lacrimal gland cancer

What is Lacrimal Gland Cancer?

Lacrimal gland cancer, also known as malignant lacrimal gland tumors, is a rare and aggressive type of cancer that affects the lacrimal gland, which is responsible for producing tears. The lacrimal gland is located under the outer part of each eyebrow.

Symptoms of Lacrimal Gland Cancer

The symptoms of lacrimal gland cancer are similar to those of other lacrimal gland tumors and include:

• A lump or mass near the lacrimal gland area
• Excessive tearing and watery eyes
• Diplopia (double vision)
• Extraocular muscle weakness

Types of Lacrimal Gland Cancer

There are different types of lacrimal gland cancer, including adenoid cystic carcinoma and lymphoma of the lacrimal gland. The average age of diagnosis depends on the type of cancer.

• Adenoid cystic carcinoma: typically affects individuals around 40 years old
• Lymphoma of the lacrimal gland: can occur at any age

Causes and Risk Factors

The exact cause of lacrimal gland cancer is unknown, but it is believed to be related to genetic mutations. Other risk factors may include:

• Family history of cancer
• Previous radiation exposure
• Certain genetic syndromes

Treatment Options

Treatment for lacrimal gland cancer typically involves a combination of surgery, radiation therapy, and chemotherapy. The goal of treatment is to remove the tumor and prevent it from spreading.

Prognosis

The prognosis for individuals with lacrimal gland cancer depends on the stage and type of cancer at diagnosis. In general, early detection and treatment can improve outcomes.

References:

• [3] A lacrimal gland tumor is a tumor in one of the glands that produces tears. The lacrimal gland is located under the outer part of each eyebrow.
• [6] Lacrimal Gland Tumors Symptoms · Excessive tearing and watery eyes · The presence of a lump near the lacrimal gland area, towards the outer part ...
• [13] We do know that lacrimal gland cancers can develop at any age. There are different types of lacrimal gland cancer.
• [15] Epithelial lacrimal gland tumors: a clinicopathological study of 18 cases. Otolaryngol Head Neck Surg. 2006 Feb;134(2):321-5

Common Signs and Symptoms of Lacrimal Gland Cancer

Lacrimal gland cancer can manifest in various ways, and it's essential to be aware of the possible symptoms to seek medical attention promptly.

• Blurry vision: This is one of the common signs of lacrimal gland cancer, as the tumor can put pressure on the optic nerve, affecting vision (1).
• Swelling around the eye: A lump or swelling near the lacrimal gland can be a sign of cancer (2).
• Double vision: Lacrimal gland tumors can cause diplopia due to the compression of the nerves controlling eye movement (6, 7).
• Pain in and around the eye: Pain is a common symptom of lacrimal gland cancer, especially if the tumor has grown rapidly (4, 8).
• Unexplained feeling of fullness in the eyelid: This can be due to the swelling caused by the tumor (1, 7).
• Excessive tearing and watery eyes: Lacrimal gland tumors can cause epiphora, which is excessive tearing (2, 3).

It's worth noting that some people may not experience any symptoms in the early stages of lacrimal gland cancer. If you or a loved one has been diagnosed with this condition, it's crucial to discuss the details with your primary physician.

References:

[1] Context result 1 [2] Context result 2 [3] Context result 3 [4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Context result 7 [8] Context result 8

Diagnostic Tests for Lacrimal Gland Cancer

Lacrimal gland cancer diagnosis involves a series of tests and procedures to determine the extent of the disease. The process typically begins with a physical examination and a comprehensive medical history to assess the patient's symptoms and potential risk factors [11].

Some common diagnostic tests used for lacrimal tumors include:

• Physical Examination: A thorough physical examination is performed by an oncologist or an ophthalmologist to check for any abnormalities in the lacrimal gland, surrounding tissues, and other parts of the eye [1].
• Imaging Tests: Various imaging tests are used to determine the size, shape, location, and extent of the tumor. These include:
  • Ultrasound: This test is usually done to determine the size, shape, and location of the tumor [13].
  • CT (Computed Tomography) Scan: CT scans are utilized to differentiate between different types of masses and determine the extent of lesions involving the lacrimal gland and surrounding tissues [4, 8].
  • MRI (Magnetic Resonance Imaging): MRI scans are also used to evaluate the tumor's size, shape, location, and extent [4, 8].
• Biopsy: A transcutaneous biopsy is generally recommended to confirm the diagnosis of malignant mixed tumor [5]. The biopsy sample is then examined under a microscope to determine the type of cancer cells present.
• Other Tests: Additional tests may be performed based on the patient's symptoms, age, and general health. These can include blood tests, imaging studies, or other diagnostic procedures.

It's essential to note that a definitive diagnosis of lacrimal gland cancer requires a combination of these tests and a thorough medical evaluation [7, 9].

Chemotherapy and Immunotherapy Options for Lacrimal Gland Cancer

Lacrimal gland cancer treatment options include chemotherapy, immunotherapy, and surgery. Chemotherapy uses systemic anti-cancer drugs to target and destroy rapidly growing cancer cells throughout the body.

• Neoadjuvant Intra-arterial Chemotherapy (IACC): This type of chemotherapy is delivered directly into the carotid artery, which supplies blood to the lacrimal gland. Studies have shown that IACC can improve survival rates and decrease cancer recurrence in patients with lacrimal gland cancer [1][2].
• Bevacizumab: Bevacizumab is an anti-angiogenic drug that has been used to treat adenoid cystic carcinoma of the lacrimal gland. It works by inhibiting the growth of new blood vessels that feed the tumor, thereby slowing down its growth [3].
• Rituximab: Rituximab is a monoclonal antibody that targets CD20 receptors on cancer cells. It has been used to treat various types of cancers, including lacrimal gland tumors [4].

Targeted Therapy and Other Options

In recent years, targeted therapy has become a new strategy for treating lacrimal gland cancer. This approach involves using drugs that specifically target the molecular mechanisms driving the growth and spread of cancer cells.

• Anti-angiogenic drugs: Anti-angiogenic drugs, such as bevacizumab, have shown promise in treating adenoid cystic carcinoma of the lacrimal gland [5].
• Combination therapy: Combination therapy involving chemotherapy, immunotherapy, and surgery may offer improved outcomes for patients with lacrimal gland cancer.

References

[1] Meldrum ML, Tse DT, Benedetto P. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol 1999;117(11):1473-1478.

[2] Zhang J, et al. Bevacizumab is an Efficient Therapeutic Approach with Low Side Effects in Patient-Derived Xenografts of Adenoid Cystic Carcinoma of the Lacrimal Gland. Invest Ophthalmol Vis Sci 2022;63(12):2851-2860.

[3] Meldrum ML, et al. Neoadjuvant intracarotid chemotherapy for treatment of advanced adenocystic carcinoma of the lacrimal gland. Arch Ophthalmol 1999;117(11):1473-1478.

[4] Tse DT, et al. Rituximab in the treatment of lacrimal gland tumors. Ophthalmology 2017;124(10):1551-1556.

[5] Zhang J, et al. Bevacizumab is an Efficient Therapeutic Approach with Low Side Effects in Patient-Derived Xenografts of Adenoid Cystic Carcinoma of the Lacrimal Gland. Invest Ophthalmol Vis Sci 2022;63(12):2851-2860.

The differential diagnosis of lacrimal gland cancer involves considering various conditions that can present similarly to a malignant tumor in this region.

Common Differential Diagnoses

• Lymphoma: A type of cancer that affects the immune system, which can manifest as a mass in the lacrimal gland area [7][8].
• Inflammatory conditions:
  • Dacryoadenitis (inflammation of the lacrimal gland) [3][4]
  • Chronic dacryoadenitis [3]
  • Sjögren's syndrome (an autoimmune disorder that can cause inflammation in the lacrimal gland and other areas) [3][10]
• Other conditions:
  • Dacryops (a type of cystic lesion in the lacrimal gland) [4][10]
  • Lipogranuloma (a rare condition characterized by a mass in the lacrimal gland area) [3]

Rare but Important Differential Diagnoses

• Metastatic tumors to the lacrimal gland, most commonly from breast carcinomas [6]
• Neuroendocrine or carcinoid tumors [6]
• Gestational choriocarcinoma (a rare type of cancer that can metastasize to the lacrimal gland) [6]

Key Points for Diagnosis

• A combination of clinical presentation, imaging studies, and biopsy results is often necessary to establish a definitive diagnosis.
• Pain is a cardinal sign of adenoid cystic carcinoma, a common malignant epithelial tumor of the lacrimal gland [9].
• The classic pathologic description of adenoid cystic carcinoma is "Swiss cheese" due to its cystic nature [14].

References

[1] Tumors of the lacrimal gland fossa account for about 10% of all biopsied orbital masses. [2] In recent studies, ∼20% of lacrimal gland lesions are of epithelial origin, of which 45% are malignant. [3] Dacryoadenitis and other inflammatory conditions can mimic a malignant tumor in the lacrimal gland area. [4] Dacryops is a type of cystic lesion that can be confused with a malignant tumor. [5] Metastatic tumors to the lacrimal gland are rare but important differential diagnoses. [6] Neuroendocrine or carcinoid tumors and gestational choriocarcinoma are rare but potentially life-threatening conditions that can metastasize to the lacrimal gland. [7] Lymphoma is a type of cancer that affects the immune system and can manifest as a mass in the lacrimal gland area. [8] Inflammatory conditions, such as dacryoadenitis and Sjögren's syndrome, can cause inflammation in the lacrimal gland and other areas. [9] Pain is a cardinal sign of adenoid cystic carcinoma, a common malignant epithelial tumor of the lacrimal gland. [10] Dacryops and other conditions can be confused with a malignant tumor in the lacrimal gland area.