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Adrenal Tumours: Adrenocortical Functioning Adenomas, Pheochromocytomas, Incidentalomas, and Adrenocortical Cancer

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Advanced Practice in Endocrinology Nursing

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Abstract

This chapter will discuss in detail the background, evaluation, and management of adrenal tumours, with an additional focus on the role of endocrine nursing in the care of these patients. It is divided into three parts, each providing a comprehensive outline of: A) Adrenocortical functioning adenomas and adrenal hyperplasia, B) Pheochromocytomas and Paragangliomas, and C) Adrenal incidentaloma and adrenocortical cancer (ACC).

Evaluation of adrenal tumours and adenomas requires a thorough history and physical examination. Family history is particularly important as adrenocortical disease can be caused by germline mutations passed down from generation to generation. More commonly, however, sporadic somatic mutations are the cause of spontaneous tumour formation and autonomous hormone secretion.

Adrenocortical adenomas, hyperplasia, and incidentalomas are non-cancerous “growths” or proliferation of cells in the adrenal cortex. Adenomas are rare in childhood but become more frequent as humans age. Approximately 20% of adenomas are functional; that is, they produce hormones to some degree in an autonomous or dysregulated manner. Functional adenomas most commonly produce cortisol or aldosterone, whereas androgen-producing tumours are quite rare and may portend a cancerous aetiology. Co-secretion of more than one hormone from adenomas/hyperplasia is also possible.

The biochemical work-up, with screening as well as confirmatory testing, and relevant imaging will be discussed in detail. The treatment, management, and long-term monitoring are also discussed for each of the adrenal tumours, respectively. Discussion in this chapter is illustrated with a rich content of figures, box inserts, and case studies.

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Abbreviations

ACC:

Adrenocortical cancer

ACE:

Angiotensin-converting enzyme

ACTH:

Adrenocorticotropic hormone

APA:

Aldosterone-producing adenoma

ARR:

Aldosterone-to-renin ratio

AVS:

Adrenal venous sampling

BMI:

Body Mass Index

CAH:

Congenital adrenal hyperplasia

CPA:

Cortisol-producing adenoma

CRH:

Corticotropin releasing hormone

DHEA:

Dehydroepiandrosterone

GRA:

Glucocorticoid-remediable aldosteronism

HNPGL:

Head and neck paraganglioma

IV:

Intravenous

l-DOPA:

l-dihydroxyphenylalanine

MRA:

Mineralocorticoid receptor antagonist

PA:

Primary aldosteronism

PAC:

Plasma aldosterone concentration

PCC:

Pheochromocytomas

PCOS:

Polycystic ovarian syndrome

PGL:

Paraganglioma

PMNT:

Phenylethanolamine N-methyltransferase

RAAS:

Renin-angiotensin-aldosterone system

SDHx:

Succinate dehydrogenase complex

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Key Reading

  1. Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. Eur J Endocrinol. 2016;175(2):G1–g34.

    Article  CAS  PubMed  Google Scholar 

  2. Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, et al. The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016;101(5):1889–916.

    Article  CAS  PubMed  Google Scholar 

  3. NIH. NIH state-of-the-science statement on management of the clinically inapparent adrenal mass (“incidentaloma”). NIH Consens State Sci Statements. 2002;19(2):1–25.

    Google Scholar 

  4. Sharma ST, Committee AAS. An individualized approach to the evaluation of cushing syndrome. Endocr Pract. 2017;23(6):726–37.

    Article  PubMed  Google Scholar 

  5. Zeiger MA, Thompson GB, Duh QY, Hamrahian AH, Angelos P, Elaraj D, et al. The American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons medical guidelines for the management of adrenal incidentalomas. Endocr Pract. 2009;15(Suppl 1):1–20.

    Article  PubMed  Google Scholar 

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Acknowledgments

With special thanks to Diane Silverman, Interim Executive Director, and Emily Collins, President, of the Pheo Para Alliance (Pheochromocytoma and Paraganglioma) www.pheopara.org for their contribution to this chapter with a case study and information on the Patient Advocacy Group. Also special thanks to Dr. Mayank Patel, MD, Special Volunteer, Section on Medical Neuroendocrinology, NICHD/NIH for providing the MRI image on pheochromocytoma.

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Authors and Affiliations

  1. National Institutes of Health, Bethesda, MD, USA

    Andrew P. Demidowich

  2. Department of Endocrinology, Birmingham University Hospital, Birmingham, UK

    Miriam Asia

  3. Service d’Endocrinologie, Reference Center for Rare Adrenal Diseases, Hôpital Cochin, Paris, France

    Jérôme Bertherat

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  1. Andrew P. Demidowich
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Correspondence to Andrew P. Demidowich .

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Editors and Affiliations

  1. School of Health Sciences, City, University of London, London, UK

    Sofia Llahana

  2. Department of Oncology, Skåne University Hospital, Lund, Sweden

    Cecilia Follin

  3. Northwest Pituitary Center Oregon Health and Sciences University, Portland, OR, USA

    Christine Yedinak

  4. Churchill Hospital, University of Oxford, Oxford, UK

    Ashley Grossman

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Demidowich, A.P., Asia, M., Bertherat, J. (2019). Adrenal Tumours: Adrenocortical Functioning Adenomas, Pheochromocytomas, Incidentalomas, and Adrenocortical Cancer. In: Llahana, S., Follin, C., Yedinak, C., Grossman, A. (eds) Advanced Practice in Endocrinology Nursing. Springer, Cham. https://doi.org/10.1007/978-3-319-99817-6_36

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