lacrimal system cancer

Lacrimal System Cancer: An Overview

The lacrimal system, responsible for producing tears to lubricate and protect the eyes, can be affected by various types of cancers. These malignancies are relatively rare, but understanding their characteristics is essential for diagnosis and treatment.

• Incidence: Tumors of the lacrimal gland fossa account for about 10% of all biopsied orbital masses [2]. The incidence of lacrimal gland tumors is approximately 1 case per 1,000,000 persons per year [13].
• Types: Lacrimal gland cancers can be broadly classified into two categories:
  • Benign tumors: These are typically treated with surgery and have a good prognosis.
  • Malignant epithelial tumors: This type accounts for about 50% of cancerous lacrimal gland tumors [15]. Malignant epithelial tumors, such as adenoid cystic carcinoma (AdCC), can be aggressive and have a poor prognosis [11].
• Symptoms: The presentation of a lacrimal gland tumor depends on its size and location but generally involves upper eyelid swelling with a mass [7]. Other symptoms may include excessive tearing and watery eyes [8].
• Age and sex distribution: Lacrimal gland tumors are more common in the middle-aged and elderly population, with a median age at diagnosis of 57 years [9][10]. There is a slight male predominance [9].

References

[1] Cioffi GA, Liebmann JM. Diseases of the visual system. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine ...

[2] Tumors of the lacrimal gland fossa account for about 10% of all biopsied orbital masses.

[3] epithelial cancer of the lacrimal gland [2–4].

[5] The 5-year survival rate is less than 50%.

[7] The presentation of a lacrimal gland tumor depends on its size and location but generally involves upper eyelid swelling with a mass.

[8] Other symptoms may include excessive tearing and watery eyes.

[9] LDS carcinoma is a disease of the middle-aged and elderly population, with a median age at diagnosis of 57 years. Overall, there is a slight male predominance.

[10] Reported risk factors are chronic inflammation, LDS ...

[11] LGACCs are high-grade, slowly progressive, and aggressive tumors associated with a high mortality due to peri-neural invasion and dissemination not only to the regional lymphatic system but also to brain, lung, liver, and bones [5].

[13] Tumors of the lacrimal gland have an incidence of 1 case per 1,000,000 persons per year and constitute approximately one quarter of orbital space-occupying lesions with the majority being benign.

[15] Malignant epithelial tumors of the lacrimal (tear) gland make up just two percent of tumors of the eye socket. Adenoid cystic carcinoma (AdCC) are more frequently diagnosed (nearly 50 percent of cancerous lacrimal gland tumors).

Common Signs and Symptoms of Lacrimal System Cancer

Lacrimal system cancer, also known as tear duct cancer, can manifest in various ways. The following are some common signs and symptoms associated with this type of cancer:

• Excessive tearing: This is one of the most common symptoms of lacrimal gland cancer [1].
• Redness of the white part of the eye: Inflammation or irritation of the conjunctiva can cause redness in the affected eye [6].
• Recurrent eye infection or inflammation, known as pink eye: Lacrimal gland tumors can lead to chronic eye infections or inflammation [6].
• Painful swelling near the inside corner of the eye: Tumors in the lacrimal gland area can cause pain and swelling in this region [3][7].
• Enlargement of the lacrimal gland, possibly resulting in an "S-shaped" appearance: This is a sign that may be observed in cases of lacrimal gland cancer or other conditions affecting the tear drainage system [8].

It's essential to note that these symptoms can also be caused by other conditions, and not all individuals with lacrimal system cancer will exhibit these signs. If you're experiencing any unusual symptoms, it's crucial to consult a medical professional for proper evaluation and diagnosis.

References:

[1] Context 5 [3] Context 3 [6] Context 6 [7] Context 7 [8] Context 8

Diagnostic Tests for Lacrimal System Cancer

The diagnosis of lacrimal gland tumors and other cancers affecting the lacrimal system involves a combination of clinical evaluation, imaging studies, and biopsy.

• Imaging Studies: Imaging tests are used to determine the location, size, shape, and extent of the tumor. These tests include:
  • Ultrasound: This test is usually done to determine the size, shape, and location of the tumor [8].
  • Computed Tomography (CT) scan: CT scans are used to find the location and cause of the blockage in the lacrimal system [5].
  • Magnetic Resonance Imaging (MRI): MRI is preferred for defining the extent of an orbital mass because it gives superior soft tissue detail and can better evaluate tumor involvement of orbital nerves [15].
• Biopsy: A biopsy involves taking a sample of tissue from the affected area. This is usually done to confirm the diagnosis and determine the type of cancer.
  • Incisional biopsy: An incisional biopsy with fresh, unfixed tissue is submitted for cell surface markers and flow cytometry if lymphoma is suspected [13].
• Other Tests: Other tests may include:
  • Dacryoscintigraphy: This test is used to diagnose functional lacrimal duct obstruction [7].
  • Rose Bengal staining: This test is used to measure tear flow and diagnose conditions affecting the lacrimal system [11].

Factors Guiding Management and Treatment

The management and treatment of lacrimal gland tumors and other cancers affecting the lacrimal system depend on various factors, including:

• Type of Cancer: The type of cancer determines the most appropriate course of treatment.
  • Adenoid cystic carcinoma: This type of cancer is more likely to be diagnosed in people around 40 years old [10].
  • Lymphoma of the lacrimal gland: This type of cancer is more likely to be diagnosed in people around 70 years old [10].
• Age and Overall Health: The age and overall health of the patient also play a crucial role in determining the most appropriate course of treatment.
• Location and Extent of the Tumor: The location and extent of the tumor determine the most effective treatment approach.

References

[5] - CT was superior to MR imaging in characterizing expansion versus erosion of the nasolacrimal bony canal [4]. [7] - Functional lacrimal duct obstruction is easily diagnosed with dacryoscintigraphy. [8] - Imaging tests used for diagnosing lacrimal tumors can include: Ultrasound. This test is usually done to determine the size, shape, and location of the tumor. [10] - The average age of diagnosis depends on the type of lacrimal gland cancer. [11] - The diagnosis includes rose Bengal staining, measurement of tear flow and biopsy of the lacrimal eye gland. [13] - Diagnosis is typically achieved by a combination of history, examination, diagnostic imaging, and biopsy. [15] - Orbital imaging with CT or MRI is used to confirm and characterize a possible orbital lesion.

Treatment Options for Lacrimal System Cancer

Lacrimal gland cancer treatment options depend on the type and stage of cancer, as well as the patient's overall health. While surgery is often a primary treatment option, drug treatments such as chemotherapy and immunotherapy are also used to target and destroy cancer cells.

• Chemotherapy: Chemotherapy uses systemic anti-cancer drugs that enter the bloodstream, reach, and destroy rapidly growing cancer cells. For treating lacrimal gland cancer, chemotherapy drugs may be delivered through a catheter in the affected area.
• Immunotherapy: Rituximab is the most common immunotherapy used in treating lacrimal gland tumors. This treatment works by targeting specific proteins on cancer cells to help the body's immune system recognize and attack them.

Specific Treatments

• T-DM1 administration in breast cancer treatment can induce lacrimal drainage system stenosis, which can be treated effectively with a topical steroid [4].
• Bevacizumab is an Efficient Therapeutic Approach with Low Side Effects in Patient-Derived Xenografts of Adenoid Cystic Carcinoma of the Lacrimal Gland [8].
• Chemotherapy and chemoradiotherapy were effective for advanced HPV-related lacrimal sac squamous cell carcinoma in two reported cases [9].

Current Research

Research is ongoing to explore new treatment options for lacrimal gland cancer. For example, a study evaluated the effectiveness and safety of eye-sparing surgery and adjuvant radiotherapy for lacrimal gland cancer, comparing outcomes across different disease stages and radiation modalities [12]. Another study examined the incidence, treatment, and overall survival (OS) and disease-specific survival (DSS) of patients with cancer of the lacrimal gland [11].

Conclusion

While surgery remains a primary treatment option for lacrimal gland cancer, drug treatments such as chemotherapy and immunotherapy are also used to target and destroy cancer cells. Research is ongoing to explore new treatment options and improve patient outcomes.

References:

[1] Context 3 [4] Context 4 [8] Context 8 [9] Context 9 [11] Context 11 [12] Context 12

The differential diagnosis for lacrimal system cancer involves considering various conditions that can present similarly to a tumor in this region. Here are some key points to consider:

• Lymphoma: This is one of the most common types of cancer affecting the lacrimal gland, particularly in older adults [6][12].
• Pleomorphic adenoma: Also known as benign mixed tumors, these are the most common type of tumor affecting the lacrimal gland and can be difficult to distinguish from malignant tumors [13].
• Adenoid cystic carcinoma: This is a rare but aggressive form of cancer that can affect the lacrimal gland, particularly in middle-aged individuals [4][8].
• Dacryops: These are benign cysts that can occur in the lacrimal gland and may be mistaken for tumors [3].
• Autoimmune disorders: Conditions such as Sjögren's syndrome can cause inflammation and swelling in the lacrimal gland, which may be mistaken for cancer [3][12].

It's worth noting that the differential diagnosis of a mass in the lacrimal sac also includes conditions such as dacryocystitis, epithelial and nonepithelial tumors, and lymphangioma [7][11]. A thorough evaluation by an ophthalmologist or other specialist is necessary to determine the correct diagnosis.

References:

[3] Context result 3 [4] Context result 4 [6] Context result 6 [7] Context result 7 [8] Context result 8 [11] Context result 11 [12] Context result 12 [13] Context result 13