eMedicine Specialties > Ophthalmology > Lacrimal System

Lacrimal Gland Tumors

Dan D DeAngelis, MD, FRCS(C), Ophthalmic Plastic and Reconstructive Surgery, Assistant Professor, Department of Ophthalmology and Vision Sciences, University of Toronto
Noelene Pang, MD, Fellow in Ophthalmic Plastic and Reconstructive Surgery, Department of Ophthalmology, University of Toronto; 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

Updated: Aug 8, 2006

Introduction

Background

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.

Differential Diagnoses

Dry Eye Syndrome
Exophthalmos
Ptosis, Adult

Workup

Imaging Studies

• Neuroimaging studies can be of great assistance in making the correct diagnosis.
• Computed tomography (CT) scan of benign epithelial lesions, such as pleomorphic adenomas, reveals a well-circumscribed, pseudoencapsulated lesion in the superotemporal fossa.
• Characteristic bony changes include expansion and remodeling in the lacrimal fossa without evidence of bony invasion or erosion.
• In contrast, malignant epithelial lesions, such as adenoid cystic carcinoma, usually present as an irregular mass, producing bony erosion (70%) and occasional calcification (20%).
• Lymphoproliferative lesions usually are eccentric in shape with significant contrast enhancement.

Other Tests

• Immunohistochemistry may be helpful in distinguishing between inflammatory, benign, and malignant lymphoproliferative lesions. Immunohistochemistry is a laboratory modality that uses special markers to demonstrate the presence of specific antigens in target tissues.
• Benign inflammatory lesions (pseudotumor) have a polyclonal morphology, whereas the lymphoid lesions tend to be monoclonal.

Histologic Findings

Histologic examination of pleomorphic adenomas reveals evidence of both epithelial and mesenchymal differentiation. Proliferation of benign epithelial cells usually is arranged in a double layer to form lumens. Stromal differentiation can be seen in the formation of bone and cartilage.

Adenoid cystic carcinomas are derived from duct cells, and they form spaces into which basement membranelike material is deposited. This confers a cribriform or "Swiss cheese" appearance to the tissue, although growth in tubules and nests also are recognized. Five histologic patterns have been observed in these lesions, as follows: (1) cribriform (the most common subtype), (2) sclerosing, (3) basaloid, (4) comedo, and (5) ductal. The basaloid type has the worst prognosis.

Treatment

Medical Care

Radiation therapy is the mainstay of treatment for lymphoid lesions, ranging from 2000-3000 cGy of total radiation. Antineoplastic agents, administered under the direction of an oncologist, usually are required for systemic disease.

Surgical Care

The treatment of lacrimal gland tumors can be divided largely into 2 categories based on the duration of symptoms, clinical evaluation, and radiographic features of the lesion.

• Patients with a long-standing, painless, slowly growing mass with a well-circumscribed appearance on imaging studies are presumed to have a pleomorphic adenoma.
  • Treatment is extirpation, consisting of a lateral orbitotomy with intracapsular removal of all lesional tissue with careful attention to prevent violation of the pseudocapsule.
  • Incisional biopsy of these lesions is contraindicated because, although histologically benign, incomplete excision often leads to repeated recurrences (as high as 30% in some studies) and malignant transformation.
  • Small, fingerlike protuberances outside the main tumor bulk with subsequent seeding of the residual tumor are believed to be responsible for this phenomenon.
  • Painful lesions of short duration (>4-8 wk), especially with concomitant bony involvement, require an incisional biopsy of the lacrimal gland lesion and careful histopathologic evaluation to rule out a malignant neoplasm.
  • If pathologic evaluation of the permanent sections of the lesion reveals adenoid cystic carcinoma, prognosis for survival is poor.
  • Treatment modalities are difficult to evaluate prospectively because of the low incidence and the tendency for long-term recurrences.
  • The classic treatment consists of radical exenteration with frontal bone excision, maxillectomy, and temporalis fossa excision as an attempt at a curative modality in these patients (because of the diffusely infiltrative nature of these lesions and the tendency for perineural spread).
  • If a malignant lesion is suspected preoperatively, complete extirpation as the initial procedure (as per a pleomorphic adenoma) and follow-up radiotherapy of 6400-6800 cGy may produce a long-term survival no worse than radical exenteration with significantly less morbidity.
  • Mortality is increased, if bony involvement is present.

Consultations

• Appropriate hematology and oncology consultations are indicated to exclude systemic involvement, if a diagnosis of lymphoma is confirmed.
• Coordinated treatment with a radiation oncologist often is used for malignant and lymphoid lesions.

Follow-up

Further Inpatient Care

• Hospitalization may be required if treatment with chemotherapeutic agents is needed.

Prognosis

• For pleomorphic adenomas, long-term studies reveal an increased incidence of malignant transformation (10% at 20 y and 20% at 30 y) associated with multiple recurrences for lesions that had frequent incisional biopsies and incomplete removal of the primary tumor.
• Annual follow-up care is suggested to monitor the effects of treatment and to observe for recurrence or systemic involvement.
• Systemic lymphoma develops in 20-30% of patients with malignant lymphoma of the lacrimal gland. Incidence is much higher if the initial presentation is with bilateral lacrimal gland involvement.
• Adenoid cystic carcinomas carry a poorer prognosis because of bony extension and perineural infiltration.
  • These patients have a 50% at 5-year and 75% at 15-year mortality rate. Death commonly is due to intracranial spread and pulmonary metastasis.
  • Histologic pattern also is of prognostic significance with a cribriform pattern having a 70% at 5-year survival compared to a 20% at 5-year survival with a basaloid pattern.

Miscellaneous

Medicolegal Pitfalls

• Early detection and treatment decreases the possibility of negative effects.

References

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

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

3. Font RL, Smith SL, Bryan RG. Malignant epithelial tumors of the lacrimal gland: A clinicopathological study of 21 cases. Arch Ophthalmol. 1998;116:613-6. [Medline].

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

5. 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].

6. 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].

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

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

9. 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].

10. Shields JA, Shields CL, Epstein JA, et al. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthal Plast Reconstr Surg. 2004;20:10-21. [Medline].

11. Stewart WB, Krohel GB, Wright JE. Lacrimal gland and fossa lesions: An approach to diagnosis and management. Ophthalmol. 1979;86:886. [Medline].

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

Keywords

neoplastic lesion, orbital lobe, palpebral lobe, epithelial neoplasm

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 Pang, MD, Fellow in Ophthalmic Plastic and Reconstructive Surgery, Department of Ophthalmology, University of Toronto
Noelene Pang, MD is a member of the following medical societies: 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, Chairman, Department of Ophthalmology and Otorhinolaryngology, Director of Fellowship Training Program, St Francis Medical Center; Associate Professor, Department of Surgery, University of Hawaii School of Medicine
Jorge G Camara, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association
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
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

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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