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Adrenocortical carcinoma

Definition
ACC is a rare and aggressive malignancy that originates in the cortex of the adrenal gland.
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Pathophysiology
The pathophysiology of ACC is not fully understood, but it is associated with the overproduction of adrenal hormones and potential genetic mutations. Overexpression of insulin-like growth factor 2 and constitutive activation of β-catenin have been identified as key factors involved in the development of ACC. Additionally, the p53-RB and the WNT-β-catenin pathways are common disease drivers.
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Epidemiology
The incidence of ACC is estimated at 0.072 per 100,000 person-years in the US and 0.03 per 100,000 person-years in Europe.
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Disease course
Clinical manifestations of ACC can vary, but most patients present with symptoms related to steroid hormone excess, such as Cushing syndrome or virilization, or abdominal mass effects. Other symptoms can include abdominal pain, weight loss, and hormonal imbalances.
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Prognosis and risk of recurrence
The prognosis is generally poor due to late-stage diagnosis, high recurrence rates, and limited treatment options. Despite advances in the field, it is associated with heterogeneous but mostly poor outcomes.
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Key sources
The following summarized guidelines for the evaluation and management of adrenocortical carcinoma are prepared by our editorial team based on guidelines from the Canadian Urological Association (CUA 2023), the American Association of Endocrine Surgeons (AAES 2022), the European Reference Network on Rare Adult Solid Cancers (EURACAN/ESMO 2020), the European Society of Endocrinology (ESE 2018), the European Society of Endocrine Surgeons (ESES/ENSAT 2017), and the Society of American Gastrointestinal and Endoscopic Surgeons (SAGES 2013).
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Guidelines

1.Classification and risk stratification

Risk of malignancy: as per AAES 2022 guidelines, consider using washout characteristics on an adrenal protocol CT to stratify the risk of malignancy for adrenal nodules when the non-contrast HU is > 10 and other clinical risk factors for malignancy are not present. Recognize that adrenal protocol CT does not improve diagnostic accuracy for nodules with non-contrast HU < 10 nor does it improve evaluation for pheochromocytoma.
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  • Staging

  • Prognosis

2.Diagnostic investigations

Clinical assessment: as per CUA 2023 guidelines, elicit a focused history and perform a physical examination to identify signs/symptoms of adrenal hormone excess, adrenal malignancy, and/or extra-adrenal malignancy in patients with an adrenal incidentaloma.
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  • Diagnostic imaging

  • Laboratory tests

3.Diagnostic procedures

Biopsy and histopathology: as per AAES 2022 guidelines, consider performing an image-guided biopsy of a radiographically indeterminate mass, only after confirming a lack of hormone excess, if the results will change overall disease management.
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4.Medical management

Setting of care
As per AAES 2022 guidelines:
Treat patients with clinical and radiographic findings consistent with ACC at high-volume multidisciplinary centers to improve recurrence outcomes.
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Perform adrenalectomy preferentially by a high-volume adrenal surgeon to optimize outcomes, including lower rates of morbidity and mortality.
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  • Management of locoregional disease (neoadjuvant chemotherapy)

  • Management of locoregional disease (surgery)

  • Management of locoregional disease (other therapeutic interventions)

  • Management of locoregional disease (adjuvant chemotherapy)

  • Management of locoregional disease (adjuvant radiotherapy)

  • Management of advanced/metastatic disease (systemic therapy)

  • Management of advanced/metastatic disease (surgery)

  • Management of advanced/metastatic disease (other therapeutic interventions)

  • Management of progressive/relapsed disease

5.Perioperative care

Preoperative evaluation: elicit clinical history and perform a physical examination to assess the following before adrenalectomy for suspected ACC:
symptoms related to hormone excess
symptoms related to local compression by a large mass
detailed family history for familial forms of cancer
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  • Perioperative corticosteroid replacement

6.Follow-up and surveillance

Follow-up: as per ESMO 2020 guidelines, obtain radiological imaging every 3 months for 2 years after complete resection, then every 3-6 months for at least another 3 years.
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