Overview of Lacrimal Gland Tumors

Tumors of the lacrimal gland fossa are relatively rare and can either be benign or malignant.

Charles H. Weaver, MD
4–6 minutes
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Tumors of the lacrimal gland fossa are relatively rare and can either be benign or malignant. Benign tumors are typically treated with surgery and non-lymphoma malignant cancers historically were treated with a combination of surgery, radiation, and chemotherapy, although intra-arterial chemotherapy prior to surgical resection of adenoid cystic carcinoma, the most common malignant epithelial lacrimal gland tumor, may offer a new direction for improving patient outcomes.1,2

An individual usually becomes aware of a lacrimal gland tumor from proptosis – protrusion of the eyeball or a “droopy eye lid. Although lacrimal gland tumors may appear at any age, cancers most commonly occur in individuals over the age of 50.

Cancers of the lacrimal gland generally carry a poor prognosis so early diagnosis and treatment at a cancer center or team that combines oculoplastic surgeons with individuals that have expertise in oncology should be sought because management can be complicated and requires a multi-specialty approach with physicians experienced in managing orbital tumors and their complications.

Orbital imaging with CT or MRI is used to confirm and characterize a possible orbital lesion. Magnetic resonance imaging is preferred for defining the extent of an orbital mass because it gives superior soft tissue detail and can better evaluate tumor involvement of orbital nerves.

The CT/MRI will guide the surgeon in performing a biopsy or complete removal of the mass, if possible, to obtain tissue to determine its origin. Tissue is required to confirm a diagnosis and direct appropriate treatment.

Possible Origins of a Lacrimal Mass4,5

Inflammatory Lesions

  • Idiopathic orbital inflammatory syndrome (IOIS), or orbital pseudotumor
  • IgG-4 related disease
  • Sarcoidosis and Granulomatosis with polyangiitis
  • Dacryoadenitis
  • Sjogren syndrome
  • Thyroid eye disease

Benign Tumors

  • Epithelial
    • Pleomorphic adenoma
    • Dacryops (lacrimal gland cyst)
    • Oncocytoma
    • Myoepithelioma
    • Cystadenoma
    • Warthin tumor
    • Reactive lymphoid hyperplasia (benign lymphoepithelial lesion)
    • Capillary or cavernous hemangioma
    • Angiolymphoid hyperplasia with eosinophilia
    • Granular cell tumor
    • Fibrous histiocytoma
    • A solitary fibrous tumor (hemangiopericytoma)
    • Neurofibroma
    • Schwannoma

Malignant Cancers of the Lacrimal Gland

    • Adenoid cystic carcinoma (ACC) is the most common malignant epithelial tumor.
    • Carcinoma ex pleomorphic adenoma (CEPA, also known as pleomorphic adenocarcinoma or malignant mixed tumor) is the second most common type of epithelial malignancy.
    • Mucoepidermoid carcinoma is a common salivary gland tumor but a rare lacrimal gland tumor.
    • ductal carcinoma (similar to mammary ductal carcinoma)
    • acinic cell carcinoma
    • myoepithelial carcinoma
    • epithelial-myoepithelial carcinoma
    • cystadenocarcinoma
    • carcinosarcoma
    • polymorphous low-grade adenocarcinoma
    • sebaceous cell carcinoma
    • squamous cell carcinoma
    • basal cell carcinoma.
    • non-Hodgkin’s B cell lymphomas. Extranodal marginal zone lymphoma (EMZL, MALT lymphoma) is most common.5

Treatment

Historically the treatment of most lacrimal gland cancers includs orbital exenteration with or without bone removal and high dose radiation 4 to 6 weeks after surgery. Orbital exenteration consists of removal of the entire eyeball, the eyelids, eyebrows, and surrounding skin.

Globe-sparing surgery to avoid the functional, cosmetic, and psychologic morbidity of orbital exenterations, with similar survival results.6,7,8

Intra-arterial cytoreductive chemotherapy (IACC) has been used successfully before orbital exenteration and radiation therapy and may be combined with globe-sparing surgery and adjuvant radiation therapy.9

Definitive treatment of lymphoma however is not surgical. If the lymphoma is confined to the orbit treatment with external beam radiation therapy may be all that is required. Some B-cell cancers however will require systemic treatment with chemotherapy.

Complications of malignant tumors are mostly related to the treatment involved in eradicating the tumor and preventing recurrences. The tumors may compress orbital structures like the globe or optic nerve if the tumors are large. Malignant lesions may erode through the orbital roof to gain access to the intracranial vault, or in the case of adenoid cystic carcinoma, may spread via the perineural route to access the CNS where severe neurologic changes may manifest.

Surgical biopsy or excision carries risks, and complications are related to the surgery performed and the techniques utilized. For anterior or lateral orbitotomies, the main complications of note include orbital hemorrhage, postoperative orbital cellulitis, damage to the extraocular muscles, globe, or optic nerve, dryness from decreased lacrimal gland function, cerebral spinal fluid (CSF) leak, or recurrence of the tumor.

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  1. Shields JA, Shields CL, Epstein JA, Scartozzi R, Eagle RC. Review: primary epithelial malignancies of the lacrimal gland: the 2003 Ramon L. Font lecture. Ophthalmic Plast Reconstr Surg. 2004 Jan;20(1):10-21.
  2. Kim JS, Liss J. Masses of the Lacrimal Gland: Evaluation and Treatment. J Neurol Surg B Skull Base. 2021 Feb;82(1):100-106.
  3. von Holstein SL, Coupland SE, Briscoe D, Le Tourneau C, Heegaard S. Epithelial tumours of the lacrimal gland: a clinical, histopathological, surgical and oncological survey. Acta Ophthalmol. 2013 May;91(3):195-206.
  4. von Holstein SL, Rasmussen PK, Heegaard S. Tumors of the lacrimal gland. Semin Diagn Pathol. 2016 May;33(3):156-63.
  5. Andreasen S, Esmaeli B, Holstein SL, Mikkelsen LH, Rasmussen PK, Heegaard S. An Update on Tumors of the Lacrimal Gland. Asia Pac J Ophthalmol (Phila). 2017 Mar-Apr;6(2):159-172.
  6. Bonavolontà P, Esmaeli B, Donna P, Tranfa F, Iuliano A, Abbate V, Fossataro F, Attanasi F, Bonavolontà G. Outcomes after eye-sparing surgery vs orbital exenteration in patients with lacrimal gland carcinoma. Head Neck. 2020 May;42(5):988-993.
  7. Han J, Kim YD, Woo KI, Sobti D. Long-Term Outcomes of Eye-Sparing Surgery for Adenoid Cystic Carcinoma of Lacrimal Gland. Ophthalmic Plast Reconstr Surg. 2018 Jan/Feb;34(1):74-78.
  8. https://journals.lww.com/oprs/abstract/2018/01000/long_term_outcomes_of_eye_sparing_surgery_for.13.aspx
  9. Tse DT, Kossler AL, Feuer WJ, Benedetto PW. Long-term outcomes of neoadjuvant intra-arterial cytoreductive chemotherapy for lacrimal gland adenoid cystic carcinoma. Ophthalmology. 2013 Jul;120(7):1313-23.
  10. https://www.aao.org/eyenet/article/diagnosis-management-of-orbital-lymphoma
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