eMedicine Specialties > Ophthalmology > Lacrimal System

Lacrimal Gland Tumors

Author: Dan D DeAngelis, MD, FRCS(C), Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto
Coauthor(s): Noelene K Pang, MD, Ophthalmic Plastic and Reconstructive Surgery; Jeff Hurwitz, MD, FRCS(C), Director of Ophthalmic Plastic Surgery, Ophthalmologist-in-Chief, Mount Sinai Hospital; Chairman, Professor, Department of Ophthalmology, University of Toronto, Canada
Contributor Information and Disclosures

Updated: Jan 14, 2010

Introduction

Background

The lacrimal gland is a bilobed eccrine secretory gland, which is situated in the superotemporal orbit. The 2 lobes of the lacrimal gland, the orbital lobe and the much smaller palpebral lobe, are separated anatomically by the lateral horn of the levator aponeurosis. Only the palpebral lobe can be visualized in the superior fornix on lid eversion. Thus, disease processes that solely affect the orbital lobe may not manifest until later in the course of the illness.

Mass lesions of the lacrimal gland can be classified broadly into inflammatory and neoplastic subtypes. Inflammatory etiologies, while not uncommon, include dacryoadenitis, sarcoidosis, and orbital inflammatory pseudotumor. For the purposes of this discussion, the focus will be on neoplastic lesions of the lacrimal gland. Most of the neoplastic lesions in the lacrimal gland are epithelial in origin, with approximately 50% classified as benign and 50% as malignant.

Benign lesions include pleomorphic adenomas (benign mixed cell tumors), benign reactive lymphoid hyperplasia, and oncocytomas. These lesions are slowly growing masses more commonly found in adults in their forth to fifth decades of life. Malignant tumors of the lacrimal gland include adenoid cystic carcinoma, adenocarcinoma, squamous cell carcinoma, mucoepidermoid carcinoma, and malignant lymphomas.

Adenoid cystic carcinoma is the most common malignant lacrimal gland tumor, comprising 50% of malignant tumors of lacrimal gland and 25% of all lacrimal gland tumors. Most cases are seen in the third decade of life with a second bimodal peak in the teenage years.

Frequency

United States

Data about the prevalence of lacrimal gland tumors is quite sparse in the literature as this condition is quite rare. Malignant epithelial neoplasms of the lacrimal gland account for approximately 2% of all orbital neoplasms. Similarly, epithelial neoplasms account for only 4% of all lacrimal gland lesions.

Mortality/Morbidity

Patients with lacrimal gland tumors, especially malignant ones, need to be observed long term before successful treatment can be claimed. The approximate 15-year mortality rate approaches 75%.

Age

Lacrimal gland tumors are seen more frequently in the third decade of life, and the second bimodal peak is in the teenage years.

Clinical

History

  • The presentation of lacrimal gland tumors varies from patients who are asymptomatic but have a slight fullness in the temporal upper lid to those who present with frank proptosis, diplopia, and an encroaching mass lesion.
  • The history of a long-standing (>1-2 y), noninfiltrating lacrimal gland lesion suggests a benign tumor, such as a pleomorphic adenoma.
  • A shorter history suggests either an inflammatory or a malignant process.
  • Pain most commonly is seen with inflammatory lesions of the lacrimal gland, but adenoid cystic carcinomas and other malignancies also can present with pain secondary to perineural or bony involvement.
  • Malignant lesions characteristically present with a subacute course of proptosis and temporal sensory loss in the distribution of the lacrimal nerve in one third of patients.
  • Diplopia and diminished visual acuity can be seen with rapidly progressive lesions.
  • Benign lesions commonly present with painless inferonasal globe displacement and fullness of the superotemporal lid and orbit.

Physical

  • Examination may reveal clues in delineating the type of lacrimal gland tumor.
  • Displacement of the globe with or without proptosis is the most common presentation of malignant lesions (seen in 75% of these tumors). It characteristically is nonaxial with inferomedial globe displacement.
  • An S-shaped contour to the upper lid also is common with lacrimal gland lesions, but relatively nonspecific to the type of tumor.
  • Similarly, a mass may or may not be palpable in the lacrimal fossa.
    • A firm, rubbery, nontender mass can be seen with either benign or lymphoproliferative lesions.
    • A decreased Schirmer test suggests an inflammatory lesion.
  • Less common findings include motility restriction, elevated intraocular pressure (IOP), and chorioretinal folds.
  • Nonocular findings include preauricular lymphadenopathy from regional metastasis in malignant lesions.

Causes

See Background.

More on Lacrimal Gland Tumors

Overview: Lacrimal Gland Tumors
Differential Diagnoses & Workup: Lacrimal Gland Tumors
Treatment & Medication: Lacrimal Gland Tumors
Follow-up: Lacrimal Gland Tumors
References
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References

  1. Tse DT, Benedetto P, Dubovy S, Schiffman JC, Feuer WJ. Clinical analysis of the effect of intraarterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma. Am J Ophthalmol. Jan 2006;141(1):44-53. [Medline].

  2. Ahmad SM, Esmaeli B, Williams M, Nguyen J, Fay A, Woog J. American Joint Committee on Cancer classification predicts outcome of patients with lacrimal gland adenoid cystic carcinoma. Ophthalmology. Jun 2009;116(6):1210-5. [Medline].

  3. Esmaeli B, Ahmadi MA, Youssef A, et al. Outcomes in patients with adenoid cystic carcinoma of the lacrimal gland. Ophthal Plast Reconstr Surg. Jan 2004;20(1):22-6. [Medline].

  4. Farmer JP, Lamba M, Lamba WR, et al. Lymphoproliferative lesions of the lacrimal gland: clinicopathological, immunohistochemical and molecular genetic analysis. Can J Ophthalmol. Apr 2005;40(2):151-60. [Medline].

  5. Font RL, Smith SL, Bryan RG. Malignant epithelial tumors of the lacrimal gland: a clinicopathologic study of 21 cases. Arch Ophthalmol. May 1998;116(5):613-6. [Medline].

  6. Forrest AW. Pathologic criteria for effective management of epithelial lacrimal gland tumors. Am J Ophthalmol. Jan 1971;1(1 Part 2):178-92. [Medline].

  7. Gamel JW, Font RL. Adenoid cystic carcinoma of the lacrimal gland: the clinical significance of a basaloid histologic pattern. Hum Pathol. Mar 1982;13(3):219-25. [Medline].

  8. Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal fossa lesions. Am J Ophthalmol. Dec 1982;94(6):785-807. [Medline].

  9. Jenkins C, Rose GE, Bunce C, et al. Clinical features associated with survival of patients with lymphoma of the ocular adnexa. Eye (Lond). Oct 2003;17(7):809-20. [Medline].

  10. Jones IS. Surgical considerations in the management of lacrimal gland tumors. Clin Plast Surg. Oct 1978;5(4):561-9. [Medline].

  11. Mafee MF, Edward DP, Koeller KK, Dorodi S. Lacrimal gland tumors and simulating lesions. Clinicopathologic and MR imaging features. Radiol Clin North Am. Jan 1999;37(1):219-39, xii. [Medline].

  12. Perez DE, Pires FR, Almeida OP, Kowalski LP. Epithelial lacrimal gland tumors: a clinicopathological study of 18 cases. Otolaryngol Head Neck Surg. Feb 2006;134(2):321-5. [Medline].

  13. Shields JA, Shields CL, Epstein JA, Scartozzi R, Eagle RC Jr. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthal Plast Reconstr Surg. Jan 2004;20(1):10-21. [Medline].

  14. Snaathorst J, Sewnaik A, Paridaens D, de Krijger RR, van der Meij EH. Primary epithelial tumors of the lacrimal gland; a retrospective analysis of 22 patients. Int J Oral Maxillofac Surg. Jul 2009;38(7):751-7. [Medline].

  15. Stewart WB, Krohel GB, Wright JE. Lacrimal gland and fossa lesions: an approach to diagnosis and management. Ophthalmology. May 1979;86(5):886-95. [Medline].

  16. Wright JE, Stewart WB, Krohel GB. Clinical presentation and management of lacrimal gland tumours. Br J Ophthalmol. Sep 1979;63(9):600-6. [Medline].

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Further Reading

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Keywords

lacrimal gland tumors, lacrimal gland tumor, neoplastic lesion, orbital lobe, palpebral lobe, epithelial neoplasm

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Contributor Information and Disclosures

Author

Dan D DeAngelis, MD, FRCS(C), Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto
Dan D DeAngelis, MD, FRCS(C) is a member of the following medical societies: American Academy of Ophthalmology, American Society of Ophthalmic Plastic and Reconstructive Surgery, California Medical Association, Canadian Medical Association, Canadian Ophthalmological Society, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Noelene K Pang, MD, Ophthalmic Plastic and Reconstructive Surgery
Noelene K Pang, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Ophthalmic Plastic and Reconstructive Surgery
Disclosure: Nothing to disclose.

Jeff Hurwitz, MD, FRCS(C), Director of Ophthalmic Plastic Surgery, Ophthalmologist-in-Chief, Mount Sinai Hospital; Chairman, Professor, Department of Ophthalmology, University of Toronto, Canada
Disclosure: Nothing to disclose.

Medical Editor

Jorge G Camara, MD, Professor of Ophthalmology, Department of Surgery and Director of Fellowship Training Program in Ophthalmic Plastic and Reconstructive Surgery for Countries Served by the Aloha Medical Mission, University of Hawaii John A Burns School of Medicine
Jorge G Camara, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and American Society of Ophthalmic Plastic and Reconstructive Surgery
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

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