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Man experiences long-term proptosis of left eye
The patient had a painful soreness only when lying down.
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A 34-year-old male landscaper presented to the eye clinic for evaluation of increasing proptosis of the left eye over a period of 8 months. He recalled that he was hit by a small rock in the area of the left lateral orbit at the time he first noticed swelling. Since then, he had intermittent episodes of redness and noted a decline in vision in the left eye. He denied constant or severe pain but noticed that he had a painful soreness that worsened when lying down. He denied having any soreness or pain when sitting upright or any fluctuations in the proptosis. He had no significant medical, surgical or ocular history and did not take any medications. Of note, his mother had thyroid disease from which she developed ocular complications.
Examination
The patient’s best corrected visual acuity was 20/20 in the right eye and 20/30 in the left eye. Pupillary response in both eyes was normal with no afferent pupillary defect. Visual fields were normal on confrontation. Ishihara color plates were 5/10 plates in the right eye and 3/10 plates in the left eye. Duction testing was full in the right eye, but the left eye demonstrated a –1 limitation in supraduction. IOP was 13 mm Hg in the right eye and 23 mm Hg in the left eye.
External exam (Figure 1) showed 3 mm to 4 mm of left eye proptosis with fullness of the left superior sulcus and mild resistance to retropulsion. Anterior segment exam of the right eye was normal. Slit lamp biomicroscopy was remarkable only for a 1-mm pterygium in the left eye; otherwise, both eyes had unremarkable anterior segment examination. Posterior segment exam revealed an optic nerve with cup-to-disc ratio of 0.1 with a mildly hyperemic nasal segment, but no retinal or choroidal folds. The right eye was unremarkable with healthy nerve appearance and 0.2 cup-to-disc ratio. Axial orbital CT showed bony fossa formation and indentation of the globe from a contrast-enhancing mass (Figure 2).
Figure 1. External photo demonstrating proptosis in worm’s eye view and fullness of the left upper lid sulcus with globe ptosis.
Images: Lee G, Kapadia M
Figure 2. Axial sections of orbital CT scan showing bony fossa formation and indentation of the globe from a contrast-enhancing mass.
What is your diagnosis?
Unilateral proptosis
The differential diagnosis of unilateral painless progressive proptosis with a mass includes pleomorphic adenoma, lymphoma, adenoid cystic carcinoma, metastatic disease and cavernous hemangioma. On further questioning, the patient was able to show a photo from 1 year prior that had evidence of fullness of the left upper sulcus. Additionally, over the past year, he had no pain and only recently began experiencing soreness of the eye likely related to exposure. Based on his CT scan, the mass was concerning for lymphoid-like molding, but careful examination showed bony fossa formation and a mild indentation of the globe. Given his history, clinical findings and imaging, he had the suspected diagnosis of pleomorphic adenoma and underwent surgical excision of the mass (Figure 3).
Figure 3. Surgical removal of well-encapsulated mass via lateral orbitotomy approach, removed in entirety.
Discussion
Pleomorphic adenoma, also known as benign mixed-cell tumor, is the most common benign epithelial tumor arising from the lacrimal gland, accounting for 53% of all epithelial lesions in the lacrimal fossa. It is composed of glandular elements mixed with mesenchymal tissue typically contained within a pseudocapsule composed of connective tissue. As seen in Figure 4, the epithelial cells form ducts with columnar or cuboidal lining with an outer myoepithelial cell layer. The mesenchymal portion is comprised of myxoid tissue, cartilage (* in Figure 4) and, occasionally, fat or bone.
Figure 4. Pathology showing ductal glandular structures with epithelial lining interspersed with mesenchymal connective tissue and cartilage (*).
Clinically, pleomorphic adenomas of the lacrimal gland usually manifest as a slow, progressive, painless superolateral orbital mass with inferonasal displacement of the globe. Absence of pain and chronicity of symptoms are suggestive of this type of tumor. Additionally, on CT scan, pleomorphic adenomas manifest as solitary encapsulated lesions without adjacent bony destruction with possible indentation of the globe, depending on the size of the tumor. It causes fossa formation in the bone as a result of long-standing pressure rather than erosion, which is typically seen in malignant tumors of the lacrimal gland.
Pleomorphic adenomas should be excised in their entirety with an intact capsule. These tumors should not be biopsied, unlike other types of tumors included in the differential diagnosis for a lacrimal gland mass (ie, lymphoid, malignant). Incomplete excision can lead to recurrence, which may be as high as 30%. Additionally, at 30 years of follow-up, recurrent tumors have been shown to have a 20% rate of malignant transformation and local infiltration of orbital soft tissue, bone and brain.
The tumor in our case had characteristics consistent with pleomorphic adenoma by history, exam and imaging. Our patient had no pain, and a picture from 1 year prior showed fullness to the sulcus but no proptosis. On CT scan, the lesion was well circumscribed with bony fossa formation and caused a slight indentation to the globe.
Conclusion
The patient underwent lateral orbitotomy with successful removal of the mass. Pathology confirmed the diagnosis of pleomorphic adenoma. On follow-up, his vision was 20/20 in the left eye, his IOP normalized to 15 mm Hg, and he had full extraocular movements with complete resolution of ptosis.
References:
Eagle RC. Eye Pathology: An Atlas and Text. 2nd ed. Philadelphia; Lippincott Williams & Wilkins; 2011.Font RL, Gamel JW. Epithelial tumours of the lacrimal gland: an analysis of 265 cases. In: Jakobiec FA, ed. Ocular and Adnexal Tumors. Birmingham, Ala.; Aesculapius; 1978:787-805.
Jakobiec FA, et al. Am J Ophthalmol. 1982;94(5)785-807.
Shields CL, et al. Ophthalmology. 1989;doi:10.1016/S0161-6420(89)32873-9.
Stewart WB, et al. Ophthalmology. 1979;doi:10.1016/S0161-6420(79)35467-7.
For more information:
Greg Lee, MD, and Mitesh Kapadia, MD, PhD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.Edited by Jennifer Renz, MD, and Avneet K. Sodhi, MD. Renz and Sodhi can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.