Pheochromocytoma: The Basics

Autor: Allyson Distel, MPH
Fecha de la última revisión: December 21, 2023

Pheochromocytoma is a tumor in the adrenal gland. Pheochromocytoma is a type of cancer that targets chromaffin cells which make hormones called catecholamines. The adrenal glands sit on top of the kidneys and often pheochromocytoma impacts one of the glands. Tumors that are found just outside the adrenal glands are called paragangliomas.

Risks

The only known risk of pheochromocytoma is genetic. Here is a list of genetic syndromes linked to pheochromocytoma:

  • Von Hippel-Lindau Syndrome (vHL).
  • Multiple Endocrine Neoplasia Syndrome type 2A and 2B (MEN2A and 2B).
  • Neurofibromatosis type 1 (NF1).
  • Succinate dehydrogenase syndromes.

Talk to your provider about genetic testing and counseling if you think you should be tested for these syndromes.

Signs of Pheochromocytoma

The signs of pheochromocytoma are from the extra hormones (catecholamines) being made. These are:

  • Hypertension (high blood pressure) is most common.
  • Headaches.
  • Increased sweating.
  • Heart palpitations.

If you are having any of these side effects, you should talk to your provider.

Diagnosis of Pheochromocytoma

If your healthcare providers think you may have pheochromocytoma, they will order tests. The tests ordered are based on the signs you are having. Here are some of the tests:

  • MRI.
  • PET.
  • CT.
  • Blood tests.
  • Urine tests.
  • Meta-iodo-benzylguanidine (MIBG) scan.

Staging

Staging helps guide treatment. The stage is based on:

  • The location of the tumor.
  • If the cancer has spread to nearby tissues and/or lymph nodes.
  • Whether it has spread (metastasized) to distant body parts.

The stages range from stage I (smallest, most confined tumors) to stage IV (metastasized to other parts of the body).

Treatment

Pheochromocytoma can be treated in these ways if it is causing symptoms:

  • Surgery: the goal is to remove the whole tumor.
  • Radiation Therapy: uses high-energy x-rays to kill cancer cells.
    • MIBG and PRRT treatments are types of radiation therapy that may be used.
  • Targeted Therapy: works to slow cancer growth. Sunitinib is a targeted therapy that treats pheochromocytoma.
  • Chemotherapy: This can be used before surgery to shrink a tumor or after surgery is done if the whole tumor is not removed.

This article is a basic guide to pheochromocytoma. You can learn more about pheochromocytoma staging and treatment by using the link below:

Pheochromocytoma: Staging and Treatment

Resources for More Information: Pheochromocytoma

Referencias

American Society of Clinical Oncology. 2022. Pheochromocytoma and Paraganglioma: Diagnosis. https://www.cancer.net/cancer-types/pheochromocytoma-and-paraganglioma/diagnosis

American Society of Clinical Oncology. 2022. Pheochromocytoma and Paraganglioma: Introduction. https://www.cancer.net/cancer-types/pheochromocytoma-and-paraganglioma/introduction

American Society of Clinical Oncology. 2022. Pheochromocytoma and Paraganglioma: Stages. https://www.cancer.net/cancer-types/pheochromocytoma-and-paraganglioma/stages

American Society of Clinical Oncology. 2022. Pheochromocytoma and Paraganglioma: Types of Treatment.https://www.cancer.net/cancer-types/pheochromocytoma-and-paraganglioma/types-treatment

Amin, M. B., & Edge, S. B. AJCC cancer staging manual. Eighth edition. 2017.

Canu L et al. Sunitinib in the therapy of malignant paragangliomas: report on the efficacy in a SDHB mutation carrier and review of the literature. 2016. Found at: https://doi.org/10.1590/2359-3997000000217

Fishbein, L. et al. 2021. The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma. https://nanets.net/images/2021/2021_NANETS_Consensus_Guidelines_for_Surveillance_and_Management_of_Metastatic_and_or_Unresectable_Pheochromocytoma_and_Paraganglioma.pdf

Medline Plus. 2023. Catecholamine Tests. https://medlineplus.gov/lab-tests/catecholamine-tests/

National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology. Neuroendocrine and Adrenal Tumors. Version 1.2023.https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf

National Institute of Health. National Cancer Institute. Pheochromocytoma and Paraganglioma PDQ. https://www.cancer.gov/types/pheochromocytoma/hp

Society of Nuclear Medicine & Molecular Imaging. Fact Sheet: What is Peptide Receptor Radionuclide Therapy (PRRT)? https://www.snmmi.org/AboutSNMMI/Content.aspx?ItemNumber=29883

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