Surgery is the mainstay of treatment for adrenocortical carcinoma. Appropriate preoperative evaluation and operative planning are really important to assure the best outcome. Lymph nodes should be removed as part of the en bloc resection. Recurrence in the peritoneum outside the tumor bed has the worst survival. Surgery is indicated in those patients with disease confined to one site or organ.

• Surgery is the mainstay of treatment for adrenocortical carcinoma.
• Adrenalectomy can also be performed even in the case of the invasion into large blood vessels, such as the renal vein or inferior vena cava.
• A large percentage of patients are not surgical candidates.
• Appropriate preoperative evaluation and operative planning are the most important to assure the best outcome.
• Preoperative imaging should be obtained to evaluate the extent of the tumor.
• Imaging should be done just before the surgery because ACC grow quickly and involvement of adjacent structures may change.
• Optimization of hormone excess in patients with functional masses especially those with Cushing’s syndrome should be done.
• Patients with widespread distant metastatic disease in multiple organs or those with multiple metastatic deposits in one organ system, unable to be completely resected, should not undergo adrenalectomy. The primary tumor can instead be treated with external beam radiation for palliation along with other adjuncts to improve local symptoms and for better control of hormone excess, if present.^[1]
• Obstruction of the vena cava by tumor thrombus can lead to massive lower extremity edema. If tumor resection is not technically operable, vena cava stents can be placed, leading to temporary prevention of occlusion.
• Debulking for control of hormone excess in the setting of known metastatic disease is also performed in some situations. The benefits of debulking must outweigh the risks of surgery in patients who have poor wound healing.

• Lymph nodes should be removed as part of the en bloc resection. The lymphadenectomy is performed based on the arterial supply of adrenal tumors.^[2]
• Risk of severe bleeding and benefits of massive removal of lymph nodes should be balanced by surgeons in every case.^[3]
• Published data comparing the efficacy of laparoscopic adrenalectomy (LA) vs open adrenalectomy (OA) for ACC are limited.

1. Incision and exploration of the peritoneal cavity.

2. Evaluation of liver for metastasis.

3. Containment: a self-retaining retractor system with towels or laparotomy pads should be placed in such a way as to exclude the rest of the peritoneal cavity from the area of the tumor and other organs requiring resection.

4. Mobilization of organs adjacent to tumor.

5. En bloc resection. Preserve any tissue overlying the tumor.

6. Regional lymphadenectomy.

7. Provide intact en bloc specimen for pathologic review.

8. Mark field to facilitate postsurgical external beam radiation therapy.

9. Dictate operative report.

• The number of organs involved by tumor at the time of the first metastasis is a predictor of survival.^[8]
• University of Michigan data shows the site of first metastasis can also be used to predict the survival.
• Recurrence in the peritoneum outside the tumor bed has the worst survival.
• Surgery is indicated in those patients with disease confined to one site or organ.
• Waiting 3 months while treating with chemotherapy to assess for tumor responsiveness of progression. If progression is not rapid, surgery may proceed with greater benefit.^[4]
• The median survival of 74 months in those undergoing complete second resections and a median survival of 16 months in those undergoing incomplete second resection.^[9]

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