Adrenocortical carcinoma (ACC) is a rare and aggressive neoplasm originating from the cortex of the adrenal gland
. It is characterized by a heterogeneous pathogenesis and often presents with an unfavorable prognosis
Pathophysiology
- The pathogenesis of ACC is incompletely understood, but overexpression of insulin-like growth factor 2 (IGF-2) and constitutive activation of β-catenin are key factors involved in its development
- ACC is composed of distinct molecular subtypes associated with different clinical outcomes
Clinical manifestations
- Most patients present with steroid hormone excess, such as Cushing syndrome or virilization, or abdominal mass effects
- A growing proportion of patients with ACC (currently >15%) is initially diagnosed incidentally
Risk factors
- Patients with a history of extra-adrenal malignancy are at increased risk for adrenal metastases
- Primary adrenal malignancy is suspected in patients with an adrenal incidentaloma > 4 cm and/or Hounsfield units (HU) > 20 on non-contrast CT and in any patient under 18 years old
- An adrenal mass is considered at increased risk of malignancy in the presence of multiple hormonal, steroid precursor, or sex hormone oversecretion; intratumoral radiological signs of malignancy; a diameter > 6 cm; evidence of local invasion; suspected metastatic lymph nodes; distant metastasis; and/or high 18F-FDG-PET uptake
Prognosis
- The prognosis of ACC is generally poor, with a high frequency of recurrence
- Prognosis should be reassessed at each evaluation during follow-up to guide treatment strategy
- Factors to consider when assessing prognosis and treatment options at initial diagnosis include tumor stage, resection status, Ki67 index (or mitotic count), autonomous cortisol secretion, and the patient's general condition
In conclusion, ACC is a rare and aggressive neoplasm with a heterogeneous pathogenesis and poor prognosis. It often presents with steroid hormone excess or abdominal mass effects, and a growing proportion of cases are diagnosed incidentally. Risk factors include a history of extra-adrenal malignancy and certain characteristics of adrenal incidentalomas. Prognosis is generally poor, with a high frequency of recurrence, and should be reassessed at each evaluation during follow-up to guide treatment strategy.