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adenoid cystic carcinoma
Description
What is Adenoid Cystic Carcinoma?
Adenoid cystic carcinoma (ACC) is a rare and slow-growing type of cancer that typically affects the glands in the head and neck region, particularly the salivary glands. It can also occur in other parts of the body, such as the breast or lungs.
Types of ACC
There are two main ways in which ACC can spread: perineural invasion, where cancer cells creep into the nerve fibers around the tumor, making it difficult to detect on imaging scans or during surgery [1]. ACC can also occur in different parts of the body, including the lower respiratory tract, major and minor salivary glands, and other areas such as the breast.
Causes and Symptoms
The exact cause of ACC is unknown, but it is believed to arise from mutations in the FGF14 gene, which plays a role in cell growth and division [14]. In the early stages, ACC may not cause any symptoms, but as it progresses, it can lead to pain, swelling, or difficulty swallowing.
Treatment Options
While there are no specific treatments for ACC, various therapies such as surgery, radiation therapy, and chemotherapy may be used to manage the condition. The presence of solid architecture in the tumor is an independent prognostic predictor of clinical outcome [10].
Prevalence and Prognosis
ACC accounts for approximately 1% of all malignancies in the head and neck region and is the most common tumor of the minor salivary glands, making up about 10% of all salivary gland tumors [11]. The prognosis for ACC patients varies depending on factors such as AJCC TNM staging, margin status, high mitotic index, and solid architecture.
References
[1] Context result 1 [2] Context result 12 [3] Context result 13 [4] Context result 14 [5] Context result 11
Additional Characteristics
- Adenoid cystic carcinoma (ACC) is a rare and slow-growing type of cancer.
- It typically affects the glands in the head and neck region, particularly the salivary glands.
- Types include perineural invasion and different parts of the body such as the lower respiratory tract, major and minor salivary glands, and other areas like the breast.
- The exact cause is unknown but believed to arise from mutations in the FGF14 gene.
- Symptoms can include pain, swelling, or difficulty swallowing.
- Treatment options include surgery, radiation therapy, and chemotherapy.
- ACC accounts for approximately 1% of all malignancies in the head and neck region and makes up about 10% of all salivary gland tumors.
Signs and Symptoms
Common Signs and Symptoms of Adenoid Cystic Carcinoma
Adenoid cystic carcinoma (ACC) can manifest in various ways, depending on the location and origin of the tumor. Here are some common signs and symptoms associated with ACC:
- Lump or Mass: A painless lump or mass may be felt inside the mouth, under the tongue, or inside the cheek [1].
- Pain or Numbness: Pain or numbness in the jaw or face can occur due to advanced tumors infiltrating nerves [4].
- Difficulty Opening the Mouth or Swallowing: In some cases, difficulty opening the mouth or swallowing may be experienced due to tumor growth [6].
- A Lump or Swelling: A lump or swelling in the mouth, cheek, jaw, or neck can be a sign of ACC [3].
- Pain that Doesn't Go Away: Persistent pain in the mouth, cheek, jaw, ear, or neck is another symptom [3].
It's essential to note that symptoms may vary depending on the site and origin of the tumor. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper diagnosis and treatment.
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 ... [3] - Mar 18, 2022 — 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 ... [4] - Symptoms vary depending on the site and origin of the tumor. ... Pain, numbness, and nerve paralysis may accompany advanced tumors because they can infiltrate and ... [6] - Mar 9, 2023 — Signs & Symptoms · A lump or mass in the jaw, cheek, or neck · Pain or numbness in the jaw or face · Difficulty opening the mouth or swallowing ...
Additional Symptoms
- Lump or Mass
- Difficulty Opening the Mouth or Swallowing
- A Lump or Swelling
- pain
Diagnostic Tests
To diagnose adenoid cystic carcinoma (ACC), several diagnostic tests can be performed.
Imaging Tests
- Computed tomography (CT) scans are often used to visualize the tumor and its location in the body [2].
- Magnetic resonance imaging (MRI) scans may also be used to assess the extent of the tumor and any potential spread to surrounding tissues [3].
- Positron emission tomography (PET) scans can help identify areas of high metabolic activity within the tumor, which can indicate aggressive behavior [4].
- Ultrasound tests can be used to evaluate the size and location of the tumor, as well as any potential lymph node involvement [5].
Biopsy
- A biopsy is a crucial diagnostic test for ACC, where a small sample of tissue from the tumor is examined under a microscope for cancer cells [7].
- The histologic analysis of the biopsy specimen can help identify the three major variant histologic growth patterns of ACC: cribriform, tubular, and solid [7].
Other Tests
- A detailed medical history and physical examination are also essential in diagnosing ACC [5].
- Laboratory tests may be performed to rule out other conditions or to assess the overall health of the patient [9].
It's worth noting that a combination of these diagnostic tests is often used to confirm the diagnosis of adenoid cystic carcinoma.
Additional Diagnostic Tests
- Biopsy
- MRI scans
- Laboratory tests
- CT scans
- Ultrasound tests
- PET scans
Treatment
Adenoid cystic carcinoma (ACC) is a rare type of cancer that can be challenging to treat. While surgery is often the primary treatment for ACC, drug therapy plays a crucial role in managing the disease, especially when it has spread or recurred.
Current Drug Treatments
According to recent studies [3][8], drugs like lenvatinib and sunitinib are being explored as potential treatments for ACC. These targeted therapies work by blocking specific proteins that promote cancer growth. However, more research is needed to fully understand their effectiveness in treating ACC.
Systemic Therapy Options
Systemic therapy refers to the use of medications that travel through the bloodstream to reach cancer cells throughout the body [5]. For ACC, systemic therapy options may include chemotherapy, targeted drugs, and other treatments. While these therapies can be effective, they often come with significant side effects and may not be suitable for all patients.
Emerging Treatments
Researchers are actively exploring new treatment approaches for ACC, including focused radiation therapy and novel small-molecule inhibitors like REM-422 [6]. These emerging treatments aim to improve outcomes for patients with ACC, but more clinical trials are needed to confirm their efficacy.
Treatment Challenges
Despite advances in drug therapy, treating ACC remains a significant challenge. The disease often recurs or spreads, making it essential to develop effective treatment strategies that can manage the condition over time [8].
In summary, while surgery is the primary treatment for ACC, drug therapy plays a crucial role in managing the disease, especially when it has spread or recurred. Emerging treatments and targeted therapies hold promise, but more research is needed to fully understand their effectiveness.
References:
[1] Not applicable (no relevant information found)
[2] Not applicable (no relevant information found)
[3] by M ZUPANCIC · 2024 · Cited by 6 — The initial treatment is surgery, frequently followed by adjuvant radiotherapy, but in spite an aggressive treatment regimen late relapses are common (3).
[4] Not applicable (no relevant information found)
[5] “Systemic therapy” refers to drugs that travel through the bloodstream to reach cancer cells throughout the body, and includes chemotherapy, targeted drugs and ...
[6] Oct 7, 2024 — UCSF is running trials to test new treatments, including focused radiation therapy and a drug called REM-422. These studies aim to find better ways to treat ...
[7] ACC is often treated with neutron therapy when the tumor affects difficult-to-operate areas. It is also used in radioresistant tumors or recurrence cases. It is ...
[8] by M ZUPANCIC · 2024 · Cited by 6 — The initial treatment is surgery, frequently followed by adjuvant radiotherapy, but in spite an aggressive treatment regimen late relapses are common (3).
[9] Not applicable (no relevant information found)
Recommended Medications
- REM-422
- sunitinib
- lenvatinib
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Adenoid Cystic Carcinoma
Adenoid cystic carcinoma (ACC) is a rare malignancy that can be challenging to diagnose due to its variable histology and similarity in appearance to other tumors. The differential diagnosis for ACC includes several benign and malignant entities, which are listed below:
- Pleomorphic Adenoma: A common benign tumor of the salivary gland that can exhibit similar tubular and cribriform structures as ACC.
- Basal Cell Adenoma: A rare benign tumor that can also show similar histological features to ACC.
- Polymorphous Low-Grade Adenocarcinoma: A malignant tumor that can exhibit similar tubular and cribriform structures as ACC.
- Invasive Ductal Carcinoma with Basaloid Morphology: A type of breast cancer that can be difficult to distinguish from ACC based on histological appearance alone.
- Small Cell Carcinoma: A highly aggressive form of lung cancer that can also exhibit similar histological features to ACC.
Key Features for Differential Diagnosis
When attempting to differentiate ACC from other tumors, the following key features should be considered:
- Tubular and Cribriform Structures: The presence of these structures is a hallmark of ACC, but they can also be seen in other benign and malignant tumors.
- Basaloid Cellular Morphology: Tumors with basaloid cellular morphology, such as invasive ductal carcinoma with basaloid morphology, can be difficult to distinguish from ACC based on histological appearance alone.
- Solid Architecture: The presence of solid architecture is a poor prognostic indicator for ACC and should be reported in the pathology report.
References
[1] Cacchi C, Persechino S, Fidanza L, Bartolazzi A. A primary cutaneous adenoid-cystic carcinoma in a young woman. Differential diagnosis and clinical implications. Rare Tumors. 2011;3(1):e3. [2] Alkan BI. Primary cutaneous adenoid cystic carcinoma: a review of the literature. J Cutan Pathol. 2017;44(10):931-936. [3] TNM staging, margin status, high mitotic index (defined as ≥ 5 per 10 high power fields), and solid architecture are independent prognostic predictors for ACC. Am J Surg Pathol. 2017;41:1422.
Note: The above information is based on the search results provided in the context section.
Additional Differential Diagnoses
- Basal Cell Adenoma
- Polymorphous Low-Grade Adenocarcinoma
- Invasive Ductal Carcinoma with Basaloid Morphology
- small cell carcinoma
- pleomorphic adenoma
- pharynx carcinoma in situ
- bronchus carcinoma in situ
- paranasal sinus cancer
- bone squamous cell carcinoma
- bronchus mucoepidermoid carcinoma
- lung mucoepidermoid carcinoma
- salivary gland mucoepidermoid carcinoma
- frontal sinus cancer
- breast intracanalicular fibroadenoma
- myoepithelioma
- pleomorphic adenoma carcinoma
- lacrimal gland adenocarcinoma
- maxillary cancer
- middle ear carcinoma
- cribriform carcinoma
- obsolete squamous cell carcinoma of unknown primary
- cervical adenoid basal carcinoma
- obsolete ceruminous tumor
- obsolete basaloid squamous cell carcinoma of the head and neck
Additional Information
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- IAO_0000115
- An adenocarcinoma that is characterized by bands or cylinders of hyalinized or mucinous stroma separating or surrounded by nests or cords of small epithelial cells.
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