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PubMed This is a summary of 19 peer-reviewed journal articles Updated
Surgical Oncology · Adrenocortical Carcinoma

Surgery and First Steps of Treatment

At a Glance

For adrenocortical carcinoma, removing the tumor completely in one piece through open surgery at an experienced center offers the best chance of cure. Afterward, cancer stage, lymph node findings, margins, and Ki-67 help guide whether mitotane or active surveillance is appropriate.

Surgery is the most critical step in treating Adrenocortical Carcinoma (ACC) and is currently the only treatment that offers a chance for a cure [1][2]. Because this cancer is rare and technically difficult to remove, it is essential that your surgery is performed by an expert surgeon at a high-volume center—a hospital that specializes in adrenal cancers and treats many cases each year [3].

The Standard of Care: Open En Bloc Resection

For most patients with suspected or confirmed ACC, the standard surgical approach is open surgery [4]. This involves a larger incision that allows the surgeon to see the tumor and surrounding organs clearly. The goal is an en bloc resection, which means removing the tumor entirely in one piece without breaking its outer “capsule” [3][5].

If the tumor has grown into nearby organs (like the kidney, spleen, diaphragm, or liver), the surgeon may need to remove those involved parts together with the tumor as one unit [6]. This can substantially change your recovery and risks. Furthermore, if your tumor is functioning or your opposite adrenal is suppressed, a clear perioperative hormone and steroid management plan must be coordinated with your endocrinologist.

Breaking the tumor or “spilling” cancer cells during surgery significantly increases the risk that the cancer will return in the abdomen (peritoneal recurrence) [7].

The Surgical Approach: Open vs. Minimally Invasive

While laparoscopic (minimally invasive) surgery is common for many conditions, it is generally discouraged for ACC unless the tumor is highly selected (very small, non-invasive) and performed by an extremely experienced oncologic surgeon [8][9].

The primary concern with minimally invasive surgery is the higher risk of positive margins (leaving microscopic cancer cells behind) or tumor rupture [7]. Studies have shown that while laparoscopic surgery might lead to a shorter hospital stay, it can sometimes result in earlier recurrence or peritoneal spread in less-selected cases [10][11].

Regional Lymph Node Assessment

During your surgery, clinical guidelines recommend that your surgeon strongly consider a regional lymph node dissection (lymphadenectomy) [12]. This involves removing nearby lymph nodes to check for cancer spread.

This is important for accurate staging, as about 17% of patients who appear to have “clean” scans actually have microscopic cancer in their lymph nodes [13]. Knowing this helps your team decide on the right follow-up treatment. Additionally, some observational research suggests that removing these nodes may improve long-term survival for patients with Stage I-III disease, though the extent of dissection is an individualized decision made by the surgeon [14].

Post-Surgery: The Decision for Adjuvant Mitotane

After surgery, you and your medical oncologist will discuss adjuvant therapy—treatment given after the “main” treatment to kill any remaining microscopic cells and lower the risk of recurrence [3]. The most common drug used is mitotane.

The decision to use mitotane is individualized and based on your specific risk profile:

  • High-Risk Patients: You are generally considered higher-risk if you have Stage III disease, an R1 or Rx margin (microscopic cancer left at the edge or indeterminate margins), or a Ki-67 index greater than 10% [3]. In these cases, observational studies suggest that mitotane may help reduce recurrences, and guidelines generally recommend discussing it [15][16].
  • Low-Risk Patients: If your surgery achieved R0 margins (completely clear), you are Stage I or II, and your Ki-67 is 10% or lower, you may be considered low-risk [17].

The ADIUVO Trial and Surveillance

For this specifically defined low-risk group, the ADIUVO trial recently provided important evidence [18]. The study compared low-risk patients (R0, Stage I-III, Ki-67 ≤10%) who took mitotane to those who were monitored with frequent scans (active surveillance). The trial found no significant difference in the risk of the cancer returning between those who took the drug and those who didn’t [18][19].

Because mitotane has significant side effects, many experts now suggest that patients fitting the ADIUVO profile can safely choose active surveillance instead of immediate medication [17][18]. Your multidisciplinary care team will help you weigh the toxicities of the drug against your personal risk of recurrence.

Common questions in this guide

What type of surgery is usually used for adrenocortical carcinoma?
For most suspected or confirmed adrenocortical carcinomas, open surgery with an en bloc resection is the standard approach. The goal is to remove the tumor in one piece without rupturing its capsule, sometimes along with nearby organs involved by the cancer.
Why should ACC surgery be performed at a high-volume center?
ACC is rare and can be technically difficult to remove, especially when it involves nearby organs. An experienced adrenal cancer surgeon and multidisciplinary team can plan complete removal, lymph node assessment, and appropriate hormone or steroid management.
Are lymph nodes removed during ACC surgery?
Guidelines recommend that the surgeon strongly consider removing nearby regional lymph nodes to check for microscopic cancer spread and improve staging. The number and extent of lymph node removal depend on the tumor and the surgeon’s assessment.
How is the decision about mitotane made after ACC surgery?
The decision depends on recurrence risk, including the cancer stage, margin status, and Ki-67 level. Mitotane is generally discussed more strongly for Stage III disease, cancer at or near the margin or an uncertain margin, or a Ki-67 above 10%; some patients with clear margins, earlier-stage disease, and Ki-67 of 10% or lower may be candidates for surveillance.
Can active surveillance be an option instead of mitotane after ACC surgery?
For selected low-risk patients with complete removal, clear margins, Stage I to III disease, and a Ki-67 of 10% or lower, the ADIUVO trial found no significant difference in recurrence between mitotane and close imaging surveillance. Whether surveillance is appropriate depends on the pathology and the multidisciplinary team’s assessment.
Why is minimally invasive surgery often avoided for ACC?
Open surgery usually gives the surgeon better visibility for removing the tumor in one piece and protecting clear margins. Laparoscopic surgery may be considered only for carefully selected small, non-invasive tumors when performed by an extremely experienced oncologic surgeon, because tumor rupture or incomplete removal can increase recurrence risk.

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References

References (19)
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    American Association of Clinical Endocrinology Disease State Clinical Review on the Evaluation and Management of Adrenocortical Carcinoma in an Adult: a Practical Approach.

    Kiseljak-Vassiliades K, Bancos I, Hamrahian A, et al.

    Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2020; (26(11)):1366-1383 doi:10.4158/DSCR-2020-0567.

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    Laparoscopic Versus Open Adrenalectomy for Localized/Locally Advanced Primary Adrenocortical Carcinoma (ENSAT I-III) in Adults: Is Margin-Free Resection the Key Surgical Factor that Dictates Outcome? A Review of the Literature.

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This page is for informational purposes only and does not constitute medical advice. Your adrenal surgeon, endocrinologist, oncologist, and multidisciplinary team should tailor surgery and post-operative treatment to your specific tumor and health.

Guide contents adrenal carcinoma
Research & Literature
02 Staging and Your Pathology Report 04 Managing Mitotane and Your Hormones