What is Benign Lacrimal Gland Tumors?
Tumors in the lacrimal gland fossa, which is an area near the eye, make up about 10% of all orbital masses, or lumps found in the eye area. These tumors could be due to a variety of causes such as infections, inflammation, or they could be neoplastic, meaning made up of abnormal cells. These abnormal cell tumors could be either harmless (benign) or dangerous (malignant). This discussion will focus on the harmless or benign tumors.
Since this area is located near the top front side of the orbit, or eye socket, any lumps in this area usually cause the eye to move downwards and inwards. Protruding of the eye isn’t always seen, but it can occur if a lump grows backwards and pushes the eye forwards. Most benign tumors grow slowly and are painless, and as a result, these changes could appear over a long time, usually 1 to 2 years before they are even diagnosed. This is a lot longer compared to the average 6 months or less it takes to diagnose malignant, or dangerous, lumps.
Many patients report their initial concern as a vague unevenness in the middle part of their face. Sometimes, tumors in the lacrimal gland are discovered unexpectedly during a regular eye check-up or through a scan for a different problem.
What Causes Benign Lacrimal Gland Tumors?
Benign tumors or noncancerous growths in the tear gland or lacrimal gland can be categorized based on their cell origins.
Some of these cell origins include:
Epithelial Tumors – These are the most common type of tumor. About half of them are not harmful. The most frequent benign ones come from a variety known as pleomorphic adenomas or benign mixed tumors; they are usually found in the orbital lobe of the tear gland. Another common benign tumor is the lacrimal gland cyst, also named dacryops or tear duct cyst. While it’s usually on the eyelid’s lobe of the tear gland, it can also occur in the orbital lobe. These cysts are typically idiopathic, meaning their cause is unknown, but some have been tied to scarring and trachoma, chemical injury, or ocular cicatricial pemphigoid. Less common epithelial lesions consist of oncocytoma, myoepithelioma, cystadenoma, and Warthin tumor.
Lymphoid Tumors – Lymphoproliferative lesions of the orbit are usually found in or around the lacrimal gland. The benign growth of lymphoid tissue is also known as reactive lymphocytic hyperplasia or benign lymphoepithelial lesion, making up around 6% of all biopsied orbital masses. In some instances, lymphoid hyperplasia has been linked with the disease known as IgG-4-related disease.
Mesenchymal Tumors – These types of tumors in the lacrimal gland are mainly vascular, like mass vessels. The most common type is angiolymphoid hyperplasia with eosinophilia. However, other types like capillary and cavernous hemangiomas and epithelioid hemangioendothelioma are also seen. Other mesenchymal tumors include granular cell tumors, fibrous histiocytoma, solitary fibrous tumor/hemangiopericytoma, neurofibroma (sometimes with systemic neurofibromatosis type I), and schwannoma.
There are also other types of benign tumors that are not listed here, but they are quite rare.
Risk Factors and Frequency for Benign Lacrimal Gland Tumors
Benign lacrimal gland tumors typically surface as people approach their middle age, although they can develop at any age. These kinds of tumors don’t prefer one gender over the other. Since these tumors are rare, specific risk factors aren’t known. However, some diseases like neurofibromatosis type I may increase the chances of certain related tumors.
- Benign lacrimal gland tumors can occur at any age but are common in middle-aged adults.
- These tumors do not favor any gender.
- The specific risk factors for these tumors aren’t established because these tumors are quite rare.
- Systemic disorders like neurofibromatosis type I can increase the risk of certain lesions.
Signs and Symptoms of Benign Lacrimal Gland Tumors
When diagnosing a condition, it’s important to gather some basic information about the patient such as their age, gender, race, and ethnicity. Medical history is another key piece of information. This includes knowledge about any recent illnesses, autoimmune or inflammatory diseases, histories of cancer, chemotherapy, or radiation, immune system problems, or previous trauma or surgeries. Additionally, investigating their symptoms can reveal connections to autoimmune or inflammatory diseases.
For eye-related symptoms, doctors will ask about the following:
- Blurry vision
- Double vision
- Changes in appearance or facial symmetry
- Pain
- Light sensitivity
- Pain with eye movement
- Swelling or redness of the eyelid
- Discharge
- Tenderness
- Tearing and dryness
Old patient photographs might also be used to spot any changes in the position of the eyes or eyelids.
An eye specialist should then perform a comprehensive eye exam. This checks for things like vision quality, pupil responses to light, color vision, and eye movement. It will also check the position of the eyes, especially if there are concerns about lacrimal gland tumors, and whether there is any resistance when the eye is moved. The doctor might also check for any masses by feeling around the eyelid or orbit, and for any swelling of the lymph nodes. A physical eye exam can also reveal lid swelling, redness, warmth, or a visibly enlarged and tender lacrimal gland. The white part of the eye may be red, particularly over the area of the lacrimal gland. The doctor also checks the front and back sections of the eye to look for signs of uveitis.
Testing for Benign Lacrimal Gland Tumors
Determining whether a growth is harmless (benign) or cancerous (malignant) can be difficult using only a routine examination. Some possible indicators include the rate of growth, as benign lesions often grow slower, and the presence of pain – certain types of cancer, like adenoid cystic carcinoma, can invade the nerve pathways causing pain. To confirm and understand the nature of the suspected growth further, doctors often use imaging techniques. These can tell us whether the growth is solid or fluid-filled (cystic), clearly outlined or spreading into nearby areas, present with any calcium deposits, and whether it has affected any surrounding bone or tissue.
A computed tomography (CT) scan, which is quick, affordable, and widely available, is often used first – particularly when a benign growth is suspected. CT scans can show us the size of the growth, its relation to neighboring structures like bones and muscles, and can pick up any calcium deposits within the growth, which can help identify it.
Magnetic Resonance Imaging (MRI), which provides more detailed images of soft tissues, is more expensive, takes longer, and is less accessible. However, it’s excellent at mapping how the growth has affected any orbital nerves – a crucial piece of information for patients with adenoid cystic carcinoma, a type of cancer that often invades nerves. Unlike CT scans, MRI scans don’t give a clear view of bones.
In some cases, doctors might use both imaging techniques and carry out a biopsy before deciding on the next step of treatment.
Another thing to consider is that evidence of the PLAG1 mutation has been connected to salivary gland tumors; so this should be screened for during evaluations.
Treatment Options for Benign Lacrimal Gland Tumors
Most tumors require some form of surgical intervention, whether it’s a biopsy or removal, as there aren’t many medical treatments available. The significant exception is lymphoid hyperplasia, which we’ll talk about a bit later. Some small, stable, or slow-growing tumors might not need immediate surgery, especially if they’re not suspected of being cancerous or if the patient isn’t well enough for an operation.
Pleomorphic adenoma is a type of tumor that’s generally treated with complete surgical removal, including the pseudo-capsule surrounding it. Surgeons can reach the tumor and take it out through an operation at the front or side of the orbit, sometimes even removing the bone at the side of the orbit for complete access. It’s crucial to delicately handle the tumor and avoid rupturing its capsule; if not removed fully, there could be chances of the tumor re-growing or becoming malignant.
Many benign and well-defined lesions are also often completely removed, like pleomorphic adenoma. Biopsies, where a small piece of the tumor is removed for examination, might be considered in unclear cases. Even with pleomorphic adenomas, the risk of recurrence or transition to cancer is very low if the surgeon also removes the pathway used for surgery during the tumor removal.
A type of cyst known as dacryops can be observed over time without immediate intervention. However, as it grows, it might cause double vision, infections, or cosmetic concerns, after which it can be surgically removed. The common surgical approach is through the conjunctiva, the clear tissue covering the eye, ensuring preservation of the tear gland ducts in the process. Draining or aspirating the cyst is usually a temporary measure as they tend to refill over time. If the cyst is associated with a disease called ocular cicatricial pemphigoid, a less invasive approach might be considered to minimize damage to the conjunctiva.
Lymphoid hyperplasia, the exception mentioned earlier, is treated quite differently. Instead of surgery, it’s typically treated with high-dose steroids, sometimes alongside radiation therapy. In certain cases, a drug named rituximab might be used to manage these proliferating lesions. Surgical removal of these lesions is rare.
What else can Benign Lacrimal Gland Tumors be?
In addition to harmless growths, other conditions may also cause changes in the lacrimal gland, which is responsible for making tear fluid. These conditions fall into two groups – inflammatory conditions and cancerous growths. Inflammatory conditions are the most frequent cause of swelling in the lacrimal gland. The most frequent among these is ‘dacryoadenitis’, which can affect one or both glands, and could be due to an inflammation or an infection.
Inflammatory Conditions:
- Orbital pseudotumor or nonspecific orbital inflammation
- IgG-4 related disease
- Sarcoidosis
- Granulomatosis with polyangiitis
- Dacryoadenitis
- Sjogren syndrome
- Thyroid eye disease
Cancerous Growths:
- Adenoid cystic carcinoma
- Lymphoma
- Ductal adenocarcinoma
- Metastasis
There are also certain conditions often mistaken for lacrimal gland tumors:
- Epidermoid or dermoid cysts usually found at the frontozygomatic suture with an intraorbital component
- Prolapsed orbital fat leading to fullness of the upper lid on the side or a visible mass in the upper, outer part of the eyeball
- Dermatolipoma is typically seen in the upper, outer part of the conjunctiva
- The prolapsed lacrimal gland which appears as though it has been displaced by a growth
In rare instances, changes to the lacrimal gland can also be seen in a condition called ‘amyloidosis’. Amyloidosis refers to the buildup of a particular type of protein in the body’s organs or tissues. However, if it affects the lacrimal gland or the eye orbit, it’s usually a local issue rather than one related to systemic amyloidosis, which affects the entire body.
What to expect with Benign Lacrimal Gland Tumors
The outlook for benign tumors, or non-cancerous growths, is typically positive. However, these tumors can cause health issues because they take up space and impact nearby tissues. For large tumors, they may cause an irregular appearance due to pushing out or shifting the eyeball. This displacement can also lead to vision changes.
Vision can become blurry if the tumor directly presses on the eye and alters its shape. When pressure is applied to the optic nerve, it can result in optic neuropathy, affecting the sharpness of vision, color perception, and visual field.
Sometimes, the tumors can be associated with generalized diseases, such as a neurofibroma with neurofibromatosis type 1. In these cases, the overall outlook depends on the severity of the generalized disease.
In regards to a specific type of tumor called pleomorphic adenoma, when it recurs, it may turn into a malignant (cancerous) tumor. This deterioration can negatively impact both the vision and overall health prognosis. This risk tends to increase with multiple recurrences and advanced patient age.
Dacryops, another type of benign tumor, have a high chance of returning if treated with simple aspiration and drainage. Though surgical removal can reduce this risk, the tumors can still recur if not entirely removed during the surgery.
Possible Complications When Diagnosed with Benign Lacrimal Gland Tumors
The complications associated with benign tumors largely result from the pressure they put on the tissues around them. If a surgical biopsy or removal of the tumor is carried out, there can be complications related to the surgery itself and the methods used during the operation.
For operations involving the front or side of the eye socket, often referred to as anterior or lateral orbitotomies, complications can include a variety of issues. Constant monitoring after eye socket surgery is important to quickly address these potentially sight-threatening complications.
- Bleeding in the eye socket
- Inflammation in the eye socket after surgery
- Damage to the muscles controlling eye movement, the eyeball itself, or the optic nerve
- Dryness due to reduced tear gland function
- Leakage of cerebral spinal fluid
- Return of the tumor
Preventing Benign Lacrimal Gland Tumors
Treating tumors in critical areas like the orbit (eye socket) can be incredibly complex. It’s important to have a detailed conversation with the patient about the possible causes, treatment options, potential side effects, and the expected course of their disease. If cancer is one of the possibilities, the medical staff needs to approach the patient with kindness and understanding. The patient might need several conversations to fully grasp the situation and make a well-informed decision.
All the risks, benefits, and alternatives to each treatment, including no treatment at all, need to be clearly explained. The emotional and psychological effects of such a diagnosis, as well as the related treatments (like surgery, chemotherapy, radiation, and long-term monitoring), could be significant, especially if complications occur.
This might require a team approach involving a primary care doctor, cancer specialist (oncologist), psychologist, or psychiatrist to give the patient the best possible care and support.
