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{{Infobox disease | Name = {{PAGENAME}} | DiseasesDB = | ICD9 = | ICD10 = | OMIM = | MeshID = | MedlinePlus = 001611 }} = Disease Entity = Lacrimal gland tumors == Disease and distribution == Lacrimal gland tumors are very rare. With a yearly incidence of one in one million, they represent only 10% of orbital space-occupying lesions.<ref name="shields1">Shields JA, Shields CL, Epstein J, et al. Primary epithelial malignancies of the lacrimal gland. The 2003 Ramon L. Font Lecture. Ophthalmic Plast Reconstr Surg 2004;20:10–21</ref><ref name="von2">von Holstein SL, Therkildsen MH, Prause JU, et al. Lacrimal gland lesions in Denmark between 1974 and 2007. Acta Ophthalmol. 2013;91:349-354</ref> The age of presentation of lacrimal gland tumors depends on the specific subtype of tumor, but overall these tumors can occur at any age. Epithelial tumors, like pleomorphic adenomas and adenoid cystic carcinomas, tend to occur in patients with an average age of 40 years. Lacrimal gland lymphomas, on the other hand, usually present in patients with an average age of 70 years.<ref name="von2"/><ref name="gao4">Gao Y, Moonis G, Cunnane ME, Eisenberg RL. Lacrimal gland masses. AJR Am J Roentgenol 2013 Sep;201(3):W371-81</ref><ref name="shields7">Shields JA, Shields CL, Scartozzi R. Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1. Ophthalmology 2004;111:997–1008.</ref> These neoplasms are generally classified into two types: epithelial and non epithelial. Non epithelial lesions are the most common, corresponding to 70-80% of solid lacrimal gland masses.<ref name="shields1"/><ref name="gao4"/> Of these, lacrimal gland lymphomas are the most common, accounting for almost 37% of malignant lacrimal gland tumors overall.<ref name="rasmussen49">Rasmussen P, Ralfkiaer E, Prause JU, Sjö LD, Siersma VD, Heegaard S. Malignant Lymphoma of the Lacrimal Gland: A Nation-Based Study. Arch Ophthalmol. 2011;129(10):1275–1280. doi:10.1001/archophthalmol.2011.270</ref> The specific types of tumors within each category are outlined in the image below.<ref name="gao4"/><ref name="bernardini5">Bernardini FP, Devoto MH, Croxatto JO. Epithelial tumors of the lacrimal gland: an update. Curr Opin Ophthalmol 2008;19:409–13</ref><ref name="santos6">Santos RR, Damasceno RW, de Pontes FS, Cursino SR, Nishiwaki-Dantas MC, Vital Filho J, Dantas PE. Ten-year follow-up of a case series of primary epithelial neoplasms of the lacrimal gland: clinical features, surgical treatment and histopathological findings. Arq Bras Oftalmol. 2010 Jan-Feb;73(1):33-9</ref> Of the epithelial lacrimal gland tumors, 55% are benign while 45% are malignant.<ref name="shields1"/> The proportional difference may be even greater in Asian populations, with studies reporting up to 72-76% representing benign epithelial tumors.<ref name="chawla9">Chawla B, Kashyap S, Sen S, Bajaj MS, Pushker N, Gupta K, Chandra M, Ghose S. Clinicopathologic review of epithelial tumors of the lacrimal gland. Ophthal Plast Reconstr Surg 2013 Nov-Dec;29(6):440-5</ref><ref name="zeng10">Zeng J, Shi JT, Li B, et al. Epithelial tumors of the lacrimal gland in the Chinese: a clinicopathologic study of 298 patients. Graefes Arch Clin Exp Ophthalmol 2010;248:1345–9</ref> The most common benign epithelial lesion in the lacrimal gland is the pleomorphic adenoma (PA, also called benign mixed tumor). Other less common benign lesions also exist, as outlined in the image below.<ref name="gao4"/><ref name="bernardini5"/> Of the malignant epithelial lesions, adenoid cystic carcinoma (ACC) comprises approximately 60%, with pleomorphic adenocarcinomas, primary adenocarcinomas, mucoepidermoid carcinomas, and miscellaneous tumors making up the remaining 40%.<ref name="shields1"/> This information is summarized in the images below.[[File:Lacrimal_Gland_Tumors_Chart.jpg|frameless|517x517px]][[File:Graph1.jpg|frameless|468x468px]][[File:Graph2.jpg|frameless|468x468px]] == Etiology == Like other neoplasms, lacrimal gland tumors are a result of abnormal cellular division. The specific cells that undergo abnormal proliferation vary based on the type of tumor. See “Histopathology” for the specific cell types involved in each tumor. Risk factors for development of lacrimal gland tumors include age, with the highest risk being between thirty and forty years, history of lymphoma, and incomplete removal of a previous benign tumor.<ref name="cancernet32">(2015, September 23). Lacrimal Gland Tumor. Retrieved from https://www.cancer.net/cancer-types/lacrimal-gland-tumor</ref> Genetic abnormalities have also been implicated in the development of lacrimal gland tumors. A fusion oncogene between MYB and NFIB along with a translocation between chromosome 6q22-23 and chromosome 9pq23-24 has been associated with adenoid cystic carcinoma. MYB is a transcription factor involved in the expression of cell differentiation factors, oncogenes, and housekeeping genes.<ref name="persson34">Persson M, Andren Y, Mark J, Horlings HM, Persson F, Stenman G. Recurrent fusion of MYB and NFIB transcription factor genes in carcinomas of the breast and head and neck. Proc Natl Acad Sci. 2009;106:18740–4.</ref><ref name="mikse35">Mikse, O. R., Tchaicha, J. H., Akbay, E. A., Chen, L., Bronson, R. T., Hammerman, P. S., & Wong, K. K. (2016). The impact of the MYB-NFIB fusion proto-oncogene in vivo. Oncotarget, 7(22), 31681–31688. doi:10.18632/oncotarget.9426</ref> Fusion with NFIB results in significant overexpression of MYB, with subsequent abnormal cellular proliferation.<ref name="persson34"/><ref name="mikse35"/> Secondary metastasis to the lacrimal gland are quite rare but are mostly found to be associated to primary breast and lung carcinomas.<ref name="santos6"/> == Histopathology == '''Dacryops''' is a benign lacrimal duct lesion characterized by lacrimal duct epithelium cysts.<ref name="bernardini5"/>  '''Pleomorphic adenoma (PA)''' is pseudoencapsulated and tends to be well-defined. It is characterized by epithelial and myoepithelial elements that form lumen and spindle structures that are mixed in with other mesenchymatous structures.<ref name="mendoza13">Mendoza PR, Jakobiec FA, Krane JF. Immunohistochemical features of lacrimal gland epithelial tumors. Am J Ophthalmol 2013 Dec;156(6):1147-1158</ref><ref name="von14">von Holstein SL, Rasmussen PK, Heegaard S. Tumors of the Lacrimal Gland. Semin Diagn Pathol. 2016 May;33(3):156-63</ref> [[File:PALG Histology Low Magnification.jpg|frameless|509x509px]] [[File:PALG Histology High Magnification.jpg|frameless|509x509px]] ''© 2019 American Academy of Ophthalmology; Images obtained from AAO: https://eyewiki.aao.org/Pleomorphic_adenoma_(benign_mixed_tumor)_of_the_lacrimal_gland'' '''Adenoid cystic carcinoma (ACC)''' is characterized by solid areas or cords of bland-appearing malignant epithelial cells. The infiltrative borders of the epithelial areas can be distinguished from the surrounding connective tissue and typically show perineural invasion.<ref name="shields1"/> Immunohistochemical staining may be used to identify perineural invasion by identifying neurofilaments.<ref name="mendoza13"/> Adenocarcinoma and PA present myoepithelial cells in the surrounding connective tissue.<ref name="shields1"/><ref name="mendoza13"/> ACC can be further subdivided into histopathologic subtypes: cribriform or 'Swiss cheese', sclerosing, basaloid, comedocarcinoma, and tubular types. [[File:Fig3eyewiki.jpg|frameless|253x253px]] [[File:Trial1.jpg|frameless|328x328px]] ''© 2019 American Academy of Ophthalmology; Images obtained from AAO: https://eyewiki.aao.org/Adenoid_Cystic_Carcinoma_of_the_Lacrimal_Gland'' '''Pleomorphic adenocarcinoma''' or carcinoma-ex-PA is a neoplasm resulting from the malignant transformation of a PA. It is characterized by areas of malignant change in a PA with unequal proportions of myxoid and chondroid structures, the epithelial cells also show carcinomatous changes. In comparison with PA, pleomorphic adenocarcinoma contains less gland structures and the cells show less differentiation. [[File:Pleomorphic Adenocarcinoma.jpg|frameless|338x338px]] ''© 2019 American Academy of Ophthalmology; Image obtained from AAO: Malignant Mixed Tumor of the Lacrimal Gland; Yazdanie, Fahd et al. Ophthalmology, Volume 123, Issue 8, 1736'' '''Primary adenocarcinoma''' also is characterized by glandular differentiation but does not have features that identify more specific carcinoma types. [[File:Primary Adenocarcinoma.jpg|frameless|235x235px]] ''© 2019 American Academy of Ophthalmology; Image obtained from AAO: Primary Ductal Adenocarcinoma of the Lacrimal Gland; Milman, Tatyana et al. Ophthalmology, Volume 112, Issue 11, 2048 - 2051'' '''Mucoepidermoid carcinoma''' contains different proportions of mucous, epidermoid, intermediate, columnar, and clear cells; a cystic component is almost always present.  '''Primary Squamous Cell Carcinoma (SCC)''' is characterized by proliferation of keratinizing malignant squamous cells that are well to moderately differentiated without any glandular structures being present. ''Image not available on AAO; image available in the following publication: Hotta, K. , Arisawa, T. , Mito, H. and Narita, M. (2005), Primary squamous cell carcinoma of the lacrimal gland. Clinical & Experimental Ophthalmology, 33: 534-536. doi:10.1111/j.1442-9071.2005.01043.'' '''Sebaceous gland carcinoma''' is rarely a primary lacrimal gland tumor, most cases are due to secondary involvement from the primary affectation in the eyelids. It is characterized by poorly differentiated cells with high mitoses, with positive staining for lipids contained in vacuoles in the cellular cytoplasm.  '''Benign lymphoepithelial lesion''' can undergo malignant transformation, resulting in B-cell lymphoma or lymphoepithelial carcinoma. Lymphoepithelial carcinoma is characterized by islands of undifferentiated carcinoma cells surrounded by a dense stroma made up of lymphocytes and macrophages. ''<nowiki>Image not available on AAO; image available in the following publication: Svante R. Orell, Jerzy Klijanienko, Chapter 4 - Head and neck; salivary glands, Editor(s): Svante R Orell, Gregory F Sterrett, Orell and Sterrett's Fine Needle Aspiration Cytology (Fifth Edition), Churchill Livingstone, 2012, Pages 38-76, ISBN 9780702031519, https://doi.org/10.1016/B978-0-7020-3151-9.00004-9.</nowiki>'' '''Basal cell adenocarcinoma''' is characterized by uniform basaloid cells without the rest of the elements found in PA. Negative Alcian blue stain and negative reaction to smooth muscle actin can help to distinguish this entity from ACC.<ref name="shields1"/> ''Image not available on AAO; image available in the following publication: Mourad Khalil, Bryan Arthurs, Basal cell adenocarcinoma of the lacrimal gland, Ophthalmology, Volume 107, Issue 1, 2000, Pages 164-168, ISSN 0161-6420, https://doi.org/10.1016/S0161-6420(99)00007-X'' == Classification == The shared embryological origin of the salivary and lacrimal glands has led to the use of the salivary gland classification for lacrimal gland tumors. In 2006, the Armed Forces Institute of Pathology (AFIP) monograph on lacrimal gland tumors provided an expanded classification based on classification of salivary gland tumors from the World Health Organization in 1991.<ref name="weis15">Weis E, Rootman J, Joly TJ, Berean KW, Al-Katan HM, Pasternak S, Bonavolontà G, Strianese D, Saeed P, Feldman KA, Vangveeravong S, Lapointe JS, White VA. Epithelial lacrimal gland tumors: pathologic classification and current understanding. Arch Ophthalmol. 2009 Aug;127(8):1016-28</ref> = Diagnosis = == Clinical History == The presentation of a lacrimal gland tumor depends on its size and location, but generally involves upper eyelid swelling with a mass.<ref name="verity26">26 Verity D.H., Durrani O.M., Rose G.E. (2014) Lacrimal Gland Tumors. In: Perry J., Singh A. (eds) Clinical Ophthalmic Oncology. Springer, Berlin, Heidelberg</ref> As the size of the tumor grows, most lacrimal gland tumors will involve the following characteristics:<ref name="von14">von Holstein SL, Rasmussen PK, Heegaard S. Tumors of the Lacrimal Gland. Semin Diagn Pathol. 2016 May;33(3):156-63</ref><ref name="santos6"/> * Facial asymmetry due to displacement of the globe * Diplopia * Ptosis * Limited ocular motility * Enlargement of the lacrimal gland ''' Pleomorphic Adenomas''' PAs tend to be slow growing over the course of months to years, with the development of a progressive facial asymmetry and painless proptosis.<ref name="bernardini5"/><ref name="perez16">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</ref><ref name="verity26"/> The mean duration of symptoms before initial ophthalmic consultation is approximately 2 years.<ref name="shields1"/> Eventually, as the size of the tumor grows, patients may complain of the general features outlined above, including diplopia and limited ocular motility. If a patient with with long-standing symptoms of a pleomorphic adenoma suddenly experiences pain or an acceleration of symptoms, malignant transformation should be suspected.<ref name="verity26"/><ref name="perzin48">Perzin KH, Jakobiec FA, Livolsi VA, Desjardins L. Lacrimal gland malignant mixed tumors (carcinomas arising in benign mixed tumors): a clinico-pathologic study. Cancer. 1980;45:2593–606.</ref> Lacrimal Gland Lymphomas Lacrimal gland lymphomas tend to present in older patients, after sixty years old, but have been reported in children as well.<ref name="jenkins36">Jenkins, C., et al. (2003). "Clinical features associated with survival of patients with lymphoma of the ocular adnexa." Eye (Lond) 17(7): 809-820.</ref> They occur bilaterally in 25% of cases and 14% present with a prior history of lymphoma. 34% present with systemic lymphoma at diagnosis. Symptoms are similar to those of other lacrimal gland tumors, including mass, diplopia, and extraocular movement restriction.<ref name="jenkins36"/> Malignant Lacrimal Gland Tumors Pain is often suggestive of malignancy and may be secondary to bone or orbital nerve involvement, particularly in patients with adenoid cystic carcinoma.<ref name="shields1"/><ref name="von14"/> Up to 85% of ACC cases show perineural involvement, indicating more aggressive behavior.<ref name="bernardini5"/><ref name="woo17">Woo KI, Yeom A, Esmaeli B. Management of Lacrimal Gland Carcinoma: Lessons From the Literature in the Past 40 Years. Ophthal Plast Reconstr Surg 2016 Jan-Feb;32(1):1-10.</ref> The mean duration of symptoms before initial ophthalmic consultation is 6 months or less for patients with adenoid cystic carcinoma.<ref name="von14"/> == Differential Diagnosis == * [[Dacryops]] * [[Dacryoadenitis]] * [[Dry_Eye_in_Sjogren%27s_Syndrome|Sjogren Syndrome]] Dacryops are lacrimal gland cysts. When the orbital lobe is affected, the consequent proptosis and "S shaped" ptosis may make it difficult to distinguish clinically from other malignant lesions.<ref name="bernardini5"/> Some patients might require surgical removal for symptomatic relief or diagnostic evaluation. Dacryoadenitis is inflammation of the lacrimal gland. This can occur as a result of viral infections, including epstein-barr virus, herpesvirus, and mumps virus.<ref name="rhem37">Rhem MN, Wilhelmus KR, Jones DB. Epstein-barr virus dacryoadenitis. Am J Ophthalmol 2000;129:372-5.</ref><ref name="foster38">Foster WJ Jr, Kraus MD, Custer PL. Herpes simplex virus dacryoadenitis in an immunocompromised patient. Arch Ophthalmol 2003;121:911-3.</ref><ref name="obata39">Obata H, Yamagami S, Saito S, et al. A case of acute dacryoadenitis associated with herpes zoster ophthalmicus. Jpn J Ophthalmol 2003;47:107-9.</ref><ref name="galpine40">Galpine JF, Walkowski J. A case of mumps with involvement of the lacrimal glands. Br Med J 1952;1(4767):1069-70.</ref> Dacryoadenitis has also been described in association with granulomatosis with polyangiitis (formerly Wegener granulomatosis), thyroid ophthalmopathy, Sjögren's syndrome, inflammatory bowel disease, and orbital idiopathic dacryoadenitis.<ref name="kiratli41">Kiratli H, Sekeroglu MA, Soylemezoglu F. Unilateral dacryoadenitis as the sole presenting sign of Wegener's granulomatosis. Orbit 2008;27(3):157-60.</ref><ref name="dutt42">Dutt S, Cartwright MJ, Nelson CC. Acute dacryoadenitis and crohn's disease: Findings and management. Ophthal Plast Reconstr Surg 1992;8(4):295-9.</ref><ref name="witmer43">Witmer, M. (2009, August 13). Unraveling the Difficult Diagnosis of Dacryoadenitis. Retrieved from https://www.reviewofophthalmology.com/article/unraveling-the-difficult-diagnosis-of-dacryoadenitis</ref> Idiopathic cases of dacryoadenitis make up approximately thirty percent of lacrimal gland biopsies.<ref name="shields20">Shields JA, Shields CL, Freire JE, Brady LW, Komarnicky L. Plaque radiotherapy for selected orbital malignancies: preliminary observations: the 2002 Montgomery Lecture, part 2. Ophthal Plast Reconstr Surg 2003 Mar;19(2):91-5</ref> Sjögren syndrome tends to affect both lacrimal glands with diffuse enlargement. The diagnosis includes rose Bengal staining, measurement of tear flow and biopsy of the lacrimal eye gland.<ref name="gao4"/> == Physical examination == A complete ocular exam needs to be done including inspection and palpation of the lacrimal gland, exophthalmometry, and evaluation for ocular displacement. Hypoesthesia in the area of the first and second divisions of the trigeminal nerve are highly suggestive of perineural invasion of orbital nerves.<ref name="shields1"/> == Imaging == Computed tomography (CT) and Magnetic resonance imaging (MRI) are the most important diagnostic imaging methods. Bone changes however, are poorly seen on MRI, making CT the better modality.<ref name="aviv46">Aviv RI, Miszkiel K. Orbital imaging: part 2. Intraorbital pathology. Clin Radiol. 2005;60:288–307.</ref> Benign tumors (pleomorphic adenomas) tend to be well defined with moderate enhancement with IV contrast. Discrete flecks of calcification may also be seen.<ref name="verity26"/> Malignant lesions appear as poorly defined masses with infiltration into surrounding tissue. Calcifications are more common and more diffuse compared to pleomorphic adenomas.<ref name="verity26"/><ref name="wright47">Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol. 1992;76:401–7.</ref> = Management = == General treatment == The management of a suspected lacrimal gland tumor depends on its imaging characteristics. Incisional biopsy should be performed in cases of bone invasion or intraorbital extension.<ref name="verity26"/> Biopsy should not be performed in cases of well-circumscribed masses. These cases should be managed as pleomorphic adenomas.<ref name="verity26"/> Dacryops This is a benign lesion, but symptomatic patients or patients desiring cosmetic improvement can undergo marsupialization or surgical excision.<ref name="dacryops50">(2019, May 4). Dacryops. Retrieved from https://eyewiki.aao.org/Dacryops</ref> Pleomorphic Adenomas Pleomorphic adenomas make up the vast majority of benign lacrimal gland tumors.<ref name="rose31">Rose GE, Wright JE. Pleomorphic adenoma of the lacrimal gland. Br J Ophthalmol. 1992;76:395–400.</ref> The specific management approach for a pleomorphic adenoma varies based on its location but generally involves complete excision. Intraoperative capsule rupture without complete removal is associated with an increased recurrence risk.<ref name="shields1"/> Excision can be approached via an upper lid crease incision or via lateral canthal rhytids.<ref name="verity26"/> Similar management principles apply to other benign lacrimal gland tumors as well. Diagnostic biopsies should be avoided.<ref name="perez16"/> If preoperative incisional biopsy is performed, subsequent treatment with complete surgical removal and excision of adjacent periorbital tissues may greatly reduce chance of recurrence.<ref name="gao4"/><ref name="alkatan19">Alkatan HM, Al-Harkan DH, Al-Mutlaq M, Maktabi A, Elkhamary SM. Epithelial lacrimal gland tumors: A comprehensive clinicopathologic review of 26 lesions with radiologic correlation. Saudi J Ophthalmol 2014 Jan;28(1):49-57</ref> See the EyeWiki page for [[Pleomorphic adenoma (benign mixed tumor) of the lacrimal gland|Pleomorphic Adenomas]]. Adenoid Cystic Carcinoma ACC management consists of tumor debulking followed by external beam radiation.<ref name="wright27">Wright JE, Rose GE, Garner A. Primary malignant neoplasms of the lacrimal gland. Br J Ophthalmol. 1992;76:401–7.</ref> Although both external beam radiation and brachytherapy have been used post-operatively, brachytherapy is not as effective because it fails to prevent recurrence in the superior orbital fissure and cavernous sinus.<ref name="verity26"/> Neoadjuvant chemotherapy delivered via the external carotid artery can be helpful to reduce tumor size prior to surgical removal.<ref name="meldrum28">Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol. 1998;116:315–21.</ref><ref name="tse29">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;141:44–53.</ref> In some patients with residual tumor after surgery, locally delivered radioactive iodine-125 plaque can be considered to avoid the need for externation.<ref name="shields20"/> See the EyeWiki page for [[Adenoid Cystic Carcinoma of the Lacrimal Gland|Adenoid Cystic Carcinoma]]. Mixed Malignant Tumors (Pleomorphic Adenocarcinoma) If the pleomorphic adenoma undergoes malignant degeneration, it is referred to as a mixed malignant tumor. This is managed with local excision and irradiation.<ref name="verity26"/> Primary Adenocarcinoma The recommended treatment for this lesion is complete excision with adjuvant radiotherapy. Additionally, patients should have regional lymph node dissection due to the aggressive nature of this lesion.<ref name="touil52">Touil A, El Abbassi S, Echchikhi Y, Maher M, Kebdani T, Benjaafar N. Adenocarcinoma of the lacrimal gland: a case report. J Med Case Rep. 2017;11(1):257. Published 2017 Sep 11. doi:10.1186/s13256-017-1412-2</ref> Mucoepidermoid Carcinoma Low grade lesions are treated with excision. Radiation is given on a case by case basis. For high grade lesions, patients require externation with radiation.<ref name="hwang53">Hwang SJ, Kim KH. High-grade Mucoepidermoid Carcinoma of the Lacrimal Gland. Korean J Ophthalmol. 2018;32(5):426–427. doi:10.3341/kjo.2018.0026</ref> Primary Squamous Cell Carcinoma This tumor is managed with local resection and radiotherapy.<ref name="hotta54">Hotta, K. , Arisawa, T. , Mito, H. and Narita, M. (2005), Primary squamous cell carcinoma of the lacrimal gland. Clinical & Experimental Ophthalmology, 33: 534-536. doi:10.1111/j.1442-9071.2005.01043.x</ref> Sebaceous Gland Carcinoma This neoplasm is managed with externation. Parotidectomy, cervical lymphadenectomy, and radiotherapy should also be considered.<ref name="briscoe55">BRISCOE D, MAHMOOD S, BONSHEK R, et al. Primary sebaceous carcinoma of the lacrimal glandBritish Journal of Ophthalmology 2001;85:625.</ref> Benign Lymphoepithelial Lesion These lesions are treated with a combination of glucocorticoids and surgical excision.<ref name="svante56">Svante R. Orell, Jerzy Klijanienko, Chapter 4 - Head and neck; salivary glands, Editor(s): Svante R Orell, Gregory F Sterrett, Orell and Sterrett's Fine Needle Aspiration Cytology (Fifth Edition), Churchill Livingstone, 2012, Pages 38-76, ISBN 9780702031519, https://doi.org/10.1016/B978-0-7020-3151-9.00004-9.</ref> Basal Cell Adenocarcinoma Treatment involves surgical excision with margins of healthy tissue.<ref name="mourad57">Mourad Khalil, Bryan Arthurs, Basal cell adenocarcinoma of the lacrimal gland, Ophthalmology, Volume 107, Issue 1, 2000, Pages 164-168, ISSN 0161-6420, https://doi.org/10.1016/S0161-6420(99)00007-X.</ref> Lacrimal Gland Lymphoma Patients with localized disease are treated with radiotherapy, chemotherapy, or a combination of the two. Widespread involvement is generally treated with chemotherapy alone.<ref name="von2"/><ref name="rasmussen49">Rasmussen P, Ralfkiaer E, Prause JU, Sjö LD, Siersma VD, Heegaard S. Malignant Lymphoma of the Lacrimal Gland: A Nation-Based Study. Arch Ophthalmol. 2011;129(10):1275–1280. doi:10.1001/archophthalmol.2011.270</ref> See the EyeWiki page for [[Orbital Lymphoma|Orbital Lymphomas]]. == Prognosis == The prognosis of a lacrimal gland tumor varies based on its classification. Below is prognostic information for the different subtypes of lacrimal gland tumors discussed on this page: Dacryops This is a benign condition, but incomplete resection can result in recurrence.<ref name="dacryops50">(2019, May 4). Dacryops. Retrieved from https://eyewiki.aao.org/Dacryops</ref> Pleomorphic Adenoma The prognosis of PA is generally good, but this type of tumor has the potential to recur locally. While it is a benign neoplasm, it has the potential for malignant transformation. Multiple recurrences increase the risk of malignant transformation, especially in patients older than 45 years.<ref name="von14"/> Adenoid Cystic Carcinoma Lacrimal gland carcinomas are often aggressive, with an average survival of only 36 months. After the initial lesion is treated, the 10-year survival of ACC is approximately 20-30%.<ref name="von14"/> Distant metastasis occurs in 50% of patients with ACC.<ref name="shields1"/> Local recurrence is more likely in cases with perineural invasion, microscopically positive resection margin, and a larger tumor size.<ref name="woo17"/> A solid growth pattern or basaloid differentiation is associated with more aggressive tumors and a reduction in disease-free survival.<ref name="perez16"/> Pleomorphic Adenocarcinoma This malignant lesion is usually well demarcated, but intracranial invasion and spinal metastasis are possible.<ref name="ahn51">Ahn JY, Chang JH, Kim SH, Lee KS. Pleomorphic adenocarcinoma of the lacrimal gland with multiple intracranial and spinal metastases. World J Surg Oncol. 2007;5:29. Published 2007 Mar 7. doi:10.1186/1477-7819-5-29</ref> Primary Adenocarcinoma This is a very aggressive tumor with poor prognosis. Due to the rare nature of this neoplasm, strong prognostic data is lacking, but estimates suggest a death rate of 70% within three years.<ref name="touil52"/> Mucoepidermoid Carcinoma These tumors have poor prognosis with metastasis to the brain, lung, and mediastinum.<ref name="hwang53">Hwang SJ, Kim KH. High-grade Mucoepidermoid Carcinoma of the Lacrimal Gland. Korean J Ophthalmol. 2018;32(5):426–427. doi:10.3341/kjo.2018.0026</ref> Primary Squamous Cell Carcinoma There is insufficient data for prognostic estimates of this tumor. Case reports have reported survival of more than fifteen years.<ref name="hotta54">Hotta, K. , Arisawa, T. , Mito, H. and Narita, M. (2005), Primary squamous cell carcinoma of the lacrimal gland. Clinical & Experimental Ophthalmology, 33: 534-536. doi:10.1111/j.1442-9071.2005.01043.x</ref> Sebaceous Gland Carcinoma This is a rare neoplasm, and prognostic data is scarce. Case reports suggest aggressive behavior, with early metastasis to preauricular and cervical lymph nodes.<ref name="briscoe55"/> Basal Cell Adenocarcinoma This is a relatively low grade malignancy, but metastasis to the lymph nodes and lungs is possible. This rare malignancy lacks sufficient prognostic data, but case reports show survival of over ten years.<ref name="mourad57">Mourad Khalil, Bryan Arthurs, Basal cell adenocarcinoma of the lacrimal gland, Ophthalmology, Volume 107, Issue 1, 2000, Pages 164-168, ISSN 0161-6420, https://doi.org/10.1016/S0161-6420(99)00007-X.</ref> = References = <references /> <ol> <li value="44">Hassan WM, Bakry MS, Hassan HM, Alfaar AS. Incidence of orbital, conjunctival and lacrimal gland malignant tumors in USA from Surveillance, Epidemiology and End</li> <li>Shields JA, Shields CL. Lacrimal gland primary epithelial tumors. In: Shields JA, Shields CL, eds. Eyelid, Orbital and Conjunctival Tumors, An Atlas and Textbook. 2nd ed. Philadelphia, PA: Lippincott, Williams and Wilkins, 2008:700–25</li> <li>Le Tourneau C, Razak AR, Levy C, Calugaru V, Galatoire O, Dendale R, Desjardins L & Gan HK. Role of chemotherapy and molecularly targeted agents in the treatment of adenoid cystic carcinoma of the lacrimal gland. Br J Ophthalmol 2011;95: 1483–1489.</li> <li>von Holstein SL. Tumours of the lacrimal gland. Epidemiological, clinical and genetic characteristics. Acta Ophthalmol 2013 Nov;91 Thesis 6:1-28</li> <li>Huang SK, Tsai CC, Lin CH, Kau HC, Kao SC, Lee FL. Lacrimal gland pleomorphic adenoma masquerading as thyroid-associated ophthalmopathy.Am J Med Sci. 2013 Aug;346(2):162-3. doi: 10.1097</li> <li>Noh JM, Lee E, Ahn YC, Oh D, Kim YD, Woo KI, Ko YH, Kim S. Clinical significance of post-surgical residual tumor burden and radiation therapy in treating patients with lacrimal adenoid cystic carcinoma.Oncotarget. 2016 Sep 13;7(37):60639-60646</li> <li>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</li> <li>Woo KI1, Kim YD, Sa HS, Esmaeli B. Current treatment of lacrimal gland carcinoma. Curr Opin Ophthalmol. 2016 Sep;27(5):449-56.</li> <li>Sant DW, Tao W, Field MG, Pelaez D, Jin K, Capobianco A, Dubovy SR, Tse DT, Wang G. Whole Exome Sequencing of Lacrimal Gland Adenoid Cystic Carcinoma.Invest Ophthalmol Vis Sci. 2017 May 1;58(6)</li> <li>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</li> <li>Heaps RS, Miller NR, Albert DM, et al. Primary adenocarcinoma of the lacrimal gland. A retrospective study. Ophthalmology. 1993;100:1856–60.</li> <li>Tse D. Clinical and microdissection genotyping analyses of the effect of intra-arterial cytoreductive chemotherapy in the treatment of lacrimal gland adenoid cystic carcinoma. Trans Am Ophthalmol Soc. 2005;103:337–67.</li> <li>Nicholas H. Andrew, Daniel Kearney, Nicole Sladden, Penny McKelvie, Albert Wu, Michelle T. Sun, Alan McNab, Dinesh Selva,</li> <li>Idiopathic Dacryoadenitis: Clinical Features, Histopathology, and Treatment Outcomes, American Journal of Ophthalmology, Volume 163, 2016, Pages 148-153.e1, ISSN 0002-9394, https://doi.org/10.1016/j.ajo.2015.11.032. (http://www.sciencedirect.com/science/article/pii/S0002939415300076)</li> </ol>
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