Media Contact: John Cramer
Phone: (410) 955-1534
E-mail:
Accurate diagnosis and conservative surgery for a rare adrenal tumor can save lives and adrenal function, according to a collaborative study led by Johns Hopkins. The current standard procedure, removing both adrenal glands, has a high complication rate.
The findings apply to people with a rare adrenal tumor that simultaneously produces two syndromes of excess hormones. Cushing's syndrome involves overproduction of adrenocorticotropic hormone (ACTH) causing excess secretion of the hormone cortisol, which results in muscle wasting, high blood pressure and other metabolic problems. The syndrome of excess catecholamine comes from excess secretion of epinephrine, also causing high blood pressure and other metabolic problems.
Previous studies have shown when these two syndromes combined, the mortality rate was 57 percent, mostly due to complications during and shortly after surgery. Benign adrenal tumors occur in one in 200,000 people, mostly women.
"We now know that accurate diagnosis and surgery can safely cure these conditions and, contrary to previous research, preserve the function of the other adrenal gland," says Robert Udelsman, M.D., the study's senior author and the director of endocrine surgery at Johns Hopkins. "Our results suggest that removal of only the gland with the tumor is enough to eliminate the problem and preserve the long-term function of the other adrenal gland."
The results were presented in April at the annual meeting of the American Association of Endocrine Surgeons and are to be published in the December issue of Surgery.
Standard surgery for these usually benign adrenal tumors, called ACTH-producing pheochromocytomas, is to remove the gland with the tumor and explore and possibly remove the other adrenal gland if it is enlarged. Adrenal glands are atop each kidney and produce hormones and biochemicals. Patients who have both glands removed have to take medication the rest of their lives and are at increased risk for some diseases.
The Hopkins study of four patients treated from 1983-1993 included a worldwide review of all reported cases of ACTH-producing pheochromocytomas. The excess ACTH causes enlargement of both adrenal glands, so in the past surgeons removed both glands to be certain the tumor was completely removed. Hopkins researchers determined which of the two glands contained the tumor, allowing them to remove only that gland.
After surgery, all four patients' hormone, biochemical and blood sugar levels returned to normal and the remaining adrenal gland began working normally again. Hopkins endocrine surgeons remove diseased adrenal glands with a laparoscope.
Other investigators were lead author Herbert Chen, M.D., of Johns Hopkins, John L. Doppman, M.D., George P. Chrousos, M.D., and Lynette K. Nieman, M.D., of the National Institutes of Health, and Jeffrey A. Norton, M.D., of Washington University, St. Louis.
