Pleomorphic Adenoma of the Lacrimal Gland With Extensive Calcification

Pleomorphic adenoma is the most common epithelial tumor of the lacrimalgland, and when this tumor is suspected, removal of the mass in its entiretywithout biopsy should be performed.^1,2 Clinical,radiological, and ultrasonographic characteristics, such as extended durationof symptoms, absence of pain, a smooth and well-defined border, absence ofbony erosion, and lack of calcification, suggest the diagnosis of pleomorphicadenoma.^3-5 Wepresent an unusual case of pleomorphic adenoma, initially appearing as a lacrimalfossa mass with dense calcification on ultrasonographic and computed tomographicevaluations.

A 35-year-old white man was seen with a drooping left upper eyelid thathe had had for 1 to 2 years (Figure 1).He denied pain or diplopia. His medical history was significant for trisomy21 and hypothyroidism for 18 months for which he took levothyroxine sodium.His surgical history included a laceration repair of his right brow at age8 years.

Ocular examination results disclosed corrected visual acuity of 20/30OU, normally reactive pupils without an afferent pupillary defect, and fullductions. He was orthophoric at distance. Mild left upper eyelid ptosis accompaniedleft globe displacement, including 0.5 mm of axial proptosis and 5 mm of inferiordisplacement (Figure 1). Anteriorsegment and dilated ophthalmoscopic examination findings were unremarkable.

Computed tomographic imaging studies revealed a well-circumscribed softtissue mass in the superotemporal left orbit, with internal calcificationand without bone destruction (Figure 2).Ultrasonographic studies of the orbit showed a round, well-circumscribed,high-reflective and calcified lesion (Figure3).

The patient underwent surgical excision of the mass through a lateralorbitotomy with replacement of the lateral orbital wall. At the time of surgery,the tumor appeared smooth, gray, and oval. Normal-appearing lacrimal glandtissue was observed anterior to the mass. The tumor was removed without violationof the tumor capsule. The patient's recovery was uneventful.

Histopathologic examination results disclosed an encapsulated tumorcomposed of numerous lobules and cords of squamoid cells separated by spindlecells in a mucoid matrix. Areas of squamous differentiation with keratin productionwere present as well as numerous areas of calcification measuring up to 250µm in diameter (Figure 4).Areas of chondroid differentiation were also present. The tumor capsule wasintact.

Pleomorphic adenomas of the lacrimal gland are typically associatedwith an excellent prognosis, provided they are initially removed in theirentirety.^1,2,6 Dispersionof tumor cells by biopsy or incomplete excision is associated with late recurrenceand occasional malignant transformation.² Preoperativeidentification of pleomorphic adenoma by clinical and radiological evidenceallows planning of an en bloc excision via a lateral orbitotomy.

Various hallmarks of pleomorphic adenoma have been described to aidin their identification. Symptoms present for more than 1 year and an absenceof pain are suggestive of this tumor.³ Anoval, encapsulated appearance without bony destruction or irregular edgeson computed tomographic studies is characteristic.⁵ Onultrasonographic studies, pleomorphic adenomas are medium to highly reflective,with a regular structure and moderate sound attenuation.⁴

This tumor was characteristic of pleomorphic adenoma in several ways.The patient had a painless change in appearance for at least 1 year. The lesionwas oval, well circumscribed, and appeared encapsulated on both computed tomographicand ultrasonographic studies. However, it showed significant, diffuse calcificationon both imaging studies.

In general, the presence of calcification is a radiological sign ofmalignancy, and calcification of masses of the lacrimal gland fossa usuallysuggests malignant disease.⁷ Only 3 histologicallyproven cases of pleomorphic adenoma with calcification can be found in theliterature.⁸ Even in pleomorphic adenomasof the parotid gland, calcification is only rarely found and suggests an oldertumor age.⁹

In summary, although calcification is more common in malignant lacrimalgland fossa tumors, the presence of calcification should not exclude a diagnosisof pleomorphic adenoma. Other clinical and radiological characteristics shouldbe considered when planning surgical management.

The authors have no relevant financial interest in this article.

Corresponding author and reprints: Shannath L. Merbs, MD, PhD, WilmerEye Institute, Maumenee 127, 600 N Wolfe St, Baltimore, MD 21287 (e-mail: smerbs@jhmi.edu).