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Adrenocortical cancer 

Adrenocortical cancer
Chapter:
Adrenocortical cancer
Author(s):

Steve Ball

, and Sajid Kalathil

DOI:
10.1093/med/9780199659579.003.0094
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date: 20 November 2019

Adrenocortical cancer (ACC) is rare and associated with poor prognosis. The incidence is estimated at 0.7–2 cases per one million. Overall survival rate at five years for ACC is 37–47%. While the pathogenesis of ACC is incompletely understood, inherited predisposition syndromes are common in childhood ACC. Clinical presentation can be with symptoms and signs of hormone excess (functional tumours), mass effects, or as an incidental radiological finding. A multidisciplinary approach combining radiology, biochemistry, and tissue-based pathology is needed to establish a diagnosis to guide a surgical approach aimed at complete resection of the tumour where possible. At present, recommended first-line therapies for advanced disease are mitotane monotherapy or etopiside, doxorubicin, and cisplatin plus mitotane. Metronomic capecitabine and gemcitabine have been used as alternatives. Adjuvant radiotherapy to the tumour bed should be considered for patients considered to be at high risk of recurrence.

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