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Cushing's Syndrome

Adrenocortical carcinoma

Read time: 30 mins
This learning resource will enable you to understand the symptoms of ACC, and the key recommendations for clinical assessment, detailed hormonal work-up, imaging, pathological work-up, and staging classification and prognostic factors.

Adrenocortical carcinoma (ACC) is a rare, aggressive endocrine malignancy arising from the adrenal cortex. It is a progressive disease associated with a poor prognosis. Most of patients (~2/3) symptoms are due to signs of hormone excess. Hypercortisolism is the most common case (50%–80%) which gives rise to rapidly developing Cushing’s syndrome (Else et al., 2014).

What is ACC?

Discover the key features of ACC, by watching the 2-minute animation below, which includes an introduction to the diagnosis and treatment recommendations associated with this disease.

Disease Overview

Epidemiology and pathogenesis

 

What is ACC?

The adrenal gland plays a crucial role in the endocrine system. Adrenal tumours are very common, affecting 3‒10% of the population (Else et al., 2014). They are normally small, benign, non-functioning adenocarcinomas (ACAs) (Else et al., 2014). The adrenal glands are also a common metastatic site for lung and other cancers. ACC arising from the adrenal cortex is one of two primary adrenal malignancies, the other being phaeochromocytoma arising from the medulla (Berruti et al., 2012).

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ESE Diagnosis Recommendations

This section details the key recommendations of the European Society of Endocrinology (ESE).

All patients with suspected and proven ACC should be discussed in a multidisciplinary expert team meeting (including health care providers experienced in the care of adrenal tumours, such as those who work in endocrinology, oncology, pathology, radiology and surgery), at least at the time of initial diagnosis (Fassnacht et al., 2018).

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ESE Treatment Recommendations 

Treatment recommendations are based on whether ACC is localised or advanced.

 

Localised ACC

Treatment options for localised ACC include both surgery and adjuvant therapy.

Surgery

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This section contains useful links to related associations, societies and patient support groups to help further your knowledge and understanding of ACC, network with other healthcare professionals, see the latest data and discover how to optimise patient support.

European Network for the Study of Adrenal Tumors (ENSAT)

Providing a wealth of further disease information including relevant clinical trial data, retrospective coverage of ENSAT meetings, and news of upcoming events.

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Welcome:

Adrenocortical carcinoma references

Benassai G, Desiato V, Benassai G, Bianco T, Sivero L, Campagna R, et al. Adrenocortical carcinoma: what the surgeon needs to know. Case report and literature review. Int J Surg. 2014;47(2)395‒407.

Berruti A, Baudin E, Gelderblom H, Haak HR, Porpiglia F, Fassnacht M et al. Adrenal cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2012:35(2):131‒8.

Creemers SG, Hoffland LJ, Korpershoek E, Franssen GJ, van Kemenade FJ, de Herder WW, et al. Future directions in the diagnosis and medical treatment of adrenocortical carcinoma. Endocrin Relat Cancer. 2016;23(1):R43‒69.

Duregon E, Volante M, Bollito E, Goia M, Buttigiero C, Zagg B, et al. Pitfalls in the diagnosis of adrenocortical tumours: a lesson from 300 consultation cases. Hum Pathol. 2015;46(12)1799‒807.

Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoli EM, et al. Adrenocarcinoma. Endocr Review 2014;35(2):282‒326.

Fassnacht M, Dekkers OM, Else T, Baudin E, Beruti A, de Krigger, et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the study of adrenal tumours. Eur J Endocrinol. 2018;179(4):G1‒G46.

Kassi E, Angelousi, Zografos G, Katalas G and Chrousos GP. Endotext. 2016. Available at: https:www.ncbi.hlm.nih.gov/books/NBK279009 (accessed April 2019)

Libé R. Adrenocortical carcinoma (ACC): diagnosis, prognosis and treatment. Front Cell Biol. 2015;3:45.

National Cancer Institute. Available at: https://www.cancer.gov/publications/dictionaries/cancer-terms/def/adrenal-gland (accessed April 2019).

ESE Diagnosis Recommendations

Else T, Kim AC, Sabolch A, Raymond VM, Kandathil A, Caoli EM, et al. Adrenocarcinoma. Endocr Review 2014;35(2):282‒326.

Fassnacht M, Dekkers OM, Else T, Baudin E, Beruti A, de Krigger, et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the study of adrenal tumours. Eur J Endocrinol. 2018;179(4):G1‒G46.

Kassi E, Angelousi, Zografos G, Katalas G and Chrousos GP. Endotext. 2016. Available at: https:www.ncbi.hlm.nih.gov/books/NBK279009 (accessed April 2019)

Williams AR, Hammer GD, Else T. Transcutaneous biopsy of adrenocortical carcinoma is rarely helpful in diagnosis, potentially harmful, but does not affect patient outcome. Eur J Endocrinol. 2014;170(6)829‒35.

ESE treatment recommendation

Fassnacht M, Dekkers OM, Else T, Baudin E, Beruti A, de Krigger, et al. European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the study of adrenal tumours. Eur J Endocrinol. 2018;179(4):G1‒G46.

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