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Pheochromocytoma crisis is not a surgical emergency

NEW YORK (Reuters Health) – Surgeons shouldn’t rush to operate on patients in pheochromocytoma crisis, a new study shows.

The study, the largest one ever published on patients in pheochromocytoma crisis, finds that emergency adrenalectomy is not necessary because patients can be stabilized medically and have urgent or elective surgery.

“I want to emphasize that pheochromocytoma crisis is a ‘medical’ emergency,” said senior author Dr. Quan-Yang Duh (pronounced “doo”) in a telephone interview with Reuters Health. Dr. Duh has been treating patients with pheochromocytoma at the University of California, San Francisco, for over 20 years.

Dr. Thomas J. Fahey, chief of endocrine surgery at Weill Cornell Medical College in New York, commented via email, “The paper is a nice review of what should be the standard protocol for managing patients with pheochromocytoma – whether in crisis or not.”

Emergency surgery should essentially never be considered for a patient with a pheochromocytoma, Dr. Fahey told Reuters Health. He wasn’t involved in Dr. Duh’s study.

Pheochromocytomas are quite rare. They can present in a variety of ways, from asymptomatic to full-blown crisis with marked hypertension leading to circulatory failure and multiple organ dysfunction.

Mechanisms known to trigger crisis include stress from any type of surgery, trauma and drugs like beta-blockers, corticosteroids, anesthetics and metoclopramide. Crisis can also occur spontaneously.

Adrenalectomy for pheochromocytoma is curative. One to two weeks of preoperative alpha-blockade followed by beta-blockade for refractory tachycardia is necessary to prevent intraoperative hypertension and postoperative hypotension.

Dr. Duh explained that adrenergic receptors are down-regulated with pheochromocytoma. Once the blood pressure is controlled, those receptors are up-regulated and the patients respond to pressors when they become hypotensive after tumor removal.

The study — a review of the authors’ experience as well as the literature on surgery for pheochromocytoma crisis — was done because some controversy surrounds the optimal timing of surgery for patients presenting in crisis.

Altogether, the authors treated 137 patients with pheochromocytoma, including 25 who presented in crisis.

The crisis cohort presented with a variety of complications. Twenty-three patients had severe hypertension, and seven of them also had severe hypotension. Thirteen patients had myocardial infarction, and six had neurologic impairment. Twenty-two had been transferred from other hospitals.

All patients, except one whose preoperative diagnosis was thought to be an aldosterone secreting tumor, were treated with the alpha-blocker phenoxybenzamine for a minimum of 10 days.

Fifteen patients were stabilized, discharged, and readmitted later for elective surgery. The other 10 patients had urgent surgery during the initial hospitalization.

Nine had laparoscopic adrenalectomy. The exception was a patient with multiple endocrine neoplasia type 2B who needed open surgery because of severe ileus and bilateral large tumors. Three of the laparoscopic cases had to be converted to open or hand-assisted procedures.

Patients having urgent surgery had significantly larger tumors, greater need for intra-aortic balloon pumps and intensive care, and more postoperative complications.

None of the authors’ patients died.

In their paper online January 2 in the Journal of Clinical Endocrinology and Metabolism, the research team also reported on a literature review. They found data on 97 patients with pheochromocytoma crisis who had adrenalectomy — and they saw some noteworthy differences between the 64 patients who had elective or urgent surgery and the 33 who had emergency operations.

Specifically, the emergency patients had significantly fewer preoperative diagnoses of pheochromocytoma (p<0.001), more instances of rupture or hemorrhage (p=0.024), and more postoperative complications (p=0.047).

There were six deaths in the emergency surgery group (18%). No one died in the elective or urgent group (p=0.002).

Pheochromocytoma crisis is a medical emergency that should be treated with stabilization and elective or urgent surgery, the authors conclude. In fact, Dr. Duh said he has never seen a patient who could not be stabilized with alpha-blockade.

“Most adrenal hemorrhages are contained,” he added. Those that rupture freely are quite rare and would be the only indication for immediate surgery.

Dr. Fahey agreed, saying emergency surgery will lead not only to uncontrolled hypertension and hemodynamic instability intraoperatively, but potentially irreversible cardiovascular collapse after removal of the tumor. “Once the patient is appropriately blocked, then a laparoscopic resection would be the way to go,” he said.

As for how to accomplish that, Dr. Duh said he tried robotic surgery many years ago but stopped after three cases. He said while robotic surgery might be better for larger lesions, he thinks standard laparoscopic surgery has the advantage of allow the surgeon to feel with the instruments; there is no tactile sensation with robotic surgery. With robotic surgery, however, the instruments can articulate and suturing is much easier.

Dr. Fahey too sees no real advantage to robotic adrenalectomy, which he noted needs larger port sites than conventional laparoscopic surgery. He said, “Perhaps single port robotic approaches will be better, but the laparoscopic approach will almost certainly always be more cost effective.”


J Clin Endocrinol Metab 2013.