with tumor cells
Image E: +GCDFP-15 (gross cystic disease fluid protein 15) stain under 400x of right eye biopsy which is specific for breast adenocarcinoma
Question:
Orbital metastasis is most frequently seen in which type of cancer?
A. Lung
B. Breast
C. Prostate
D. Colon
Answer and Discussion
Answer B (breast cancer)
Unilateral proptosis carries a broad differential diagnosis including thyroid disease, orbital pseudotumor, orbital cellulitis, cavernous sinus thrombosis, or intra-orbital neoplasms. It can also be present in 25% of sarcoidosis patients 2. Intra-orbital neoplasms occur due to metastatic breast (40%), lung (11%) and prostate (8%) cancer 1. Ocular sarcoidosis is normally responsive to glucocorticoid therapy, while ocular metastasis is treated with radiation therapy 3. If a patient with a history of sarcoidosis presents with proptosis and does not respond to steroid therapy, then workup for malignancy including ocular biopsy and imaging of the rest of the body for staging of the neoplasm should be done..
Take Home Points:
-Orbital pseudo-tumor, lymphoproliferative disorders, metastatic disease, & sarcoidosis should be considered while evaluating a case of eye proptosis.
-Failure to respond to initial treatment with high dose glucocorticoids should raise the suspicion for underlying malignancy.
-Although intra-orbital neoplasms are rare, breast cancer is the most common cause of metastases to the eye when compared to other neoplasms.
-External beam radiotherapy is the mainstay treatment for eye metastases and provides symptom relief in most cases.
References
1. Birnbaum AD, French DD, Mirsaeidi M, Wehrli S. Sarcoidosis in the National Veteran Population: Association of Ocular Inflammation and Mortality. Ophthalmology 2015.
2. Civit T, Colnat-Coulbois S, Freppel S. Neurochirurgie. 2010 Apr-Jun;56(2-3):148-51.
3. Mavrikakis I, Rootman J. Diverse clinical presentations of orbital sarcoid. Am J Ophthalmol. 2007 Nov;144(5):769-775.
Authors
1. Mohit Shukla, MD, Rheumatology fellow PGY-5
2. Halyna Kuzyshyn, MD, Attending Rheumatologist
Cooper University Hospital, Department of Rheumatology
401 Haddon Ave Camden, NJ, USA 08103-1489