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Answers: 2010 Series : March 16, 2010
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Photos courtesy of: Bita Esmaeli, M.D. / M.D. Anderson Cancer Center
Used with permission. Not to be reproduced.

A 25-year-old female presented with a 3-year history of progressive proptosis of the right globe, inferonasal globe dystopia, and decreased abduction. Her CT and lesion histology are shown.

1. The most likely diagnosis is:

a -- pleomorphic adenoma

This young adult has a classic presentation of pleomorphic adenoma of the lacrimal gland. A chronic history (often greater than 1 year) of painless proptosis due to slow growth of the mass, with inferonasal globe dystopia is typical. As in this case, imaging shows a well circumscribed, ovoid lacrimal mass and may show bony remodeling as in this case without bony destruction. Histology shows the classic areas of both epithelial and myxoid stromal patterns (benign mixed tumor).

2. Which statement is true?

c -- This is the most common primary benign epithelial lacrimal gland tumor.

Statements A, D, and E are true of adenoid cystic carcinoma, the most common primary malignant epithelial lacrimal gland tumor. Pleomorphic adenoma is typically seen in younger patients, not the elderly.

3. Treatment of choice is:

b -- en-bloc complete surgical excision

Because disruption of the pseudocapsule that surrounds pleomorphic adenomas can lead to malignant transformation, incisional biopsy is contraindicated if the disease is suspected on clinical and radiographic grounds. The tumor should be removed in total with a rim of normal orbital tissue to ensure complete removal and decrease risk of malignant transformation. Exenteration may be considered for malignant epithelial lacrimal gland tumors. Observation is not recommended as de novo malignant transformation may occur over time.