Ask A Physician
Submit Your Question for Dr. Mark Lewis
Dr. Mark A. Lewis is an assistant professor in General Oncology at MD Anderson Cancer Center and the Vice President of the Hageman Foundation board of directors. In 2009, Dr. Lewis diagnosed himself with Multiple Endocrine Neoplasia type 1 (MEN-1).
Since then, he has written extensively about the process of self-diagnosis and the discovery of his own parathyroid and pancreatic tumors, sharing his story in the Journal of Clinical Oncology and The New York Times. Click here to read his story and his biography.
Q: If there is a tumor, and it is not taken out soon enough, is there a possibility that it can become cancerous? Do many other people get parathyroid tumors?
A: Almost all parathyroid growths in MEN are adenomas (non-invasive) rather than carcinomas (invasive). There is a tiny fraction that are carcinomas, but the treatment is essentially the same: surgical removal. Primary hyperparathyroidism is the most common cause of Hypercalcemia in American outpatients. The difference in MEN patients is that ALL 4 (or more) parathyroid glands are affected, not just one.
Q: If there is a tumor and it is taken out, and the problem still is not fixed, what will happen?
A: Removing 3 or 3 1/2 parathyroid glands should fix the hyperparathyroidism issue for a number of years, likely up to a decade. When the Hypercalcemia recurs, the surgery can be repeated. It is actually usually necessary to take supplemental calcium for weeks, months, or even years after the surgery until the remaining parathyroid gland.
Q: What is the latest treatment regimen if you have to have your entire parathyroid removed?
A: Most surgeons here make an incision at the base of the throat roughly 3-4 inches in length from side to side. They may or may not choose to “auto-transplant” the remaining piece of parathyroid tissue into the arm, depending on which gland is being left behind, where it is in the neck, and how easy it would be to get to with repeat neck surgery.
Q: What is a typical “compressed Mayo week” like? Hours per day, number of appointments, possibility of surgery by end of week?
A: Very difficult to answer. Varies on a person-to-person basis. “Next day” surgery needs to be requested ahead of time, but is generally accommodated. I do know that the number of active endocrine surgeons went from 3 to 2 as recently as December, so wait times might be slightly longer. I can speak from personal experience that Dr. Grant is superb and a world-class surgeon. I had wonderful care from him at the time of my parathyroidectomy.
Q: What does a rising and falling PTH indicate? Can a rising and falling PTH cause pain and bone loss but still have ionized calcium show up normal? What causes the pain associated with high PTH?
A: Variable activity of the parathyroid glands. Most parathyroid adenomas continue to release PTH regardless of the calcium level. In a normal patient, the calcium level feeds back to the parathyroid glands, such that a high calcium ought to suppress PTH release. It’s not the PTH itself that’s likely to cause pain, but rather the high calcium, which typically causes “bones, stones, moans, and groans”, i.e. bone pain, kidney stones, abdominal pain (often related to slow bowel motility/constipation), and neuropsychiatric disturbance
Q: Does a negative genetic test mean that I do not have the genetic mutation that causes Multiple Endocrine Neoplasia (MEN)?
A: I would be extremely cautious in interpreting a “negative” genetic test as meaning that someone does not have MEN1. Remember that 10-15% of patients with a personal or family history consistent with the syndrome will test “negative”. The menin gene is 8000 base pairs long; we currently know of about 1200 errors in that sequence that give rise to MEN1, but there are probably hundreds, if not thousands, more such mistakes that are waiting to be discovered and cataloged.
Imagine if the phone book required people to self-report their number to be included. Some people would give the information right away and could be easily found for years; other people would take longer to give their details and thus more time would elapse before you could find them easily. Hunting for gene defects is a similarly laborious and incomplete process. If I had been tested before 2007, I would have been “negative” too because my specific mutation wasn’t described until then.
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