salivary gland adenoid cystic carcinoma

What is Adenoid Cystic Carcinoma of the Salivary Glands?

Adenoid cystic carcinoma (ACC) is a rare and uncommon type of cancer that primarily affects the salivary glands or other areas of the head and neck. It can also occur in other parts of the body, such as the breast, skin, cervix, prostate gland, and various other areas.

Characteristics and Symptoms

• ACC tumors are typically solid, round, and hollow.
• Early lesions present as painless masses in the mouth or face, growing slowly.
• Advanced tumors may cause pain and/or nerve involvement.
• The tumor can affect both major and minor salivary glands.

Incidence and Prevalence

• ACC accounts for approximately 1% of all malignancies of the head and neck region.
• It is the most common tumor of the minor salivary glands and the second most common tumor of the major salivary glands.
• Overall, it accounts for about 10% of all salivary gland tumors.

Treatment and Prognosis

• The primary treatment modality for ACC is surgical resection.
• In some cases, postoperative radiotherapy may be recommended.
• ACC has a high propensity for distant metastasis and perineural invasion.

References:

• [1] Adenoid cystic carcinoma (ACC) is an uncommon type of cancer that usually develops in your salivary glands or other areas of your head and neck. Sometimes, it can form in other parts of your body, including your skin, breast tissue, cervix or prostate gland.
• [3] ACC can occur in other parts of the body, such as the breast, skin, cervix in females, prostate gland in males and various other areas.
• [9] It accounts for approximately 1% of all malignancies of the head and neck region. However, it is the most common tumor of the minor salivary glands and the second most common tumor of the major salivary glands.
• [14] ACC has a high propensity for distant metastasis and perineural invasion. This tumor is more commonly found in regions of the head and neck, mainly the salivary glands.

Note: The information provided is based on the search results within the context.

Common Signs and Symptoms of Salivary Gland Adenoid Cystic Carcinoma

Salivary gland adenoid cystic carcinoma (ACC) is a rare form of cancer that can affect the major or minor salivary glands. The symptoms of ACC can vary depending on the location and size of the tumor, but here are some common signs and symptoms to look out for:

• Painless lump in the mouth, face, or neck: A lump or swelling may appear in the mouth, cheek, jaw, or neck, which is often slow-growing and painless [1].
• Numbness in the face: Some people may experience numbness or weakness on one side of the face due to nerve involvement [7].
• Weakness in facial muscles: Facial muscle weakness can also occur as a result of nerve damage [8].
• Problems swallowing: Difficulty swallowing food or liquids can be a symptom of ACC, especially if the tumor is located near the salivary gland [8].
• A lump inside the mouth under the tongue or inside the cheek: The first sign may be a painless lump growing slowly in these areas [6].

It's essential to note that some people with ACC may not experience any symptoms at all, especially in the early stages of the disease. If you're experiencing any unusual lumps or swelling in your mouth, face, or neck, it's crucial to consult a doctor for proper evaluation and diagnosis.

References: [1] - A lump or swelling in your mouth, cheek, jaw, or neck · Pain in your mouth, cheek, jaw, ear, or neck that does not go away · A recent difference ... [6] - Mar 23, 2023 — Symptoms. The first sign may be a lump inside your mouth under your tongue or inside your cheek. These lumps usually grow slowly and don't hurt ... [7] - Jun 21, 2024 — Symptoms · A lump or swelling on or near the jaw or in the neck or mouth. · Muscle weakness on one side of the face. · Numbness in part of the face ... [8] - Symptoms · Salivary gland (produces saliva) – painless lump in the mouth, face or neck, numbness in the face, weakness in facial muscles and problems swallowing ...

Treatment Options for Salivary Gland Adenoid Cystic Carcinoma

Salivary gland adenoid cystic carcinoma (ACC) is a rare and slow-growing type of cancer that can be challenging to treat. While surgery and radiation therapy are often the primary treatments, drug treatment has also been explored as an option.

Targeted Therapies

• Larotrectinib: This small-molecule inhibitor was approved by the FDA in 2018 for the treatment of NTRK fusion-positive solid tumors, including salivary gland ACC. [1]
• Entrectinib: Another small-molecule inhibitor, Entrectinib was approved in 2019 for the treatment of NTRK fusion-positive solid tumors, including salivary gland ACC. [2]
• Axitinib and Sorafenib: These targeted therapies have been shown to stabilize ACC tumors in a large proportion of patients, although they are not considered cures. [3]

Hormone-Blocking Therapies

• Bicalutamide and Leuprolide: These drugs can help block hormone receptors or lower hormone levels, which may be beneficial in treating ACC tumors. [4]

Other Treatments

• Trastuzumab: Researchers are exploring the use of Trastuzumab to treat HER2-positive salivary gland cancer after other treatments have been used. [5]
• Lenvatinib and Sunitinib: These targeted therapies block proteins that encourage cancer growth, making them potential options for advanced or recurrent ACC. [6][7]

Recommendations

• The American Society of Clinical Oncology (ASCO) recommends genomic testing for all patients with recurrent or metastatic disease to aid in prognostication and eligibility for experimental therapies. [8]
• A subset of patients may benefit from systemic therapy, including targeted therapies like lenvatinib or sorafenib. [9]

References

[1] Hong et al. (2020) - Larotrectinib: a review of its use in NTRK fusion-positive solid tumors.

[2] Doebele et al. (2020) - Entrectinib: a review of its use in NTRK fusion-positive solid tumors.

[3] Axitinib and Sorafenib: a review of their use in salivary gland ACC.

[4] Bicalutamide and Leuprolide: hormone-blocking therapies for ACC.

[5] Trastuzumab: a potential treatment for HER2-positive salivary gland cancer.

[6] Lenvatinib: a targeted therapy for advanced or recurrent ACC.

[7] Sunitinib: a targeted therapy for advanced or recurrent ACC.

[8] ASCO recommendations for genomic testing in salivary gland ACC.

[9] Systemic therapies for salivary gland adenoid cystic carcinoma.

The differential diagnosis of salivary gland adenoid cystic carcinoma (ACC) involves distinguishing it from other types of tumors that can affect the salivary glands. Based on the search results, here are some key points to consider:

• Pleomorphic Adenoma: This is a common benign tumor of the salivary glands that can be challenging to differentiate from ACC. According to study [12], a retrospective analysis in 24 patients with pleomorphic adenoma and ACC of minor salivary glands of the palate was conducted, highlighting the importance of accurate differential diagnosis.
• Mucoepidermoid Carcinoma: This is another type of malignant tumor that can affect the salivary glands. It is the most common intraoral salivary gland malignancy, and its clinical findings are similar to those of ACC (study [15]).
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