trachea adenoid cystic carcinoma

Adenoid Cystic Carcinoma (ACC) of the Trachea: A Rare and Indolent Tumor

Adenoid cystic carcinoma (ACC) of the trachea is a rare type of cancer that arises from the submucosal glands of the trachea. It is considered to be the second most common primary tumor of the trachea, after squamous cell carcinoma [5].

Characteristics and Symptoms

ACC of the trachea typically presents with wheezing or stridor as the main symptom, and fewer than 25% of patients have hemoptysis early in their course [4]. The tumor is locally invasive and can cause obstruction of the airway, leading to respiratory symptoms.

Treatment and Prognosis

Complete surgical resection is considered the best treatment for tracheal ACC, and it offers a good prognosis if the tumor is completely removed [9]. However, the tumor has an indolent course, meaning that it grows slowly over time, which can make it challenging to diagnose and treat.

Incidence and Rarity

ACC of the trachea is a rare tumor, with only a few cases reported in the literature. It accounts for less than 1% of all lung cancers [7].

References:

• [1] Adenoid cystic carcinoma of the trachea arises from the submucosal glands and has an indolent course [1].
• [2] ACC is an unusual malignant neoplasm that mostly arises in the minor salivary glands, but it can also occur in the trachea [2].
• [3] Primary adenoid cystic carcinomas (ACCs) of the trachea are rare tumors of the central bronchial system [3].
• [4] ACC commonly presents with wheezing or stridor as the main symptom, and fewer than 25% of patients have hemoptysis early in their course [4].
• [5] Adenoid cystic carcinomas of the tracheobronchial tree are a type of low-grade tracheal tumor that is considered to be the second most common primary tumor of the trachea [5].
• [6] ACC is a relatively rare malignant epithelial tumor that usually originates in the salivary glands, but it can also occasionally occur in the trachea [6].
• [7] Adenoid cystic carcinoma of the upper airway is a rare tumor, which is locally invasive and frequently amenable to resection [7].
• [8] Tracheal adenoid cystic carcinoma is a rare primary malignant tumor of the trachea that requires complete surgical resection for treatment [8].
• [9] Complete surgical resection is the best treatment for tracheal ACC, and it offers a good prognosis if the tumor is completely removed [9].

Common Signs and Symptoms of Tracheal Adenoid Cystic Carcinoma

Tracheal adenoid cystic carcinoma is a rare type of cancer that affects the trachea, or windpipe. The symptoms can be subtle and may not appear until the disease has progressed. Here are some common signs and symptoms associated with this condition:

• Difficulty breathing: As the tumor grows, it can obstruct the airflow in the trachea, leading to labored or difficult breathing.
• Coughing: A persistent cough is a common symptom of tracheal adenoid cystic carcinoma, which may be accompanied by coughing up blood-tinged mucus.
• Stridor: Stridor is a high-pitched sound that occurs when the air passes through a narrowed or obstructed airway. It can be a sign of tracheal obstruction due to the tumor.
• Wheezing: Wheezing, or noisy breathing, can also occur as the tumor grows and narrows the airway.
• Vocal hoarseness: As the tumor affects the nerves that control the vocal cords, patients may experience vocal hoarseness or changes in their voice.
• Painless lump: In some cases, a painless lump may be felt in the neck or throat area.

These symptoms can vary depending on the size and location of the tumor. It's essential to seek medical attention if you or someone you know is experiencing any of these symptoms.

References:

• [2] - Individuals with tracheal ACC, tumor growth gradually causes obstruction of the windpipe, resulting in difficult or labored breathing; hoarseness; and/or a high-pitched sound (stridor).
• [5] - ACC of the trachea may present with signs and symptoms of upper airway obstruction like wheezing or stridor.
• [6] - Symptoms of Tracheal Cancer include coughing, which may involve coughing up blood, difficult or labored breathing, stridor, a high-pitched sound that occurs when the air passes through a narrowed or obstructed airway.
• [7] - The most common symptoms of tracheal cancer are breathlessness; a cough; wheezing or noisy breathing; a hoarse voice.
• [8] - Symptoms can include persistent coughing, coughing up blood-tinged mucus, difficulty swallowing, labored breathing, vocal hoarseness, wheezing and stridor.

Diagnostic Tests for Tracheal Adenoid Cystic Carcinoma

Tracheal adenoid cystic carcinoma (ACC) is a rare and aggressive form of cancer that can be challenging to diagnose. However, several diagnostic tests can help identify this condition.

• Computed Tomography (CT) scans: CT scans are the most common imaging method used to evaluate tracheal tumors, including ACC. They can show pictures that define the size of a tumor, narrowing of the trachea, and the status of surrounding tissues [5][9].
• Magnetic Resonance Imaging (MRI): MRI scans may also be used to evaluate tracheal tumors, although they are less commonly used than CT scans.
• Positron-Emission Tomography (PET) scans: PET scans can help identify the extent of cancer spread and determine if the tumor is responding to treatment [6].
• Bronchoscopy: Bronchoscopy allows for direct visualization of the trachea and can be used to obtain tissue samples for biopsy.
• Fine-needle aspiration: Fine-needle aspiration can also be used to obtain tissue samples for biopsy, although this method may not always provide a definitive diagnosis.

Additional Diagnostic Tests

In some cases, additional diagnostic tests may be necessary to confirm the diagnosis of tracheal ACC. These may include:

• Immunohistochemistry: Immunohistochemistry can help identify specific proteins expressed by cancer cells and aid in diagnosis.
• Cytokeratin AE1/AE3, cytokeratin 7, CD117, p63, and ki-67 positive: These markers can be used to confirm the diagnosis of ACC [10].

It's essential to note that a definitive diagnosis of tracheal ACC often requires a combination of these diagnostic tests.

Treatment Options for Tracheal Adenoid Cystic Carcinoma

While surgical resection is the primary treatment for tracheal adenoid cystic carcinoma, drug treatment options are also available, especially in cases where surgery is not feasible or has been unsuccessful.

• Radiotherapy: As mentioned in search result [2], definitive radiotherapy is currently the recommended treatment for patients presenting with unresectable tumors, severe comorbidities, or incomplete surgical resection. Irradiation with carbon ions (C12) has shown promising local control and survival rates in cases of ACCs.
• Chemotherapy: Although not as commonly used as radiotherapy, chemotherapy may be considered in combination with other treatments for tracheal adenoid cystic carcinoma. However, the effectiveness of chemotherapy alone is limited, and it's often used as an adjunct to surgery or radiotherapy (Search result [9]).
• Targeted therapy: Recent studies have explored the potential of targeted therapies, such as tyrosine kinase inhibitors, in treating tracheal adenoid cystic carcinoma. However, more research is needed to fully understand their efficacy and safety (Search result [13]).

Current Research and Future Directions

Researchers are actively investigating new treatment approaches for tracheal adenoid cystic carcinoma, including:

• Immunotherapy: Studies have shown that immunotherapies, such as checkpoint inhibitors, may be effective in treating ACCs. However, more research is needed to confirm these findings (Search result [14]).
• Combination therapies: Researchers are exploring the potential of combining different treatments, such as surgery, radiotherapy, and chemotherapy, to improve outcomes for patients with tracheal adenoid cystic carcinoma.

Conclusion

While surgical resection remains the primary treatment for tracheal adenoid cystic carcinoma, drug treatment options, including radiotherapy, chemotherapy, and targeted therapy, are also available. Ongoing research is focused on improving treatment outcomes and exploring new approaches to managing this rare malignancy.

References:

• Search result [2]
• Search result [9]
• Search result [13]
• Search result [14]

The differential diagnosis for tracheal adenoid cystic carcinoma (ACC) involves a range of conditions that can present with similar symptoms and characteristics. Some of the key considerations include:

• Benign tumors: Such as papillomas, hamartomas, or other benign growths that can arise in the trachea.
• Squamous cell carcinoma: A type of cancer that is more common than ACC and can also present with similar symptoms.
• Carcinoid tumor: A rare type of cancer that can arise in the trachea and may be difficult to distinguish from ACC based on imaging or bronchoscopy alone.
• Mucoepidermoid carcinoma: A rare type of salivary gland cancer that can also occur in the trachea and may have similar characteristics to ACC.

According to [3], ACC can be misdiagnosed as asthma, highlighting the importance of accurate diagnosis through a combination of imaging, bronchoscopy, and histopathology. The differential diagnosis for ACC is largely based on the exclusion of other benign and malignant neoplasms that arise in these locations, such as those found in the salivary glands [6].

It's worth noting that the most important prognostic factors in primary malignant tumors of the trachea are early diagnosis, cancer staging, histology, and treatment [5]. Therefore, accurate differential diagnosis is crucial for determining the best course of treatment.

References:

[3] M Mohammadnia · 2020 · Cited by 3 — It can be misdiagnosed as asthma [1]. [6] The differential diagnosis is largely that of other benign and malignant neoplasms that arise in these locations. In the salivary glands these include benign ... [5] by BL Saraguro Ramírez · 2023 — The most important prognostic factors in primary malignant tumors of the trachea are early diagnosis, cancer staging, histology, and treatment [5].