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Arch Ophthalmol. 2004;122:778-780.

Pleomorphic adenoma is the most common epithelial tumor of the lacrimal gland, and when this tumor is suspected, removal of the mass in its entirety without biopsy should be performed.^1-2 Clinical, radiological, and ultrasonographic characteristics, such as extended duration of symptoms, absence of pain, a smooth and well-defined border, absence of bony erosion, and lack of calcification, suggest the diagnosis of pleomorphic adenoma.^3-5 We present an unusual case of pleomorphic adenoma, initially appearing as a lacrimal fossa mass with dense calcification on ultrasonographic and computed tomographic evaluations.

Report of a Case.
A 35-year-old white man was seen with a drooping left upper eyelid that he had had for 1 to 2 years (Figure 1). He denied pain or diplopia. His medical history was significant for trisomy 21 and hypothyroidism for 18 months for which he took levothyroxine sodium. His surgical history included a laceration repair of his right brow at age 8 years.

View larger version (86K): [in this window] [in a new window] Figure 1. A 35-year-old man with long-standing, painless left globe displacement.

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

Computed tomographic imaging studies revealed a well-circumscribed soft tissue mass in the superotemporal left orbit, with internal calcification and without bone destruction (Figure 2). Ultrasonographic studies of the orbit showed a round, well-circumscribed, high-reflective and calcified lesion (Figure 3).

View larger version (48K): [in this window] [in a new window] Figure 2. A, Axial computed tomographic studies demonstrating a well-circumscribed tumor with areas of calcification. B, Same features seen in coronal view.

View larger version (43K): [in this window] [in a new window] Figure 3. A, Paraocular B-scan ultrasonographic study showing the lacrimal gland lesion. The tumor is well outlined, and multiple foci of calcium are evident. B, Standardized A-scan ultrasonographic study showing the internal structure and high reflectivity produced by the calcification.

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

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

View larger version (228K): [in this window] [in a new window] Figure 4. A, Histopathologic appearance of the tumor showing squamous metaplasia with keratin pearls and areas of calcification (hematoxylin-eosin, original magnification x40). B, Light microscopy study demonstrating mixed epithelial and mesenchymal elements (hematoxylin-eosin, original magnification x100). C, Areas of the tumor exhibiting chondroid differentiation (hematoxylin-eosin, original magnification x100). D, High-power light microscopic view showing a keratin cyst with central calcification (hematoxylin-eosin, original magnification x100).

Comment.
Pleomorphic adenomas of the lacrimal gland are typically associated with an excellent prognosis, provided they are initially removed in their entirety.^1-2,6 Dispersion of tumor cells by biopsy or incomplete excision is associated with late recurrence and occasional malignant transformation.² Preoperative identification of pleomorphic adenoma by clinical and radiological evidence allows planning of an en bloc excision via a lateral orbitotomy.

Various hallmarks of pleomorphic adenoma have been described to aid in their identification. Symptoms present for more than 1 year and an absence of pain are suggestive of this tumor.³ An oval, encapsulated appearance without bony destruction or irregular edges on computed tomographic studies is characteristic.⁵ On ultrasonographic 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 lesion was oval, well circumscribed, and appeared encapsulated on both computed tomographic and ultrasonographic studies. However, it showed significant, diffuse calcification on both imaging studies.

In general, the presence of calcification is a radiological sign of malignancy, and calcification of masses of the lacrimal gland fossa usually suggests malignant disease.⁷ Only 3 histologically proven cases of pleomorphic adenoma with calcification can be found in the literature.⁸ Even in pleomorphic adenomas of the parotid gland, calcification is only rarely found and suggests an older tumor age.⁹

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

The authors have no relevant financial interest in this article.

AUTHOR INFORMATION

S. Tonya Stefko, MD
Pittsburgh, Pa

Cathy DiBernardo, RN, RDMS, ROUB; W. Richard Green, MD; Shannath L. Merbs, MD, PhD
Baltimore, Md

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

REFERENCES
1. Forrest AW. Epithelial lacrimal gland tumors: pathology as a guide to prognosis. Trans Am Acad Ophthalmol Otolaryngol. 1954;58:848-866. PUBMED 2. 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. 3. Stewart WB, Krohel GB, Wright JE. Lacrimal gland and fossa lesions: an approach to diagnosis and management. Ophthalmology. 1979;86:886-895. ISI | PUBMED 4. Byrne SF, Green RL. Ultrasound of the Eye and Orbit. 2nd ed. St Louis, Mo: Mosby; 2002:324. 5. Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal fossa lesions. Am J Ophthalmol. 1982;94:785-807. PUBMED 6. Zimmerman LE, Sanders TE, Ackerman LV. Epithelial tumors of the lacrimal gland: prognostic and therapeutic significance of histologic types. Int Ophthalmol Clin. 1962;2:337-367. FULL TEXT 7. Froula PD, Bartley GB, Garrity JA, Forbes G. The differential diagnosis of orbital calcification as detected on computed tomographic scans. Mayo Clin Proc. 1993;68:256-261. PUBMED 8. Rose GE, Wright JE. Pleomorphic adenoma of the lacrimal gland. Br J Ophthalmol. 1992;76:395-400. FREE FULL TEXT 9. Harrison JD. Ultrastructural observation of calcification in a pleomorphic adenoma of the parotid gland. Ultrastruct Pathol. 1991;15:185-188. PUBMED

SECTION EDITOR: W. Richard Green, MD

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