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The patient had the full suite of symptoms — and a jaw that somehow moved out of alignment. That was the clue something was wrong.
The 53-year-old woman bit into her salad, enjoying the crispness. But as she swallowed, a chunk of lettuce stuck in her throat. She coughed, and it loosened and came back up. It was a problem she had frequently these days. Her teeth on the right side of her jaw didn’t touch anymore. It was hard to chew her food well. What was really upsetting was that she’d had this problem before — for most of the previous year. When she couldn’t put up with it anymore, she’d gone to her dentist, Dr. Robert Souferian, in Bay Ridge, Brooklyn, who confirmed that her bite had shifted a little. He was reassuring, said that it happened sometimes and offered to file her teeth a little to make the fit better. That worked beautifully — for a few months. But it was now clear that her jaw had shifted again.
The woman studied her face in the bathroom mirror. She ate a good diet and did yoga at least once a day. She did everything she could to stay healthy and keep in shape, but she could see that, somehow, she wasn’t aging well. And she wasn’t the only one who noticed. On a recent Zoom call, her mother, with her loving but brutal maternal honesty, said, “Your eyes look awful.” She then joked that maybe the two of them should get “a little work done” together once the pandemic was over. She knew what her mother meant. She had bags under her eyes, and the skin around them was puffy. Her face shone with sweat from near-constant hot flashes. She hardly recognized the face that looked back at her.
She returned to Souferian’s office and asked if he could file her teeth once more. This time the dentist looked worried. “That doesn’t make sense,” he told her. Why would her teeth move again? He examined her mouth and jaw carefully. He could see that her bite had shifted, but he couldn’t see why. He was concerned that she could have a growth or tumor inside one of her temporomandibular joints. She wasn’t tender there, and he couldn’t feel anything. Still, it was a possibility he couldn’t ignore. He referred her to a colleague who could do a 360-degree X-ray to look for a mass. When the X-ray didn’t show anything, that dentist sent her for an M.R.I. of the jaw.
Her jaw was normal, she was told a few days later. There was no tumor there. However, the dentist continued, the radiologist saw something else she needed to know about. He saw a lesion on her pituitary gland. She should see a neurologist to get that checked out.
A Closer Look at the Master Gland
The pituitary gland is just below the middle of the brain, at the level of the eyes. It used to be called the “master gland” because it secretes the hormones that run many of the body’s functions: growth, metabolism, lactation, sex drive and reproduction. She wasn’t sure what it meant to have a lesion there, but she immediately took the dentist’s advice and made an appointment with a neurologist. The doctor was a middle-aged man with a tidy gray beard and a warm, slightly accented voice. He heard her story and reviewed the M.R.I. of her jaw. He agreed that she needed an M.R.I. of the brain so they could see all the surrounding structures and get a better look at the lesion.
After the scan she got a call from the neurologist. He confirmed that she did have a lesion on her pituitary. The good news: It wasn’t cancerous. The bad news was that it was large. It would have to be removed. When she heard the word cancer, the woman’s heart began to race. She never even considered the possibility of cancer. She was relieved it wasn’t that. But what did he mean, she would have to get it removed? Did she need brain surgery? “You don’t have to get this done tomorrow,” he answered gently, “but it should be removed within the year.” She would need to see three more doctors, he continued: an ophthalmologist, because the gland and its growth were just above the optic nerves and could affect her vision. She would also need an endocrinologist, and finally she would certainly need a neurosurgeon.
She saw the ophthalmologist first. No problem there. That was a relief. She hadn’t felt as if there was anything wrong with her vision, but she hadn’t felt there was anything wrong with her brain either. Then it was back to the internet to find an endocrinologist. She found one who had good reviews and was covered by her insurance. Amazingly, she was able to make an appointment for the next day.
Dr. Joan Cantero, the endocrinologist she found, spoke with an air of quiet confidence. The mass was big, Cantero noted, about the size of a marble, which made it twice the size of the gland it was affecting. It would definitely have to be removed.
Before her appointment, the patient had done some investigating. She read about pituitary tumors on the internet and asked all her doctor friends. It wasn’t the kind of brain surgery often shown on TV where the head is shaved, the skull cut open with a saw and the surgeon cuts into the puddinglike tissue of the brain itself. This type of brain surgery was done with a scope inserted into the nose, removing the tumor from below. By the time she saw Cantero, the woman was eager to do whatever was needed. As awful as the idea of surgery was, the thought of something growing back there was scary and creepy.
Checking an Old Photo
Not so fast, Cantero warned. First, they needed to see if the tumor was making any of the pituitary’s hormones. The doctor suspected the patient had an excess of one of these hormones — an overproduction of growth hormone will cause unregulated enlargement of the soft tissues throughout the body, a disorder called acromegaly. The patient was a small woman, but, the doctor noticed, her hands and feet were huge. Can you take your mask down? Cantero asked. And show me an old photo? The difference in the two faces added to Cantero’s clinical suspicion. But a diagnosis like this requires more than suspicion. Cantero sent the patient to the lab, where a half dozen tubes of blood were drawn and sent off. She returned to the endocrinologist’s office two weeks later. Her growth-hormone levels were nearly five times what they should be: She had acromegaly. The woman had surgery two weeks later.
Acromegaly is rare. It is most extreme when the oversecretion of the hormone starts before puberty, when bones can still grow. André Roussimoff, more familiarly known as André the Giant, was 7-foot-4 when he finally stopped growing taller. After puberty, when bone growth stops, only soft tissues will enlarge. That can still cause serious changes in appearance and in health. Untreated, patients with acromegaly will often have obstructive sleep apnea from enlarging tissues in the mouth and throat, high blood pressure, joint breakdown and sometimes an enlarged but weakened heart. This patient, it turned out, had everything except the enlarged heart.
After getting this diagnosis, the patient immediately started reading up on the disease. If asked before her diagnosis was made, the only symptom she would have identified was the crooked jaw. Reading about other people’s experiences, she realized how many of the irritations and medical issues she’d been through were from this excess of growth hormone and not, as she had assumed, from the effects of menopause on an active life and an aging body. She’d seen changes in her face. Her hands were so big she couldn’t wear rings. Her feet were enormous. For most of her adult life, she wore a size 8½ shoe. By the time she had her surgery, her feet were so wide she wore a men’s size 9½. Her tongue was so large that she often bit it, and she had sleep apnea. She also had high blood pressure.
She was thin for her entire life but needed a knee replacement at 49. She was hot all the time and sweated like crazy. Menopause, she figured — until she read about this tumor.
Two days after leaving the hospital, she could fit into her mother’s shoes, a woman’s size 8½. She’s no longer hot and sweaty all the time. It sounds minor, she told me, but that was one of the worst parts of the whole ordeal. And a year after her surgery, she tells me that she looks at least five years younger. Her acquaintances suspect a face lift. Her friends know it was a different kind of surgery. Best of all, she has watched as her face has slowly reverted back to the one she knew so well.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write her at Lisa.Sandersmdnyt@gmail.com.