Adrenocortical carcinoma

Epidemiology

Most cause signs and symptoms
Most patients present with stage III or IV disease - symptoms are often caused by the mets
May be associated with increased steroid hormone secretion with biochemical features of Cushing's syndrome

CT: irregular borders, higher attenuation values, heterogenous, central necrosis, invasion, possibly local lymphatic mets or distant disease
Tend to be very large at presentation (9-13cm mean size), and have usually spread beyond adrenal gland
Histopathological criteria: Weiss (reticulin alterations in the presence of at least one of necrosis, high mitotic rate, venous invasion)

FDG PET
CT CAP
Consider MRI to check for local invasion
Consider brain imaging (brain mets are rare, but do happen)
Exclude functional tumours especially phaeo (>50% of adrenocortical carcinoma are functional - most commonly Cushing, followed by virilisation)
Do not biopsy percutaneously if being considered for surgery

Resect stage I-III - radical
- Role for locoregional lymphadenectomy is unclear at this point, but resect all visible disease, including en bloc resection of adjacent organs
- Can be especially hard for large right-sided lesions due to direct extension into IVC or heart. Intravascular extension may require resection on ECMO to prevent embolisation.
- Open adrenalectomy is generally better for malignancy
Stage IV
- Chemotherapy - mitotane, a derivative of DDT that is a direct adrenocortical toxin
- Can be given as adjuvant therapy, which probably improves survival
- Radiotherapy can be considered, especially for R1 resections, palliative, or when there is a biopsy tract to be dealt with

40% 5-year survival among patients who undergo R0 resection
- Extremely limited survival for those who have incomplete resection (<1 year)
- Even if R0, local recurrence tends to occur within two years
Overall 5-year survival of 15-20%