Adrenocortical carcinoma surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Surgery is the mainstay of treatment for adrenocortical carcinoma.

Surgery

  • Surgery is the mainstay of treatment for adrenocortical carcinoma.
  • Surgery can also be performed even in the case of invasion into large blood vessells, such as the renal vein or inferior vena cava.
  • Large percentage of patients are not surgical candidates.

Appropriate preoperative evaluation and operative planning by a surgeon experienced in the resection of malignant adrenal tumors is of the utmost importance to assure optimal outcome.

Preoperative imaging should be obtained to evaluate the extent of tumor, possible invasion of surrounding anatomic structures, and technical ability of the tumor to be

completely resected. Imaging studies also help to guide the surgeon as to the expected extent of resection required. Imaging should be obtained as close as possible to the anticipated date of surgery, because many aggressive ACCs grow quickly and involvement of adjacent structures may change, thereby altering the operative plan. Intravascular ultrasound or venography may complement other imaging studies to estimate extent of tumor involvement. Other preoperative considerations include management and optimization of those patients with hormone excess, especially those with Cushing’s syndrome due to the numerous deleterious effects of elevated cortisol Although surgery is the treatment of choice for non-metastastatic ACC, the decision for resection of the primary tumor in stage 4 disease needs to be individually addressed. In general, those 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 better control hormone excess, if present (247). Some groups attempt to assess the tempo of disease progression, waiting for several months to restage the patient by imaging and may treat with chemotherapy and/or mitotane in the interval. If tumor burden remains stable or decreases, then surgical treatment is pursued and vice versa. Adrenalectomy in the setting of tumor thrombus within the vena cava (if the tumor is otherwise technically resectable) is reasonable. Obstruction or occlusion of the vena cava by tumor thrombus can lead to significant lower body and gastrointestinal (GI) tract edema, which leads to significant patient suffering. Lack of resection in the setting of vena cava thrombus can quickly lead to death. If tumor resection is not technically feasible for other reasons, 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 long-term durability of hormone control is usually limited as the metastatic disease progresses. The benefits of debulking must outweigh the risks of surgery in these patients who have poor wound healing and lengthy recovery periods due to preexisting debilitation

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