Appendix
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Ophthalmology

Lacrimal Gland Malignancies

Lacrimal gland masses can arise from many etiologies, including neoplastic and non-neoplastic causes. Of the neoplastic causes, the malignant tumors generally have a poor prognosis and thus are treated aggressively. This usually involves a combination of extensive surgical resection (often orbital exenteration), radiation, and chemotherapy. The most notable exception is orbital lymphoma, which is not treated surgically. Recent developments in treatment strategies involving intra-arterial chemoth

v1 · Reviewed Nov 7, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • Malignant lacrimal gland lesions generally carry a poor prognosis; thus, quick and early identification is critical to improving a patient's morbidity and mortality.
  • Most malignant lesions grow more quickly than benign lesions, with a median interval from onset to diagnosis of less than 6 months, compared with 1 to 2 years for benign lesions.
  • Extension of a mass posteriorly towards the orbital apex ("wedge sign") is a finding that correlates with disease of concern and the need for biopsy.
  • Pain or hypoesthesia is often an indicator of malignancy, as most benign lesions are painless.
  • The first step in all suspected malignancies is establishing a formal histological diagnosis, usually achieved via incisional biopsy.
  • Orbital imaging is usually helpful and often necessary to confirm and characterize a suspected orbital lesion.
  • Computed tomography is expedient, widely available, cost-effective, and especially helpful for lacrimal fossa anatomy.
  • It is widely regarded as the initial imaging modality of choice for lacrimal gland lesions, particularly when a benign tumor is suspected.
  • Magnetic resonance imaging (MRI) is more expensive, more difficult to obtain, and has a longer acquisition time, but provides superior soft-tissue detail.
  • Many cases may require both computed tomography and MRI before further management decisions are made.
  • This is followed by a systemic workup by an oncologist, which may include systemic imaging and/or a bone marrow biopsy.
  • The first step in all suspected malignancies is establishing a formal histological diagnosis, usually achieved via incisional biopsy.
  • In some cases, small or easily accessible lesions can be excised in their entirety.
  • Treatment can then be directed by tumor grading and staging.
  • Historically, the treatment of most lacrimal gland carcinomas has included orbital exenteration with or without bone removal and adjuvant high-dose radiation 4 to 6 weeks after surgery.
  • If the lymphoma is confined to the orbit, external-beam radiation therapy is the established initial treatment, except for DLBCL and mantle cell lymphoma, which are treated with chemotherapy.
  • The most notable exception is orbital lymphoma, which is not treated surgically.
  • There has been a push towards globe-sparing surgery due to the functional, cosmetic, and psychological morbidity of orbital exenterations, with similar survival results.
  • More recently, 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.
  • If only a microscopic tumor is present on histopathology, plaque radiation therapy can be considered a treatment option for any lacrimal gland malignancy.
  • except for DLBCL and mantle cell lymphoma, which are treated with chemotherapy.
  • Chemotherapy is indicated if there is systemic involvement, with possible radiation therapy as needed in select cases.
  • Complications of malignant tumors are mostly related to the treatment involved in eradicating the tumor and preventing recurrences.
  • Large tumors may compress orbital structures, including the globe and optic nerve.
  • 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.
  • Radiation therapy can also have adverse effects, including damage to surrounding tissues, skin dermatitis, bone and soft-tissue atrophy, and ocular effects such as retinopathy and dry eye disease.
  • Chemotherapy can lead to many side effects, the most concerning being immunosuppression and the risk of infection.
  • Vigilant short- and long-term monitoring after treatment is crucial to managing these complications and monitoring for tumor recurrences.
  • review
    Lacrimal Gland Malignancies. StatPearls [Internet] — Andrew J. Mueller, Craig N. Czyz — StatPearls Publishing, 2023

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov