|Incidence: ||0.5 to 2.0 cases per one million population ||Stage at Presentation |
|Deaths: ||0.2% of cancer deaths ||Stage I: ||3% |
|Median age: ||Bimodal median, at age 4 years and ages 40–50 years ||Stage II: ||29% |
|Male to female ratio: ||1:1.3 ||Stage III: ||19% |
| || ||Stage IV: ||49% |
Cohn K et al. Surgery 1986;100:1170–1177
Wooten MD, King DK. Cancer 1993;72:3145–3155
Unlike renal cell carcinoma, adrenocortical cancer stains positive for vimentin
>20 mitoses per HPF—median survival 14 months
≤20 mitoses per HPF—median survival 58 months
Tumor necrosis—poor prognosis
Vascular invasion—poor prognosis
Capsular invasion—poor prognosis
Weiss LM et al. Am J Surg Pathol 1989;13:202–206
Survival After Complete Resection
Icard P et al. Surgery 1992;112:972–980; discussion 979–980
Icard P et al. World J Surg 1992;16:753–758
CT scan of chest, abdomen, and pelvis to determine extent of disease
MRI of abdomen may help to identify and follow liver metastases
If IVC is compressed, consider IVC contrast study, ultrasound, or MRI to assess disease involvement before surgical exploration, although apparent extent of involvement should not deter exploration
Serum and 24-hour urinary cortisol; 24-hour urinary 17-ketosteroid
Additional studies can be performed to determine the functional status of the tumor including: serum estradiol, estrone, testosterone, dehydroepiandrosterone sulfate (S-DHAS), 17-OH-progesterone, and androstenedione
|Stage I ||<5-cm tumor confined to adrenal |
|Stage II ||>5-cm tumor confined to adrenal |
|Stage III ||Positive lymph nodes or local invasion with tumor outside adrenal in fat or adjacent organs |
|Stage IV ||Distant metastasis |
Macfarlane DA. Ann R Coll Surg Engl 1958;23:155–186
Sullivan M et al. J Urol 1978;120:660–665
Primary therapy: Primary therapy is complete surgical resection
Surgery and Radio Frequency Ablation (RFA) as therapies for recurrences: When possible, local recurrences should be addressed surgically. Some advocate surgical resection of metastatic disease, and although it may improve survival, firm evidence is lacking. Radio frequency ablation may be used as an alternative if the recurrence is deemed amenable to ablation and has an expendable margin. Just as incomplete resections should not be embarked on, neither should incomplete ablations be performed
Management of excess hormone production: Excess hormone production should not be ignored. Manage severe hypercortisolism aggressively. Because chemotherapy is usually ineffective, treatment of hormonal excess should not be delayed in expectation that chemotherapy will reduce the tumor burden and improve symptoms. Instead, use steroidogenesis inhibitors either singly or in combination. Mitotane is the cornerstone of any strategy and should be started as soon as a diagnosis has been made. Use mitotane at the highest tolerable dose. However, because a therapeutic mitotane level and steady state will not ...
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