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INTRODUCTION

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Epidemiology

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Epidemiology
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

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Pathology

  1. Unlike renal cell carcinoma, adrenocortical cancer stains positive for vimentin

  2. >20 mitoses per HPF—median survival 14 months

    ≤20 mitoses per HPF—median survival 58 months

  3. Tumor necrosis—poor prognosis

  4. Vascular invasion—poor prognosis

  5. Capsular invasion—poor prognosis

Weiss LM et al. Am J Surg Pathol 1989;13:202–206

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Survival After Complete Resection

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Survival After Complete Resection
5-Year Actuarial Survival
Stages I–II 54%
Stage III 24%
1-Year survival
Stage IV 9%

Icard P et al. Surgery 1992;112:972–980; discussion 979–980

Icard P et al. World J Surg 1992;16:753–758

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Work-up

  1. CT scan of chest, abdomen, and pelvis to determine extent of disease

  2. MRI of abdomen may help to identify and follow liver metastases

  3. 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

  4. Serum and 24-hour urinary cortisol; 24-hour urinary 17-ketosteroid

  5. 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

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Staging

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Staging
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

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Expert Opinion

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  1. Primary therapy: Primary therapy is complete surgical resection

  2. 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

  3. 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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