Carcinoma ex-pleomorphic adenoma (CXPA) is a type of cancer that develops in the salivary glands. This type of cancer develops from an existing tumor that was previously benign (noncancerous).

Carcinoma refers to a type of cancer that develops in skin cells or tissues lining organs. Pleomorphic describes the appearance of the tumor.

This type of cancer is relatively uncommon, and doctors may find it difficult to diagnose. Treatment typically involves surgery to remove the tumor, and a person may have radiation or chemotherapy afterward.

Read on to learn more about the symptoms and causes of CXPA, as well as the process of diagnosing and treating this condition.

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An adenoma is a benign tumor that can develop in the epithelial tissue, which is the tissue that makes up the skin and lines the organs and glands. Some adenomas contain abnormal cells that can become cancerous over time if not removed.

CXPA refers to a tumor of this type that has grown in a salivary gland.

These tumors are most common in the parotid glands, which are just in front of the ears on each side of the face. The tumors can also develop in the submandibular glands below the jaw and the minor salivary glands inside the mouth.

CXPA often presents as a small lump. It may grow rapidly after becoming cancerous, even if a person has had the lump for a long time.

These tumors are often high grade, which is commonly known as “aggressive.” The cells will look abnormal and disorganized under a microscope and may grow and spread faster than lower grade tumors.

According to a 2011 review, some people with CXPA experience no symptoms. In some cases, individuals with CXPA may experience the following symptoms:

CXPA develops from a benign adenoma in the salivary glands. These adenomas are not common, but according to a 2019 case report, around 6% of these tumors become cancerous.

Researchers do not know what causes benign tumors to become CXPA. However, some believe that these tumors always contained abnormal cells or were precancerous. Another possibility is that the cells in the tumor acquire cancerous properties due to exposure to external factors such as radiation.

Possible risk factors for developing the initial pleomorphic adenoma include:

Research from 2014 notes that doctors must be vigilant and investigate potential CXPA thoroughly because the condition can present very similarly to a benign adenoma, and there may be no difference in symptoms between the two.

Histopathological examination (examining tissues under a microscope) is an important part of the process for diagnosing CXPA.

When diagnosing CXPA, doctors may use the following methods:

  • Physical examination: Swelling in the area around the salivary glands may indicate CXPA.
  • Fine-needle aspiration: A doctor will insert a small needle into the tumor and draw out some of the tissue. If the results show a mix of benign and malignant (cancerous) cells, this indicates CXPA.
  • Ultrasound: An ultrasound showing that cancer has spread to lymph nodes may indicate a diagnosis of CXPA.

Treatment for CXPA often involves surgery to remove the tumor. This may be a parotidectomy, which involves completely or partially removing the parotid glands, depending on whether and how much the tumor has grown into surrounding tissues.

In some cases, an individual may need reconstructive surgery to preserve movement and function in the face.

A doctor may also offer radiotherapy, chemotherapy, or a combination of the two. This is particularly important in cases of high grade tumors, or when the surgeon has not been able to fully remove the tumor.

The outlook for an individual CXPA will depend on several factors. These can include when a doctor diagnoses the condition, how much the cancer has spread, and how aggressive the cancer is.

If a doctor can correctly diagnose CXPA and treat it early, this will improve the outlook for the individual.

CXPA is an aggressive type of cancer, and reports of survival rates vary. A 2011 review lists various survival rates reported by different studies, with a 5-year survival rate between 25–65%.

Asking a doctor some of the following questions may help an individual with CXPA better understand their condition and treatment plan:

  • Has the cancer spread to other parts of my body?
  • Will I need more tests before I begin treatment, and what will they be?
  • Which types of doctors will I need to see when going through treatment?
  • Can you recommend a doctor to give me a second opinion on a diagnosis or treatment plan?
  • How serious or aggressive is my cancer?
  • What is the likely outcome for my cancer?

CXPA is a type of tumor that develops from a benign tumor in the salivary glands. These tumors are often high grade, meaning they are aggressive and spread quickly.

It is very important for a doctor to investigate any potential instances of CXPA and deliver a diagnosis as early as possible.

Intense treatment, including surgical removal of the tumor, radiotherapy, and, in some cases, chemotherapy, can improve a person’s outlook.