Lacrimal gland tumors
Lacrimal gland tumors are rare neoplasms that occur in the tear-secreting glands located above the outer corners of the eyes. These tumors can be benign, such as pleomorphic adenomas, or malignant, like adenoid cystic carcinomas. Generally, benign tumors are more prevalent among adults aged 40 to 50, while malignant forms are often seen in teenagers and individuals in their third decade of life. The exact causes of these tumors remain unknown, and they account for approximately 10% of orbital space lesions, affecting about one in a million people.
Symptoms may vary; while some individuals may not experience any noticeable issues, others could present with eyelid fullness, proptosis (bulging of the eye), blurry or double vision, or even a visible mass on the eyelid. Diagnosis typically involves a tissue biopsy along with imaging techniques such as CT scans and MRIs to assess the extent of the tumor. Treatment approaches differ based on the tumor type, with benign tumors often requiring excisional biopsy and malignant tumors necessitating more extensive surgical interventions. Prognosis varies, as incompletely excised benign tumors have a risk of becoming malignant, while malignant tumors are associated with a significantly poor prognosis and high mortality rates.
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Lacrimal gland tumors
ALSO KNOWN AS: Orbital tumors
RELATED CONDITIONS: Lymphomas
DEFINITION: Lacrimal gland tumors are rare neoplasms of tear-secreting glands located above the outer corner of each eye. The most common benign and malignant lacrimal gland tumors are called pleomorphic adenomas and adenoid cystic carcinomas, respectively.
Risk factors:Benign tumors are commonly diagnosed in adults between the ages of forty and fifty years, while malignant neoplasms typically occur during the third decade and the teenage years. Individuals previously diagnosed with are at risk for developing orbital lymphoma.
Etiology and the disease process: The etiology of lacrimal gland tumors is unknown. Benign tumors arise from epithelial or mesenchymal cells, while malignant lesions originate from duct cells.
Incidence: Lacrimal gland tumors make up around 10 percent of orbital space lesions and occur in one out of one million people. There are only a few reports of this tumor in the literature because of its rarity.
Symptoms: Some patients may be asymptomatic, while some may complain of a mild fullness in the affected eyelid. Occasionally, patients may have gross bulging of the affected eye (proptosis), blurry or double vision, and a visible mass on the eyelid. Eye pain may or may not be present.
Screening and diagnosis: A tissue biopsy is the definitive tool for diagnosing lacrimal gland tumors. Imaging tests such as a computed tomography (CT) scan, (MRI), bone scans, and positron emission tomography (PET) scanning are useful for determining adjacent tissue involvement. The TNM (tumor/lymph node/metastasis) system is used for staging lacrimal gland tumors.
Tumor size and location are described as Tx (primary tumor not evaluable), T0 (no tumor), T1 (tumor is 2 centimeters (cm) or less and limited to the lacrimal gland), T2 (tumor is 2 to 4 cm and limited to the lacrimal gland), T3a (tumor is greater than 5 cm), and T4 (tumor invasion of surrounding structures such as the orbital structures or brain). Node involvement is classified as Nx (regional not evaluable), N0 (no metastasis to regional nodes), and N1 (metastasis to regional nodes). Metastasis is assessed as Mx (distant metastasis not evaluable), M0 (no distant metastasis), or M1 (distant metastasis present).
Treatment and therapy: Treatment depends on the type of tumor. Benign lacrimal gland tumors are treated by complete excisional biopsy. Adenoid cystic carcinoma is treated by surgically removing all orbital structures and bone (exenteration). Before surgery or radiation, intra-arterial cytoreductive chemotherapy (IACC) is sometimes used.
Prognosis, prevention, and outcomes: An incompletely excised benign tumor of the lacrimal gland has a 10 to 20 percent chance of becoming malignant. Because of bone and nerve involvement, malignant lacrimal gland tumors have a poor prognosis. The fifteen-year mortality rate is nearly 75 percent.
Bibliography
Debnam, J. Matthew. Imaging Atlas of Ophthalmic Tumors and Diseases. Springer International Publishing, 2023.
Kim, Jane S., and Jason Liss. "Masses of the Lacrimal Gland: Valuation and Treatment." Journal of Neurological Surgery Part B: Skull Base, vol. 82, no. 1, 2021, pp. 100-106. doi:10.1055/s-0040-1722700.
Mueller, Andrew J., and Craig N. Czyz. “Lacrimal Gland Malignancies.” www.ncbi.nlm.nih.gov/books/NBK578187. Accessed 20 June 2024.
Shields, Jerry A., and Carol L. Shields. Eyelid, Conjunctival, and Orbital Tumors and Intraocular Tumors: An Atlas and Textbook. 3rd ed., Wolters, 2016.
Von Holstein, Sarah Linea. "Tumors of the Lacrimal Gland. Epidemiological, Clinical and Genetic Characteristics." Acta Ophthalmologica, vol. 91, no. 6, 2013, pp. 1–28. doi:10.1111/aos.12271.
Wilson, Fred M., and Preston H. Blomquist. Practical Ophthalmology: A Manual for Beginning Residents. 8th ed., American Academy of Ophthalmology, 2021.