Odd Symptoms Traced to Rare Pituitary Disease
Over the last several years, John Sekula, 52, of Wauwatosa, noticed strange things happening to his body. Little did he know, one tiny gland could explain all of his symptoms.
"Four or five years ago,” said John Sekula of Milwaukee, “I saw a flier at work about echocardiogram tests for staff, so I got one. I was surprised the test showed I had a leaky aortic valve. My primary care physician has watched it over the years. So far, no treatment has been needed.”
Around the same time, John’s wife, Patsy Coccia, suggested John get tested for sleep apnea due to snoring. “I stopped breathing about 90 times per hour,” John said, “so I was put on a CPAP (pressurized air)machine at night.
“About three years ago, I developed an open bite— I couldn't bite on my front teeth. My dentist adjusted my molars.”
A year later, John developed an underbite. Other unusual body changes followed. John’s shoe size increased. His hands were cold and became numb during sleep. He had benign skin growths — and so on. John began to look for answers and learned about a condition called acromegaly. “I had all the symptoms,” he said.
A Rare Pituitary DisorderAcromegaly, a rare condition, is caused by excessive secretions of growth hormone from the pituitary gland. It occurs in six of every 100,000 adults. In more than 90 percent, a benign tumor (an adenoma) is responsible for producing the excess growth hormone.
Prolonged exposure to excess growth hormone causes abnormal growth patterns in adults and gigantism in children. In adults, it can cause enlargement of the hands, feet and jaw, and swelling of the body’s soft tissue. Symptoms are caused by the tumor pressing on surrounding tissues and excess growth hormone in the blood. Acromegaly is associated with an increased risk of diabetes, heart attack, high blood pressure and certain types of cancer.
Because of its slow progression and symptoms that appear unrelated, the disease is hard to diagnose in the early stages.
“A pituitary disorder diagnosis can easily be overlooked,” said Medical College of Wisconsin endocrinologist Kawaljeet Kaur, MD. “From symptom onset to diagnosis, it could be several years.”
In March 2009, John called his physician to have a growth hormone test. “My growth hormone level was three times what it should have been.” An MRI scan revealed that a tumor (benign macroadenoma) on John’s pituitary gland was causing the overproduction of growth hormone. John’s pituitary tumor was 2.4 cm (.9 inches) — larger than the pituitary gland itself.
About Pituitary Diseases“The pituitary gland —the size of a pea — is connected to the hypothalamus in the brain,” Dr. Kaur said. “It’s considered the ‘master’ gland because it secretes a number of significant hormones that control other glands of the body.”
Most diseases of the pituitary gland involve tumors in the pituitary or hypothalamus. Although most pituitary tumors are benign, when a tumor causes the pituitary gland to produce too much or too little of a hormone, serious health problems occur.
Endocrine ExpertsPatsy asked her doctor, Medical College of Wisconsin otolaryngologist Todd Loehrl, MD, to recommend a physician for John. Dr. Loehrl recommended Medical College of Wisconsin endocrinologist Diana Maas, MD. She and other Medical College of Wisconsin endocrinologists have expertise in treating even the rarest pituitary tumors.
“Roughly 15 percent of intracranial tumors are pituitary tumors, and more than 90 percent of pituitary tumors are benign,” Dr. Maas said. “Most are non-secreting tumors that don’t grow or require treatment. Because of improved imaging such as MRI and CT scans, more pituitary tumors are being detected. This often occurs when someone is having a scan for something else.
“Pituitary secreting tumors can change how a person looks and feels, which may initiate a visit to a doctor. But a growth hormone secreting tumor is often detected much later, because changes in a person’s appearance occur so slowly. A physician who sees a patient regularly for 20 years may not even notice.”
It Takes A TeamThe Pituitary Disorders Clinic at Froedtert & The Medical College of Wisconsin provides comprehensive, timely evaluation and treatment. Treatment is based on a patient’s unique needs, and team members discuss all options with each patient.
“We offer a great team approach to patient care,” Dr. Maas said. “Most patients with pituitary tumors are first seen by a neurosurgeon and an endocrinologist in the Pituitary Disorders Clinic. Our team members discuss treatment for endocrine patients in weekly case conferences.” Other physicians— neuro-interventional radiologists, radiation oncologists and otolaryngologists— are also part of the team, as is a neuro-ophthalmologist. (A large tumor on the pituitary gland can cause headaches and/or affect a person’s vision).
Careful DiagnosisTests for pituitary disease are performed in the Endocrine Diagnostic Unit. An MRI to locate a pituitary or other nearby tumor is done only after confirming blood tests are complete. “Sometimes, a scan can’t detect a growth, but a person still has an over or underproduction of hormones,” Dr. Kaur said. “Growths in the pituitary region may cause pituitary dysfunction, because they compress pituitary tissue.”
Treatment Options“When we find an asymptomatic tumor, its size, its nearness to eye nerves, and whether it is causing production of excess hormone dictates what we do,” Dr.Maas said. “If a pituitary tumor is large enough to compress surrounding structures, or if a small tumor is causing overproduction of growth hormone or ACTH, we remove it.”
Surgery may also remove hormone-producing tissue, reduce tumor volume, or reduce or remove non-hormoneproducing tissue that may threaten surrounding structures.
Because of the gland’s location in the brain, a neurosurgeon works with an otolaryngologist to remove a pituitary tumor. Medical College of Wisconsin neurosurgeon Grant Sinson, MD, and Dr. Loehrl performed John’s surgery.
They used a minimally invasive approach called endonasal transsphenoidal endoscopy. In southeastern Wisconsin, Froedtert & The Medical College are unique in offering this approach. This advanced technique results in minimal blood loss, significantly less post-operative discomfort, and it may reduce possible complications.
“Not all pituitary tumors require surgical removal,” Dr. Maas said. “If a small tumor causes underproduction of a hormone, we replace the hormone with oral medication, injections, a patch or skin gel and simply watch the tumor. Prolactin secreting tumors are usually treated with drugs, regardless of size.”
After SurgeryJohn returned home just two days after surgery. “My soft tissue growth should go back to normal,” John said, “but it may be months to years before I know if all my symptoms will disappear.” To monitor John’s progress, Dr. Maas continues to check his growth hormone levels.
“If you’re having unusual symptoms, headaches or vision problems, see your physician,” Dr. Kaur said. “There are other causes, but if pituitary tumors aren’t considered, the correct diagnosis can't be made. Your physician may refer you to an endocrinologist.”
Medical College of Wisconsin endocrinologists see patients at Froedtert & The Medical College, the Endocrinology Center at Community Memorial Medical Commons, Lincoln Avenue Primary Care Clinic, St. Francis Medical Arts Pavilion and the West Bend Clinic. For more information, call 414-805-3666 or 800-272-3666.
Source: Froedtert Today
Date: September 2009