Eye and Ocular Adnexa Manifestations of Warthin Tumor

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 by Michael T Yen, MD on June 28, 2023.

Article Summary

Warthin’s tumor (WT), also known as papillary cystadenoma lymphomatosis or adenolymphoma, is a benign neoplasm that primarily affects the parotid glands (1). It is a rare extra-parotid lesion that can involve structures including the minor salivary glands, throat, mouth or eye (1, 2). WT affecting the ocular structures is known as WT of the eye (WTE) (1).   Within the realm of ophthalmology, there are eight reported cases of WTE documented in the English literature. Four cases involve the caruncle, while there exists one case for each of the following: the plica semilunaris, the lacrimal gland, the lacrimal sac, and the eyelid (1-8). Diagnosis of WTE heavily relies on histology of the excised tissue and, across all cases, treatment involves a local excision of the tumor with no recurrence reported following the procedure.

Disease Entity

D11.9 is the ICD-10 code associated with Warthin’s tumor of the salivary gland. However, there is no specific code for Warthin’s tumor of the eye and ocular adnexa.

History and Prevalence

WT accounts for approximately 4-11% of all salivary gland tumors and is the second most common tumor of the parotid glands (6, 9, 10). However, in rare cases, WT can occur in orbital spaces (1). The first case of WTE was reported in 1963 by Oaks & Jenson in the American Journal of Ophthalmology (3, 4). Upon review of the English literature, only seven other cases of WT have been reported involving the structures of the eye (Table 1).

Risk Factors and Primary Prevention

There is a strong association between Warthin’s tumor of the salivary glands and smoking (8, 13).  However, the association between smoking and WTE is unknown and has not been studied (6). As outlined in Table 1, four cases did not specify the smoking status of the patient with WTE, whereas the other four cases identified their patients as non-smokers.  

Advanced age has been proposed as a risk factor, specifically for development of WT of the lacrimal gland (2). Liu et al., 2019 hypothesized that the increase of age causes the lacrimal caruncle to become hypertrophied and lose its functional capacity, which in turn increases the risk of developing tumors (1).

Table 1: Eight reported cases of WTE.* (M: Male, F: Female, OD: right eye, OS: left eye, +/-: Positive for/Negative for)                          
Publication   Publication Year Age   Gender  


Eye Location Smoking (+/-) Treatment  
Oaks & Jenson 1963 65   F OD Caruncle   Unknown   Surgery  
Carden 1983 70   F OD Plica Semilunaris   Unknown   Surgery  
Mathur et al. 1989 60   M OS Eyelid   - Surgery  
Gupta et al.   1990 60   F OD Lacrimal sac   Unknown   Surgery  
Bonavolontà et al. 1997 62   F OD Lacrimal gland   Unknown   Surgery  
Zepeda & Lai 2013 90   M OD Caruncle   -   Surgery  
Liu et al. 2019 71   F OD Caruncle   - Surgery  
Iacob et al.   2021 76   M OS Caruncle   - Surgery

* Table data modified and derived from Liu et al., 2019 and Iacob et al., 2021.

Affected Patient Populations

Patients with WTE typically present with advanced age (1). There is no clear association between gender and WTE (2).


Warthin’s tumor (WT) is a slowly progressing, benign epithelial tumor (1). WT frequently occurs in the parotid gland and rarely affects extra-parotid tissue (1, 14). These rare sites of localization include the eye, throat, mouth and lips (2, 3, 6, 7, 15, 16, 17). WT commonly demonstrates a history of growth and decline, which is a hallmark feature of the disease (1). Malignant transformation of WT is extremely rare, with a malignancy rate of approximately 1% (18, 19). A change in phenotype of cells through metaplasia to squamous cell differentiation is a well-known feature of WT (20).

Presently, there remains debate around the pathophysiology underlying the distant extraparotid involvement of Warthin’s tumor. For periparotid and cervical involvement, however, the present pathophysiological understanding is that the delayed encapsulation of the parotid gland during embryogenesis permits undifferentiated lymphoid stromal tissue to interact with the developing meshwork of salivary ducts. Once encapsulation is complete, some of the salivary ducts and acini may be trapped within the differentiated extraparotid lymph nodes (21). For the more distant extraparotid Warthin’s tumors (pharynx, larynx, sinuses, lacrimal gland), it is believed that ectopic proliferation of salivary gland tissue in lymph nodes or lymphatic tissue somewhat explains these manifestations (1). In short, more research needs to be done to further elucidate the pathogenesis (1).

Clinical Presentation

Patients with WTE typically present with progressively growing, “fleshy”, painless mass that is partly solid and partly cystic on various sites of the eye, including the caruncle, lacrimal gland and eyelid (4, 5, 8). Patients usually deny eye pain, eye discharge, vision changes or visual disturbances (4, 5, 6, 8). The duration of onset is usually over the course of several months to several years (2, 6, 7).

Making the Diagnosis

  • Physical Examination Findings
    • On gross exam, patients typically present with a spherical, well-encapsulated fibrous nodule at affected site (4). Across the reported cases of WTE, no consistent ophthalmic exam findings are specific or sensitive for WTE. One case report describing WT in the lacrimal caruncle reported diminished visual acuity of affected eye (0.1 in affected eye compared to 0.5 in unaffected eye) (1). Another case of WT of the lacrimal gland noted downward and inward proptosis of the affected eye with mechanical blepharoptosis, with an otherwise unremarkable ophthalmic exam (7). However, the remaining cases of WT affecting the eye do not overtly describe visual acuity findings. With this in mind, ophthalmologists may possibly need to rely more heavily on histology for clinical diagnosis.   
  • Imaging
    • Various imaging modalities can be useful in diagnostic work up for WTE. Magnetic resonance imaging (MRI) can be useful in visualizing any abnormality of the orbit and surrounding structures of the eye (1). Computed tomography (CT) can also be used for lower resolution visualization of the orbit and adjacent structures (4).  
  • Histopathology
    • On histology, WTE is characterized by intertwined islands of bi-layered epithelial tissues with eosinophilic cytoplasm encircled by a background of lymphocytes that demonstrate occasional germinal center formation (8). Biopsy samples of WTE typically demonstrate a lymphocytic component, consisting of lymphocytes and plasma cells, alongside an epithelial component (5, 7). The lymphocytic component is focal with follicular organization, while the epithelial component is formed by solid nests and papillary infoldings of columnar cells (5, 7).

Differential Diagnoses

Oncocytomas of the eye have been reported in the literature, with the highest prevalence occurring in the caruncle (22). It is important to distinguish an oncocytoma from WTE as they are both benign tumors that have similar clinical presentations (5). A histopathologic difference is noted where WTE presents as both as epithelial cells lining cystic spaces and lymphoid tissue while an oncocytoma demonstrates large round epithelial cells with acidophilic cytoplasm (7).


  • Medical Management
    • No medications have been reported to treat WTE (8).  
  • Surgical Management
    • The most effective treatment to date for WTE is local excision. This provides patients with a favorable prognosis (1, 8).
      • Post-Operative Follow-Up
        • The post-excision healing period typically lasts for approximately three weeks (5). For all patients treated with local excision, there were no complaints of pain, discomfort, or tearing (3). These patients were seen for several months after surgery for recurrence monitoring and no recurrence was documented (3, 5, 7, 8).
      • Post-Surgical Complications
        • No complications were reported (8).  


After surgical intervention, patients diagnosed with WTE had a good prognosis and made a full recovery (1, 8).  

Concluding Remarks

WTE is extremely rare and poses very little risk for malignancy (1, 18). However, it is important to biopsy the tissue to confirm the diagnosis and differentiate between benign pathology versus malignant transformation (1). Excision of tumor from ocular and adnexal parts of the eye has proven to be low-risk to the patient and has subsequently provided favorable outcomes to all treated (1).  


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