Could brain surgery solve her baffling symptoms?
By the time A.A. arrived in my office, she had spent almost a year looking for answers. In November 2012, she was 45 and struggling to lose weight and keep her blood pressure down. What sounds like a common scenario, however, was anything but.
By the time A.A. arrived in my office, she had spent almost a year looking for answers.
In November 2012, she was 45 and struggling to lose weight and keep her blood pressure down. What sounds like a common scenario, however, was anything but.
A.A. was experiencing fatigue and malaise, and the area around her eyes bruised easily. Another puzzling symptom: She said she was acutely aware of her neck. It wasn't pain, but awareness. She was losing more hair than usual in her brush and had stopped menstruating, and her skin broke open easily. Her primary-care physician thought it was early menopause.
She asked family and friends, but no one had such symptoms at menopause. She was increasingly self-conscious as she gained weight. Her primary-care provider referred her to an OB/GYN, and a variety of tests came back normal, including a pap, thyroid, female hormones, and a transvaginal ultrasound.
Worst of all, A.A. struggled emotionally. She felt as though she were in a constant state of agitation, with depression and anxiety. A.A.'s symptoms slowly took over her life. She was becoming a person she hardly recognized.
In July, she ran into a friend who was a nurse. Noticing the puffiness of her face, the nurse asked A.A. whether she was on prednisone. Learning she wasn't, the nurse suggested A.A. might have Cushing's syndrome, which results from too much cortisol in the body for long periods. It can be caused by taking a corticosteroid, like prednisone, or by something inside the body signaling the adrenal glands to produce too much of the hormone.
A visit to an endocrinologist confirmed the diagnosis after a 24-hour urine-cortisol test, and an MRI appeared to reveal a small adenoma on the pituitary gland. The endocrinologist referred her to Jefferson to see a surgeon.
Although she was not looking forward to brain surgery, A.A. was relieved to have an answer.
But neurosurgeon James Evans, Jefferson's director of pituitary surgery, did not think the Cushing's was caused by the pituitary adenoma. He ordered an additional MRI and blood work, which confirmed his hunch, and he referred her to Jefferson Endocrinology for further detective work.
When A.A. walked into my office, she was extremely stressed and exhausted. I ordered a chest CT, which revealed a nodule. But it did not fluoresce during a nuclear medicine test, as it likely would have had it been causing the Cushing's. Next up was a series of scans, but all came back clear.
I still felt the tumor should come out and referred her to cardiothoracic surgeon Scott Cowan.
Three days after surgery to remove one lobe of her lung and the tumor, A.A.'s face already was noticeably slimmer.
Her Cushing's was caused by a carcinoid tumor the size of a pencil eraser in her lung. The tumor - although not large enough to fluoresce during testing - had been signaling her adrenal glands, which produced enough cortisol, the fight-or-flight hormone, for 24 people.
Cushing's accounted for all her physical and emotional symptoms. The syndrome can be missed because it mimics obesity in many ways.
With the tumor out, her adrenal glands would effectively go to sleep. She'd need prednisone, which would slowly be tapered over the next year. Fortunately, A.A.'s lymph nodes were clear, and she did not need radiation or chemotherapy.
Over the next year, A.A. got her life and her body back. By January, A.A. was completely off prednisone, feeling and looking like herself.