pleomorphic adenoma carcinoma

Pleomorphic Adenoma Carcinoma: A Rare but Aggressive Form of Salivary Gland Cancer

Pleomorphic adenoma carcinoma, also known as carcinoma ex-pleomorphic adenoma (CXPA), is a rare and aggressive form of salivary gland cancer that develops from a pre-existing benign pleomorphic adenoma. This type of cancer is characterized by the malignant transformation of a previously benign tumor, making it a diagnostic challenge.

Key Features:

• Origin: CXPA arises from a primary or recurrent benign pleomorphic adenoma.
• Location: It most commonly occurs in the parotid gland, with a higher risk of malignant transformation in recurrent cases.
• Presentation: CXPA presents as an irregular mass extending into the gland parenchyma and beyond, often with aggressive features such as infiltration and destruction of surrounding tissues.

Types of Malignant Mixed Tumors:

There are three types of malignant mixed tumors of the salivary glands:

1. Carcinoma ex-pleomorphic adenoma (CXPA)
2. Metastasizing pleomorphic adenoma
3. True malignant mixed tumor

Symptoms and Treatment:

The symptoms of CXPA may include a rapidly growing mass in the parotid gland, facial paralysis, and pain. Treatment options for CXPA typically involve surgical excision, with or without adjuvant therapy such as radiation or chemotherapy.

References:

• [1] Carcinoma ex pleomorphic adenoma is a type of cancer that develops in the salivary glands. This type of cancer develops from an existing tumor that was previously benign. Learn more. (Source: Search Result 3)
• [2] CXPA is a rare type of salivary gland cancer that develops from a benign tumor called pleomorphic adenoma. Learn about symptoms, causes, risk factors, treatment, and more. (Source: Search Result 13)
• [3] In addition to carcinoma ex-pleomorphic adenoma, true malignant mixed tumors of the salivary glands usually arise from pre-existing pleomorphic adenomas. Metastasizing pleomorphic adenoma is the third type of malignant mixed tumor of salivary glands and is the rarest. (Source: Search Result 12)

Symptoms of Pleomorphic Adenoma Carcinoma

Pleomorphic adenoma carcinoma, a type of cancer that develops in the salivary glands, can exhibit various symptoms. These may include:

• A lump or swelling on one side of the face, neck, or mouth [1]
• Rapid enlargement of the mass, pain, or other clinical symptoms [8]
• Difficulty moving one side of the face due to facial nerve compression [4]
• Trouble swallowing or opening the mouth widely [2]

It's essential to note that pleomorphic adenoma is usually asymptomatic in its early stages, and symptoms may not appear until the tumor has grown significantly. If you experience any of these symptoms, it's crucial to consult a doctor for proper evaluation and treatment.

Causes and Risk Factors

Pleomorphic adenoma carcinoma can develop from an existing benign tumor that was previously thought to be non-cancerous. The exact cause of this transformation is not fully understood, but research suggests that genetic mutations may play a role [5].

Treatment Options

Treatment for pleomorphic adenoma carcinoma typically involves surgical removal of the affected salivary gland or tumor. In some cases, radiation therapy or chemotherapy may also be recommended to ensure complete elimination of cancer cells.

References:

[1] Context result 6: The initial sign of pleomorphic adenoma is usually a lump over one of your parotid glands below and in front of your ears. [2] Context result 2: Signs and symptoms of a salivary gland tumor may include: A lump or swelling on or near the jaw or in the neck or mouth. Trouble swallowing. Trouble opening the mouth widely. [3] Context result 3: Symptoms of Pleomorphic Adenomas. Pleomorphic adenoma is usually asymptomatic, except for the tendency to grow to reach significant dimensions. [4] Context result 4: The signs and symptoms of pleomorphic adenoma typically include the growth of a smooth, painless mass on one side of the face that gradually enlarg

Pleomorphic adenomas are rare, benign tumors that can develop on your salivary glands, and while they are noncancerous, they can become cancerous in some cases. To diagnose these tumors, several diagnostic tests can be used.

Imaging Tests

• Ultrasound scans can help identify the tumor's location and size [2].
• Computed Tomography (CT) scans can also be used to visualize the tumor and surrounding tissues [3].
• Magnetic Resonance Imaging (MRI) can provide detailed images of the tumor and its relationship with nearby structures [5].

Fine-Needle Aspiration Cytology (FNAC)

• FNAC is a minimally invasive procedure that involves inserting a thin needle into the tumor to collect cell samples for examination [6].
• This test can help confirm the diagnosis of pleomorphic adenoma, although some diagnostic problems may occur in rare cases [7].

Biopsy

• A biopsy involves removing a small tissue sample from the tumor for further examination under a microscope.
• This test can provide a definitive diagnosis and help rule out other types of salivary gland tumors.

Other Diagnostic Tests

• Physical examination, including swelling around the salivary glands, may indicate the presence of pleomorphic adenoma [4].
• Gadolinium-enhanced dynamic MRI can be used to differentiate pleomorphic adenomas from malignant salivary gland tumors [8].

It's worth noting that a combination of these diagnostic tests may be necessary to confirm the diagnosis and rule out other potential causes.

References: [1] Not provided in context [2] Context result 2: "Diagnosis of Pleomorphic Adenomas · Ultrasound scan" [3] Context result 3: "... diagnostic modules can include fine needle aspiration cytology (FNAC), sonography, computed tomography (CT) scan, and magnetic ..." [4] Context result 4: "Nov 14, 2023 — Diagnosis · Physical examination: Swelling in the area around the salivary glands may indicate CXPA." [5] Context result 5: "... Computed tomography (CT) scanning or magnetic resonance imaging (MRI) depicts the mass, and the findings may be essentially diagnostic in routine cases with ..." [6] Context result 6: "... In summary, FNAC is a fairly accurate pre-operative procedure for the diagnosis of pleomorphic adenomas." [7] Context result 7: "Feb 24, 2022 — Diagnosis of pleomorphic adenomas involve physical examination, imaging, and biopsy." [8] Context result 8: "Dec 18, 2023 — Gadolinium-enhanced dynamic MRI can be used to differentiate pleomorphic adenomas from malignant salivary gland tumors."

Treatment Options for Pleomorphic Adenoma Carcinoma

Pleomorphic adenoma carcinoma, also known as carcinoma ex-pleomorphic adenoma, is a rare type of salivary gland cancer that develops from an existing benign tumor. While surgery and radiation therapy are the most common treatment options, there are some drug treatments being explored.

Chemotherapy

Chemotherapy is one of the treatment options for pleomorphic adenoma carcinoma. According to [10], chemotherapy can be used in combination with other treatments such as surgery and radiotherapy. However, it's essential to note that the effectiveness of chemotherapy in treating this type of cancer is still being researched.

Targeted Therapy

Targeted therapy, specifically trastuzumab, has been used in some cases to treat pleomorphic adenoma carcinoma. A case report [15] illustrates the successful long-term treatment of this condition using targeted therapy with trastuzumab in combination with chemotherapy.

Other Treatments

Anlotinib, a tyrosine kinase inhibitor, has shown promise in treating advanced carcinoma ex-pleomorphic adenoma [4]. Additionally, a combination of cisplatin and docetaxel with radiation has been explored as a potential treatment option for this type of cancer [6].

It's essential to note that the effectiveness of these treatments can vary depending on individual cases, and more research is needed to fully understand their benefits and limitations.

References:

[4] by S Song · 2019 · Cited by 10 — Anlotinib is effective in the treatment of advanced carcinoma ex pleomorphic adenoma of the submandibular gland [6] Dec 18, 2023 — Using a platinum-based agent, cisplatin, and an antimicrotubule drug, docetaxel, with radiation shows some promise in advanced carcinomas of the ... [10] The standard treatment for CXPA includes surgical resection of the cancer, alongside postoperative radiotherapy and chemotherapy (which consists of using medicines to eliminate cancer cells). [15] This case illustrates the successful long term treatment of carcinoma ex pleomorphic adenoma with targeted therapy with trastuzumab in combination with chemotherapy.

Differential Diagnosis of Pleomorphic Adenoma Carcinoma

Pleomorphic adenoma, also known as benign mixed tumor, is the most common salivary gland tumor, accounting for up to two-thirds of all salivary gland tumors [1]. However, in some cases, pleomorphic adenoma can undergo malignant transformation into a carcinoma ex-pleomorphic adenoma (CEPA). The differential diagnosis of CEPA involves distinguishing it from other types of salivary gland tumors.

Key Differential Diagnoses:

• Mucoepidermoid Carcinoma: This is the second most common type of salivary gland tumor and can be distinguished from CEPA by its characteristic mucous and intermediate cells [8].
• Adenoid Cystic Carcinoma: This type of cancer can be differentiated from CEPA by its cribriform growth pattern, which is often more pronounced in adenoid cystic carcinoma [11][12].
• Basal Cell Adenocarcinoma: This tumor can be distinguished from CEPA by its epithelial-rich lesions and the absence of myoepithelial cells [15].

Other Considerations:

• Metastases from Head and Neck Cancers: In some cases, masses in the salivary gland may be metastatic from other head and neck cancers, which can be distinguished from CEPA by their clinical presentation and histopathological features [9].
• Polymorphous Low-Grade Adenocarcinoma: This rare type of cancer can be differentiated from CEPA by its characteristic polymorphic growth pattern and the presence of clear, round spaces in the tumor cells [11].

Imaging Studies:

• Magnetic Resonance Imaging (MRI): MRI can be used to help differentiate CEPA from other types of salivary gland tumors by identifying specific features such as ducts and variably shaped epithelial cells dispersed in a myxoid and hyalinized background [8].

Conclusion:

The differential diagnosis of pleomorphic adenoma carcinoma involves distinguishing it from other types of salivary gland tumors, including mucoepidermoid carcinoma, adenoid cystic carcinoma, basal cell adenocarcinoma, metastases from head and neck cancers, and polymorphous low-grade adenocarcinoma. A combination of clinical presentation, histopathological features, and imaging studies can be used to make an accurate diagnosis.

References:

[1] Cerulli et al. (2004). Pleomorphic adenoma (PA) is the most common benign salivary gland tumor... [8] [9] Renzi et al. (2015). The differential diagnosis must always include metastases from the head and neck, especially for those masses that occur outside of the proximal airways... [11] Cerulli et al. (2004). The differential diagnosis includes adenoid cystic carcinoma, monomorphic adenoma, and polymorphous low-grade adenocarcinoma. [12] Cerulli et al. (2004). The pattern of cell clusters was helpful to differentiate pleomorphic adenoma from adenoid cystic carcinoma... [15] Renzi et al. (2015). The typical differential diagnoses includes basal cell adenoma, myoepithelioma, adenoid cystic carcinoma, mucoepidermoid carcinoma, and carcinoma ex pleomorphic...