Lacrimal Gland Tumors
- Author: Dan D DeAngelis, MD, FRCSC; Chief Editor: Hampton Roy, Sr, MD more...
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.
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.
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%.
Lacrimal gland tumors are seen more frequently in the third decade of life, and the second bimodal peak is in the teenage years.
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. 2006 Jan. 141(1):44-53. [Medline].
Rasmussen P, Ralfkiaer E, Prause JU, et al. Malignant lymphoma of the lacrimal gland: a nation-based study. Arch Ophthalmol. 2011 Oct. 129(10):1275-80. [Medline].
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. 2009 Jun. 116(6):1210-5. [Medline].
Esmaeli B, Ahmadi MA, Youssef A, et al. Outcomes in patients with adenoid cystic carcinoma of the lacrimal gland. Ophthal Plast Reconstr Surg. 2004 Jan. 20(1):22-6. [Medline].
Farmer JP, Lamba M, Lamba WR, et al. Lymphoproliferative lesions of the lacrimal gland: clinicopathological, immunohistochemical and molecular genetic analysis. Can J Ophthalmol. 2005 Apr. 40(2):151-60. [Medline].
Font RL, Smith SL, Bryan RG. Malignant epithelial tumors of the lacrimal gland: a clinicopathologic study of 21 cases. Arch Ophthalmol. 1998 May. 116(5):613-6. [Medline].
Forrest AW. Pathologic criteria for effective management of epithelial lacrimal gland tumors. Am J Ophthalmol. 1971 Jan. 1(1 Part 2):178-92. [Medline].
Gamel JW, Font RL. Adenoid cystic carcinoma of the lacrimal gland: the clinical significance of a basaloid histologic pattern. Hum Pathol. 1982 Mar. 13(3):219-25. [Medline].
Jakobiec FA, Yeo JH, Trokel SL, et al. Combined clinical and computed tomographic diagnosis of primary lacrimal fossa lesions. Am J Ophthalmol. 1982 Dec. 94(6):785-807. [Medline].
Jenkins C, Rose GE, Bunce C, et al. Clinical features associated with survival of patients with lymphoma of the ocular adnexa. Eye (Lond). 2003 Oct. 17(7):809-20. [Medline].
Jones IS. Surgical considerations in the management of lacrimal gland tumors. Clin Plast Surg. 1978 Oct. 5(4):561-9. [Medline].
Mafee MF, Edward DP, Koeller KK, Dorodi S. Lacrimal gland tumors and simulating lesions. Clinicopathologic and MR imaging features. Radiol Clin North Am. 1999 Jan. 37(1):219-39, xii. [Medline].
Mallen-St Clair J, Arshi A, Tajudeen B, Abemayor E, St John M. Epidemiology and treatment of lacrimal gland tumors: a population-based cohort analysis. JAMA Otolaryngol Head Neck Surg. 2014 Dec. 140(12):1110-6. [Medline].
Perez DE, Pires FR, Almeida OP, Kowalski LP. Epithelial lacrimal gland tumors: a clinicopathological study of 18 cases. Otolaryngol Head Neck Surg. 2006 Feb. 134(2):321-5. [Medline].
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. 2004 Jan. 20(1):10-21. [Medline].
Skinner HD, Garden AS, Rosenthal DI, Ang KK, Morrison WH, Esmaeli B, et al. Outcomes of malignant tumors of the lacrimal apparatus: the University of Texas MD Anderson Cancer Center experience. Cancer. 2011 Jun 15. 117(12):2801-10. [Medline].
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. 2009 Jul. 38(7):751-7. [Medline].
Stewart WB, Krohel GB, Wright JE. Lacrimal gland and fossa lesions: an approach to diagnosis and management. Ophthalmology. 1979 May. 86(5):886-95. [Medline].
Wilson KF, Ward PD, Spector ME, Marentette LJ. Orbitocranial approach for treatment of adenoid cystic carcinoma of the lacrimal gland. Ann Otol Rhinol Laryngol. 2011 Jun. 120(6):397-400. [Medline].
Wright JE, Stewart WB, Krohel GB. Clinical presentation and management of lacrimal gland tumours. Br J Ophthalmol. 1979 Sep. 63(9):600-6. [Medline].