mixed lacrimal gland cancer

Mixed Lacrimal Gland Cancer: A Rare and Malignant Tumor

Mixed lacrimal gland cancer, also known as malignant mixed tumor or pleomorphic adenocarcinoma, is a rare and aggressive type of cancer that arises from the epithelial cells of the lacrimal gland. This type of cancer accounts for approximately 20% of all primary epithelial malignancies of the lacrimal gland (PEML) [13].

Characteristics

Mixed lacrimal gland cancer typically presents as a slow-growing mass in the lacrimal gland fossa, with symptoms similar to those of other lacrimal gland tumors, including diplopia and extraocular muscle dysfunction [2]. The tumor can contain a mix of various tissue types, including epithelial, connective tissue, and cartilage [4].

Clinical Features

The clinical features of mixed lacrimal gland cancer include:

• Recent rapid onset of symptoms (orbital protrusion, eyelid swelling) [9]
• Localized swelling, globe displacement, diplopia, and ptosis [7]
• Pain is a cardinal sign of adenoid cystic carcinoma, but not typically associated with mixed lacrimal gland cancer [7]

Prognosis

The prognosis for patients with mixed lacrimal gland cancer is generally poor, with survival intervals after the initial operation varying depending on whether the tumor has spread to other parts of the body [14]. Long-term follow-up data have emphasized the malignant nature of this neoplasm.

Incidence

Mixed lacrimal gland cancer accounts for approximately 1 case per 1,000,000 persons per year, making it a rare and uncommon type of cancer [15].

References:

[4] - Context result 4 [7] - Context result 7 [9] - Context result 9 [13] - Context result 13 [14] - Context result 14 [15] - Context result 15

Common signs and symptoms of mixed lacrimal gland cancer may include:

• Blurry vision [1]
• Eye swelling or protrusion
• Excessive tearing and watery eyes
• The presence of a lump near the lacrimal gland area, towards the outer part of the eyelids [3]

Additionally, some people may experience:

• A painful, tender swelling in the lacrimal gland area
• An S-shaped deformity of the upper lid
• Conjunctival redness and injection (Fig. ...) [5]
• Recent rapid progression of lateral upper eyelid swelling and/or proptosis
• The presence of pain
• Evidence of bony destruction [8]

It's worth noting that these symptoms can also be indicative of other conditions, and a biopsy is usually needed to confirm the diagnosis.

Pain is not always a reliable indicator of malignancy, as benign lacrimal gland tumors can also sometimes induce mild pain. However, symptoms such as blepharoptosis, ocular motility disturbance, and diplopia are more likely to occur in malignant tumors [15].

References:

[1] Context result 1 [3] Context result 3 [5] Context result 5 [8] Context result 8 [15] Context result 15

To diagnose mixed lacrimal gland cancer, several diagnostic tests can be employed.

Imaging Tests

• Ultrasound: This test is often used to determine the size, shape, and location of the tumor. It can help identify any abnormalities in the lacrimal gland.
• CT (Computed Tomography) scans: CT scans are utilized to differentiate between different types of masses and determine the extent of lesions involving the lacrimal gland.
• MRI (Magnetic Resonance Imaging): MRI is also used to evaluate the tumor's size, shape, and location.

Biopsy

• Fine-needle aspiration biopsy (FNAB): FNAB or incisional biopsy should be considered for all epithelial tumors of the lacrimal gland. This test involves taking a sample of tissue from the tumor using a fine needle.
• Histology: The diagnosis ultimately relies on histology, as does the choice of treatment and the prognosis.

Other Tests

• Complete ocular exam: A complete ocular exam is necessary to evaluate any potential complications associated with lacrimal gland tumors. This includes inspection and palpation of the lacrimal gland, exophthalmometry, and evaluation for ocular symptoms.
• Rose Bengal staining: Rose Bengal staining can be used to assess tear flow and diagnose conditions such as Sjögren syndrome.

It's essential to note that the choice of diagnostic tests may vary depending on individual factors and the specific characteristics of the tumor. A cancer care team should be consulted to determine the best course of action for diagnosis and treatment.

References:

• [3] Sjögren syndrome tends to affect both lacrimal glands with diffuse enlargement.
• [4] by JS Kim · 2021 · Cited by 29 — Some authors propose that fine-needle aspiration biopsy (FNAB) or incisional biopsy should be considered for all epithelial tumors of the lacrimal gland ...
• [7] Imaging tests used for diagnosing lacrimal tumors can include: Ultrasound.
• [8] Lacrimal gland cancers are usually initially diagnosed clinically based on imaging and later confirmed with biopsy. Biopsy is ordinarily only obtained after ...

Treatment Options for Mixed Lacrimal Gland Cancer

Mixed lacrimal gland cancer, also known as malignant mixed tumor or carcinoma ex pleomorphic adenoma, is a rare and aggressive type of cancer that affects the lacrimal gland. While surgery and radiation therapy are often used to treat this condition, drug treatment options are also available.

Chemotherapy

Chemotherapy uses systemic anti-cancer drugs to target and destroy cancer cells. For mixed lacrimal gland cancer, chemotherapy may be used as a standalone treatment or in combination with other therapies such as radiotherapy (chemoradiotherapy). The most commonly used chemotherapy drugs for this condition include:

• Cisplatin [6]
• Doxorubicin-based concurrent chemoradiotherapy [6]
• Apatinib and nedaplatin were also successful in treating lacrimal gland tumors [6]

Immunotherapy

Immunotherapy is a type of treatment that uses the body's immune system to fight cancer. For mixed lacrimal gland cancer, rituximab is the most common immunotherapy used in treating this condition [7].

Neoadjuvant Intracarotid Chemotherapy

Neoadjuvant intracarotid chemotherapy involves delivering chemotherapy directly into the carotid artery to target the tumor. This treatment option has been shown to be effective in treating advanced adenocystic carcinoma of the lacrimal gland [8].

Contemporary Strategies

In recent years, contemporary strategies such as orbit sparing surgeries and neoadjuvant intra-arterial chemotherapy have gained popularity in treating mixed lacrimal gland cancer. However, these treatments remain controversial due to the limited availability of data [10].

It's essential to note that the optimal treatment for mixed lacrimal gland cancer remains unclear, and more research is needed to determine the most effective treatment options [11].

The differential diagnosis for mixed lacrimal gland cancer, also known as pleomorphic adenocarcinoma, can be challenging due to its varied presentation and potential overlap with other conditions.

Possible Differential Diagnoses:

• Benign Mixed Epithelial Tumor: This is a non-cancerous tumor that arises in the cells lining the lacrimal gland. It can present similarly to pleomorphic adenocarcinoma, making it a differential diagnosis (7).
• Malignant Mixed Epithelial Tumor: This is a rare and aggressive type of cancer that affects the lacrimal gland. It can be difficult to distinguish from pleomorphic adenocarcinoma based on imaging alone (9).
• Adenoid Cystic Carcinoma: This is a type of cancer that can affect the lacrimal gland, but it has distinct histological features that may help differentiate it from pleomorphic adenocarcinoma. Imaging studies and biopsy are often required for definitive diagnosis (5).

Other Conditions to Consider:

• Lymphomatous lesions: These can range from reactive hyperplasia to malignant lymphoma and can be difficult to differentiate from cancer of the lacrimal gland, especially in early stages (6).
• Sjögren syndrome: This is an autoimmune disorder that can cause enlargement of both lacrimal glands. It may present similarly to pleomorphic adenocarcinoma, but a complete ocular exam and biopsy are often required for diagnosis (10).

Imaging Studies:

Computed tomography (CT) and magnetic resonance imaging (MRI) are essential tools in evaluating the signs and symptoms related to a lacrimal gland-origin neoplasm. These studies can help narrow down the differential diagnosis, but may not always provide a definitive answer (12).

In conclusion, the differential diagnosis for mixed lacrimal gland cancer is complex and requires careful consideration of various conditions, including benign and malignant tumors, lymphomatous lesions, and autoimmune disorders. A combination of clinical evaluation, imaging studies, and biopsy may be necessary to arrive at an accurate diagnosis.

References:

• [7]
• [5]
• [6]
• [10]