On certain days my Tampa office turns into a shelter of sorts for those who are lost without a diagnosis, and when I first saw Debbie a year ago she appeared to be the most stranded of all. A 49-year-old mother of four, she looked tired, like a pelican stuck on top of a pole in the gulf, uncertain as to whether she could ever fly home.
Debbie was anxious and desperate for answers. She had a growth on her face that had been there for a year; she told me that it “itches and bleeds.”
I performed a routine biopsy and found she had a basal cell skin cancer. When the lab report came back I also found that my initial biopsy had removed all of the cancer and that the margins were clear. I was as pleased as Debbie that no more work seemed necessary.
But on her second visit, I realized that our optimism had been misplaced. It quickly became apparent that she had a plethora of other conditions that needed to be woven together into a coherent diagnosis.
“I’ve been losing my hair,” she said, indicating the top of her scalp. “And lately I feel like my legs are getting thinner but I’m getting thicker in the center.”
“You’ve gained weight?”
“I’ve put on about 40 pounds, and it feels like it’s all right here in my gut,” she said, pointing.
I reviewed her medical and social background. She had a history of gallbladder and ovarian cyst surgery, smoked a pack of cigarettes every three days, and said she was not an alcohol abuser. In her records I saw she had suffered from depression.
“How are you doing now?” I asked regarding that part of her life.
“It’s gotten worse,” she said. “I tried Prozac and it seemed to help for a while, but now it just doesn’t. And I get agitated a lot.”
“More than in the past?” I asked.
“A lot more,” she admitted.
In fact, her depression had been quite severe at times, twice requiring hospitalization.
“What else?” I asked.
“While I’m losing hair up here,” she said, patting the top of her head, “I’ve got hair everywhere else—just like a man. And I’m only getting my period every three months or so.”
On examination, I saw that she did have hair loss at the top of her scalp and excessive hair growth elsewhere. There was puffiness on her upper chest above the clavicles. I also noticed that she had some bruising on the skin of her arms. She wasn’t taking steroids, which can bring on bruising, and her skin was not abnormally thin. I ordered some blood tests, including a reading of testosterone levels, and scheduled a follow-up visit for two weeks later.
In women, hair on the chin, upper lip, or arms and legs can create an appearance of old age or masculinization and can be quite psychologically disturbing. Hirsutism is the medical term for such excess hair in places where it should grow only on adult men. It is usually caused by an increased sensitivity to or increased production of hormones called androgens (testosterone and its metabolites). A disorder known as hyperandrogenism—increased levels of male hormone production in women—affects up to 10 percent of all women and commonly brings on irregular menstrual cycles.
At her next appointment, Debbie appeared even more agitated. I checked her testosterone results, which proved to be elevated. A number of causes for this needed to be considered. One common condition that leads to hyperandrogenism is polycystic ovary syndrome (PCOS). One in 10 women has PCOS, a condition that causes cysts to grow in their ovaries. Along with high levels of androgens, it can create irregularities in the menstrual cycle and trigger excessive hair growth.
Tumors of the adrenal cortex can also bring on these symptoms. Identifying a tumor and distinguishing which kind of tumor it might be—benign (an adenoma) or malignant—requires specialized scanning techniques and hormone investigations. Debbie’s abnormal blood test results, with her overproduction of hormones, indicated the need to do more imaging studies. So I ordered an abdominal CT scan and told the radiologist to get back to me with her findings as soon as she could.
The adrenal glands are triangular, orange-colored endocrine glands that sit atop both kidneys. They are primarily responsible for regulating the stress response, including the secretion of cortisol and adrenaline. The adrenal cortex secretes a variety of steroid hormones including cortisol, aldosterone, and testosterone. A tumor of the adrenal cortex of any kind can elevate steroid hormones and manifest a variety of clinical behaviors, such as anxiousness, and symptoms like the bruising and puffiness of Debbie’s upper chest.
The next day, one of my nurses caught me as I left an examining room. She told me that the radiologist was on the phone about Debbie.
“Dr. Norman,” the radiologist said, “this looks like an adrenal tumor.”
The next step was clear. I had referred Debbie to an endocrinologist to rule out disorders such as Cushing’s syndrome, a serious condition marked by weight gain (but not on the limbs), bruising, and even male-pattern hair growth in females. It was a possible diagnosis, but the endocrinologist quickly ruled it out.
Now, in order to move forward with our new information, we needed to determine whether the tumor was functional (hormonally active), nonfunctional, or malignant. The treatment for a hormonally active adrenal tumor was surgery. If the tumor was malignant, the treatment would depend on the cell type, spread, and location of the primary tumor.
The size of the tumor is considered an important predictive characteristic: Larger tumors are more likely to be malignant. Debbie’s was fairly small, a very good prognostic sign.
Expensive additional testing and invasive procedures are necessary to rule out the slight possibility of an early adrenocortical carcinoma, a rare form of cancer.
Luckily, Debbie had an adrenocortical adenoma—a “functional” tumor, meaning that it produces hormones including glucocorticoids, mineralcorticoids, and/or sex steroids. In doing so, such tumors can cause endocrine disorders that, among other things, feminize men or masculinize women. The good news is that functional adrenocortical adenomas are surgically curable.
The adrenal gland—no bigger than a walnut—has an effect on the body far out of proportion to its size. In most cases, adrenocortical adenomas are less than 2 centimeters in diameter and weigh less than 50 grams, or about 2 ounces.
Now was the time to consult my friendly neighborhood surgeon. Unlike the characters in House, doctors in the real world welcome other physicians to do surgical procedures such as the unilateral adrenalectomy that Debbie required. The surgery is usually a minimally invasive laparoscopic procedure.
After the adrenocortical adenoma was removed, Debbie improved greatly. “I feel a lot more like Debbie again,” she told me. Her disposition was back to normal, and before long she had a new job and began losing some of the excess weight and hair.
I was also happy that, as she returned to an active life, she promised to take my advice about using sunblock to prevent the kind of skin cancers that had brought her to my office in the first place.
Robert Norman is a dermatologist based in Tampa, Florida. The cases in Vital Signs are real, but names and certain details have been changed.
