Pheochromocytoma and Paraganglioma: Stages

Approved by the Cancer.Net Editorial Board, 09/2021

ON THIS PAGE: You will learn about how doctors describe a tumor's growth or spread. This is called the stage. Use the menu to see other pages.

What is staging?

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body.

Staging can be clinical or pathological. Doctors use diagnostic tests to find out the tumor’s stage, so clinical staging may not be complete until all of the tests are finished. If surgery is done on the primary tumor, the medical team will also do pathological staging. Knowing the stage helps the doctor recommend the best kind of treatment, and it can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different types of tumors.

This page provides detailed information about the systems used to find the stage of pheochromocytoma and paraganglioma and the stage groups for these tumors, such as stage II or stage IV.

General staging system for pheochromocytoma and paraganglioma

Pheochromocytoma or paraganglioma may be described using a general system or the TNM system (see below). In the general system, the tumor will be described as localized, regional, or metastatic:

  • Localized pheochromocytoma. The tumor is detected at an early stage in the adrenal medulla in 1 or both adrenal glands.

  • Localized paraganglioma. The tumor is detected at an early stage in 1 area of the body only.

  • Regional pheochromocytoma or paraganglioma. Cancer has spread from where the tumor began to nearby surrounding tissues and/or lymph nodes.

  • Metastatic pheochromocytoma or paraganglioma. Cancer has spread from where the tumor began to a distant part of the body.

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TNM staging system

One tool that doctors use to describe the stage is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?

  • Metastasis (M): Has the cancer spread to other parts of the body? If so, where and how much?

The results are combined to determine the stage of the tumor for each person.

There are 4 stages: stages I through IV (1 through 4). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

Here are more details on each part of the TNM system for pheochromocytoma and paraganglioma:

Tumor (T)

Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Tumor size is measured in centimeters (cm). A centimeter is roughly equal to the width of a standard pen or pencil.

Stage may also be divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be assessed.

T1: The tumor is less than 5 cm in size, and it has not grown beyond the adrenal gland.

T2: The tumor is 5 cm or more in size, and it has not grown beyond the adrenal gland. Or, it is a paraganglioma that causes symptoms.

T3: The tumor is any size and invades nearby tissues, such as the liver, pancreas, spleen, or kidneys.

Node (N)

The “N” in the TNM staging system stands for lymph nodes. These small, bean-shaped organs help fight infection. Lymph nodes near where the cancer started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

NX: The regional lymph nodes cannot be assessed.

N0 (N zero): The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to the regional lymph nodes.

Metastasis (M)

The “M” in the TNM system describes whether the cancer has spread to other parts of the body, called metastasis.

M0 (M zero): There is no metastasis.

M1a: There is metastasis only to the bone.

M1b: There is metastasis only to distant lymph nodes, the liver, or a lung.

M1c: There is metastasis to bone and other sites.

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Stage groups for pheochromocytoma and paraganglioma

Doctors combine the T, N, and M information to say what stage the tumor is.

Stage I: The tumor is smaller than 5 cm. It is located only in the adrenal gland. No other organs are involved (T1, N0, M0)

Stage II: The tumor is 5 cm or more in size, and it has not grown beyond the adrenal gland. Or, it is a paraganglioma that causes symptoms. No other organs are involved. (T2, N0, M0)

Stage III: Any of the following applies:

  • The tumor is smaller than 5 cm. It is located only in the adrenal gland. The cancer involves the regional lymph nodes. (T1, N1, M0)

  • The tumor is 5 cm or larger but does not grow beyond the adrenal gland. Or, it is a paraganglioma that causes symptoms. The cancer involves the regional lymph nodes. (T2, N1, M0)

  • The tumor is any size and invades nearby tissues, such as the liver, pancreas, spleen, or kidneys. (T3, any N, M0)

Stage IV: There is metastasis. (Any T, any N, M1)

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Recurrent pheochromocytoma and paraganglioma

Recurrent pheochromocytoma or paraganglioma is a tumor that has come back after treatment. If the tumor does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

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Used with permission of the American College of Surgeons, Chicago, Illinois. The original and primary source for this information is the AJCC Cancer Staging Manual, Eighth Edition (2017), published by Springer International Publishing.

Information about the tumor’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.