Masses of the Lacrimal Gland: Evaluation and Treatment

Abstract

Lacrimal gland lesions account for approximately 9 to 10% of all biopsied orbital masses. Potential causes include nongranulomatous and granulomatous inflammation, autoimmune disease, lymphoproliferative disorders, benign epithelial proliferation, malignant neoplasia, and metastatic disease. Inflammatory lesions and lymphoproliferative disorders are the most common and may be unilateral or bilateral; they may also be localized to the orbit or associated with systemic disease. Both benign and malignant epithelial lacrimal gland masses tend to be unilateral and involve the orbital lobe, but a more rapid onset of symptoms and periorbital pain strongly suggest malignant disease. On orbital imaging, both inflammatory and lymphoproliferative lesions conform to the globe and surrounding structures, without changes in adjacent bone, whereas epithelial lacrimal gland masses often show scalloping of the lacrimal gland fossa. Malignant epithelial lacrimal gland tumors can also have radiographic evidence of bony invasion and destruction. Masses of the lacrimal gland may be due to a broad range of pathologies, and a good working knowledge of common clinical characteristics and radiographic imaging findings is essential for diagnosis and treatment. All patients with inflammatory, lymphoproliferative, and epithelial neoplastic lesions involving the lacrimal gland require long-term surveillance for disease recurrence and progression.

Keywords: adenoid cystic carcinoma; carcinoma ex pleomorphic adenoma; dacryoadenitis; lacrimal gland mass; orbital lymphoma; orbital pseudotumor; pleomorphic adenoma; surgical debulking of lacrimal gland.

Fig. 1

Nonspecific orbital inflammation (NSOI) involving the lacrimal gland. ( a ) A 36-year-old woman with chronic left-sided proptosis and recurrent bouts of inflammation of the lacrimal gland and surrounding structures. Systemic workup was negative for underlying autoimmune disease, and lacrimal gland biopsy confirmed the diagnosis of NSOI. ( b ) T1-weighted contrast-enhanced magnetic resonance imaging of the orbits showed marked and diffuse enhancement of the lacrimal gland, superior rectus muscle, and adjacent connective tissues on the left.

Fig. 2

Lymphoma. ( a ) A 73-year-old man with a history of hypothyroidism presented with significant enlargement of right greater than left lacrimal glands. On examination, both lacrimal glands were firm, immobile, and nontender. ( b ) Computed tomography of the orbits showed enlarged, homogeneous, moderately enhancing lacrimal glands. Positron emission tomography revealed diffuse visceral disease. The patient is currently being monitored without treatment for indolent disease by his hematologist-oncologist.

Fig. 3

Pleomorphic adenoma (benign mixed tumor). ( a ) A 32-year-old woman with no prior medical history presented with symptoms of progressive proptosis, diplopia, and decreased vision on the right for over 5 years. ( b ) There was marked limitation of upgaze on the right. ( c ) T1-weighted magnetic resonance imaging of the orbits showed a large, round, heterogeneously enhancing mass of the orbital lobe of the lacrimal gland, with inferonasal globe displacement. The mass was removed en bloc by lateral orbitotomy.

Fig. 4

An orbital prosthesis, as seen on the patient's left, can be attached to the exenterated socket by adhesives, magnets, or osseointegrated implants. Broad-rimmed spectacles can help conceal the edges of the orbital prosthesis.

Fig. 5

Adenoid cystic carcinoma. An axial view on T1-weighted magnetic resonance imaging of the orbits shows a large, enhancing superolateral mass causing 5 mm of proptosis and displacing the globe downward, with a positive tail sign ( arrowhead ). The patient underwent exenteration alone, but developed a pulmonary nodule a year later, for which she was treated with carboplatin and paclitaxel. Approximately 7.5 years after diagnosis, she is alive with stable pulmonary nodules.