Patient presents with progressive proptosis

Imaging revealed a lesion of the left lacrimal gland.

Catherine A. Cox

Jordana F. Goren

A 52-year-old man presented to the oculoplastics service at the New England Eye Center for evaluation of bulging of his left eye, which had occurred gradually over the past 3 to 4 years. He denied any associated ocular pain, headache, double vision or change in vision.

Ocular history was unremarkable. Medical history was significant for HIV and hepatitis C. The patient was a smoker but denied alcohol use.

Examination

Distance visual acuity without correction was 20/25 in the right eye and 20/30 in the left eye. IOP was 19 mm Hg in both eyes. Pupils were equal and reactive to light bilaterally, and there was no afferent pupillary defect. Extraocular movements were full in both eyes. External examination demonstrated left-sided proptosis, measuring 6 mm by Hertel (Figure 1). A firm mass was palpated at the left superior temporal orbit. Slit lamp and dilated fundus exams were unremarkable.

The patient underwent CT and MRI scans, which revealed a 3 cm × 3 cm lesion of the left lacrimal gland (Figure 2). The heterogeneously enhancing mass showed no evidence of intracranial extension or bony invasion.

Figure 1. External exam revealed 6 mm of proptosis and a firm palpable mass at the left superotemporal orbit. Images: Carmody JN, Kapadia M

Figure 2. CT scan (right image) and T2 MRI (left image) demonstrating a 3 cm × 3 cm irregular, heterogenous mass of the left lacrimal gland.

What is your diagnosis?

Lacrimal gland lesion

The differential diagnosis of a lacrimal gland mass includes benign tumors such as pleomorphic adenoma, benign reactive lymphoid hyperplasia, dacryops, and malignant tumors such as pleomorphic adenocarcinoma, adenoid cystic carcinoma, lymphoma, mucoepidermoid carcinoma, and inflammatory lesions such as orbital pseudotumor or sarcoidosis.

Based on the irregularity and heterogeneity of the lesion, a benign lesion such as pleomorphic adenoma was thought to be unlikely, and an incisional biopsy of the lacrimal gland lesion was performed. The biopsy results were consistent with adenoid cystic carcinoma (Figure 3).

Figure 3. Biopsy of the lacrimal gland lesion showed a cribriform and tubular growth pattern of tumor cells with pools of mucin.

Discussion

Adenoid cystic carcinoma is the most common malignant tumor of the lacrimal gland and accounts for 23% of all lacrimal gland epithelial tumors. The tumor presents most commonly in the fourth decade of life and is slightly more common in women.

Patients may present with proptosis, pain and diplopia. The duration of symptoms is often less than 6 months and usually less than 12 months. Orbital pain is present in 10% to 40% of patients and is due to perineural invasion and bony destruction. Exam findings include exophthalmos, downward globe displacement, ptosis, a palpable mass, limitation of extraocular movements, hypoesthesia of the fifth cranial nerve, decreased vision, optic nerve swelling and choroidal folds.

Imaging by CT or MRI reveals a poorly demarcated heterogeneous and irregular lacrimal gland lesion. There may be foci of calcification and bony destruction as well. Definitive diagnosis is by biopsy, which typically demonstrates a non-encapsulated mass with solid nests and tubules of tumor cells and pools of mucin. Staining is positive for pankeratin and CK-7.

Treatment options include exenteration with radical orbitectomy or surgical debulking. Recent literature reports that radical surgery does not lead to improved disease-free survival, and therefore many favor the surgical debulking approach. Post-surgical radiation therapy is recommended as adjunctive therapy, as it has been shown to delay recurrence and death by 4 to 5 years. Preliminary reports have shown success with intraarterial chemotherapy followed by debulking or exenteration, although this treatment is still considered relatively experimental.

The prognosis for adenoid cystic carcinoma is poor, with 50% mortality at 5 years and 80% at 10 years. The rate of recurrence is up to 88% within 6 years of diagnosis, regardless of the treatment option pursued. Recurrences are multiple and may include the ethmoid sinus, frontal sinuses, skull base, dura or cavernous sinus. Metastasis rates are also high, with the most common locations being lung, liver, bone, lymph nodes, kidney and brain.

Follow-up

The patient underwent surgical debulking of the lacrimal gland tumor and radiation treatment. A PET scan revealed uptake in the right parotid gland, concerning for metastasis; he is currently scheduled for biopsy of the parotid gland lesion.

References:

• Ahmad SM, Esmaeli B, Williams M, et al. American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma. Ophthalmology. 2009;116(6):1210-1215.
• Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the lacrimal gland: an update. Curr Opin Ophthalmol. 2008;19(5):409-413.
• Esmaeli B, Ahmadi MA, Youssef A, et al. Outcomes in patients with adenoid cystic carcinoma of the lacrimal gland. Ophthal Plast Reconstr Surg. 2004;20(1):22-26.
• Shields JA, Shields CL, Epstein JA, Scartozzi R, Eagle RC. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthal Plast Reconstr Surg. 2004;20(1):10-21.
• Tse DT, Benedetto P, DuBovy S, Schiffman JC, Feuer WJ. Clinical analysis of the effect of the intraarterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma. Am J Ophthalmol. 2006;141(1):44-53.
• Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol. 1992;76(7):401-407.

• Jill N. Carmody, MD, and Mitesh Kapadia, MD, PhD, can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.

• Edited by Catherine A. Cox, MD, and Jordana F. Goren, MD, MS. Drs. Cox and Goren can be reached at New England Eye Center, Tufts University School of Medicine, 800 Washington St., Box 450, Boston, MA 02111; 617-636-4219; fax: 617-636-4866; website: www.neec.com.