2-surgeon Bilateral Adrenalectomy May Be Better for Ectopic Cushing’s

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by Margarida Maia |

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A two-surgeon approach to bilateral adrenalectomy — the surgical removal of both adrenal glands — may lead to fewer complications and shorter operating time in patients with ectopic Cushing’s syndrome, compared with other surgical approaches.

That finding from a small study, “Adrenalectomy in ectopic Cushing’s syndrome: a retrospective cohort study from a tertiary care centre,” was published in the Journal of Neuroendocrinology by a team of researchers in Sweden.

Cushing’s syndrome comprises a group of conditions characterized by the excessive levels of the stress hormone cortisol. Cushing’s disease, a form of Cushing’s syndrome, is caused by the brain’s pituitary gland making too much adrenocorticotropic hormone (ACTH), which stimulates the production of cortisol by the adrenal glands.

Ectopic Cushing’s syndrome, a rarer form of Cushing’s syndrome, is caused by tumors found outside the pituitary gland that release large amounts of ACTH. Some of these tumors may be neuroendocrine neoplasms, which are abnormal masses of tissue arising from cells of the nervous or endocrine (hormonal) systems.

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The treatment of neuroendocrine tumors “is challenging” and “there is no established evidence-based recommendation,” the researchers wrote. However, some treatment options are available.

When tumor removal is impossible, adrenal inhibitors — also known as steroidogenesis inhibitors — may offer a way of lowering blood cortisol levels. Another way to cut down cortisol is to perform a bilateral adrenalectomy, which is a surgery to remove both adrenal glands.

There are various approaches to bilateral adrenalectomy. Open surgery involves cutting the skin and tissues to expose the adrenal glands. A less-invasive approach, laparoscopy, uses a small cut to view the adrenal glands with the aid of a camera. Because it causes less pain and allows patients to recover faster, laparoscopy is considered the gold standard approach for bilateral adrenalectomy.

Laparoscopic transperitoneal adrenalectomy (LTA) is the most commonly used laparoscopic technique for bilateral adrenalectomy. The most recent posterior retroperitoneoscopic adrenalectomy (PRA) technique, however, is thought to provide a series of advantages, including less time in the operating room, less blood loss, and faster recovery. It can be performed by one or two surgeons, which may further reduce the time of the procedure.

To understand which approach is best for those with ectopic Cushing’s syndrome, the research team reviewed the medical files of 53 patients — 35 women and 18 men, with a mean age of 53 — who underwent bilateral adrenalectomy from 1986 to 2019.

The primary tumor was located in the thorax in 30 patients and in the pancreas in 14 patients; in the other nine patients tumor location was unknown.

Bilateral adrenalectomy was performed on 37 patients (70%) at a median of two months after receiving a diagnosis of ectopic Cushing’s syndrome. From these, 19 had open surgery, eight underwent LTA, and 10 had PRA.

Prior to surgery, 32 patients (86%) had taken adrenal inhibitors to control their cortisol levels. After surgery, ectopic Cushing’s syndrome resolved completely in 33 patients (89%). In the other four patients, high cortisol levels remained after the procedure, since surgery was less extensive than intended due to certain limitations.

Mean operating time was 1.5 times longer for open surgery than for laparoscopy (246 vs. 162 minutes). PRA performed simultaneously by two surgeons had the shortest operating time (94 minutes). Median hospital stay also was longer for open surgery than for laparoscopy (33 vs. nine days).

The most frequent surgical complication was infection. Severe complications occurred during or after surgery in 12 patients (34%), 10 of whom had undergone open surgery.

“Complication rate was high, probably as a result of the combination of metastatic [invasive] disease and metabolic disorders caused by high cortisol levels,” the researchers wrote.

However, there were fewer severe complications in patients undergoing laparoscopy than in those undergoing open surgery. There were no deaths related to bilateral adrenalectomy.

The median survival time after bilateral adrenalectomy was 24 months, or about two years, and there were no differences between the different approaches.

“Our results indicate that PRA performed by two surgeons simultaneously is the method of choice for patients with [ectopic Cushing’s syndrome],” the researchers concluded.

Yet, the team also noted that despite allowing for a shorter operating time and being associated with fewer complications, “the endoscopic approach did not seem to improve overall survival.”