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Stages of Pheochromocytoma and Paraganglioma

Key Points

  • After pheochromocytoma and paraganglioma have been diagnosed, tests are done to find out if the tumor has spread to other parts of the body.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • There is no standard staging system for pheochromocytoma and paraganglioma.
  • Pheochromocytoma and paraganglioma are described as localized, regional, or metastatic.
    • Localized pheochromocytoma and paraganglioma
    • Regional pheochromocytoma and paraganglioma
    • Metastatic pheochromocytoma and paraganglioma

After pheochromocytoma and paraganglioma have been diagnosed, tests are done to find out if the tumor has spread to other parts of the body.

The extent or spread of cancer is usually described as stage. It is important to know whether the cancer has spread in order to plan treatment. The following tests and procedures may be used to determine if the tumor has spread to other parts of the body:

  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. The abdomen and pelvis are imaged to detect tumors that release catecholamine. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the neck, chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma and paraganglioma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Octreotide scan: A type of radionuclide scan used to find certain tumors, including tumors that release catecholamine. A very small amount of radioactive octreotide (a hormone that attaches to certain tumors) is injected into a vein and travels through the bloodstream. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.
  • FDG-PET scan (fluorodeoxyglucose-positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of FDG, a type of radioactive glucose (sugar), is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood :

  • Tissue. The cancer spreads from where it began by growing into nearby areas.
  • Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
  • Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

  • Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
  • Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if pheochromocytoma spreads to the bone, the cancer cells in the bone are actually pheochromocytoma cells. The disease is metastatic pheochromocytoma, not bone cancer.

There is no standard staging system for pheochromocytoma and paraganglioma.

Pheochromocytoma and paraganglioma are described as localized, regional, or metastatic.

Localized pheochromocytoma and paraganglioma

The tumor is found in one or both adrenal glands (pheochromocytoma) or in one area only (paraganglioma).

Regional pheochromocytoma and paraganglioma

Cancer has spread to lymph nodes or other tissues near where the tumor began.

Metastatic pheochromocytoma and paraganglioma

The cancer has spread to other parts of the body, such as the liver, lungs, bone, or distant lymph nodes.

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