HERSHEY, Pennsylvania—Thyroid cancer accounts for about 3% of all cancer cases in the United States, and only 2%-3% of those are medullary thyroid carcinoma (MTC). The rare and rather aggressive disease is diagnosed in fewer than 1600 Americans annually.

David Goldenberg, MD, FACS, chair of the department of otolaryngology-head and neck surgery at the Penn State University (PSU) College of Medicine in Hershey, Pennsylvania. ​Credit: PSU

Yet if caught in time, MTC is quite manageable, said David Goldenberg, MD, FACS, a surgical oncologist and professor and chair of the department of otolaryngology-head and neck surgery at Penn State University College of Medicine in Hershey, Pennsylvania. 

“Medullary thyroid cancer can get aggressive and nasty,” but it is not necessarily a death sentence, Dr. Goldenberg told Rare Disease Advisor in a recent interview.

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“As a matter of fact, the vast majority of people—those with early‑stage disease—will do well,” he said. “These patients come in with a unifocal tumor, so it’s only one lobe or one spot. We remove the whole thyroid and adjacent lymph nodes, and they do very well.”

However, “those patients who have large tumors, regional metastasis at diagnosis, or one of the multiple endocrine neoplasia (MEN) syndromes have a more aggressive course,” he explained. “In those patients with MEN syndromes, their disease is multifocal, and they present decades earlier than those who have the sporadic type.”

Dr. Goldenberg, who was educated at Israel’s Ben-Gurion University of the Negev, completed his residency in otolaryngology-head and neck surgery at Rambam Medical Center in Haifa, Israel, then went on to do a 3-year fellowship in head and neck surgery as well as oncology at Johns Hopkins University in Baltimore, Maryland.

The author of 230 journal articles, Dr. Goldenberg recently published Endocrine Surgery of the Head and Neck: A Comprehensive Textbook, Surgical, and Video Atlas. The 512-page book—written by Dr. Goldenberg and some of the world’s top experts on thyroid disease, both benign and malignant—is organized into 7 sections and 62 chapters. That includes a chapter devoted entirely to MTC and others that focus on thyroid surgery overall, including MTC treatments such as tyrosine kinase inhibitors (TKIs), radiation, and chemotherapy.

There are also 29 narrated videos, which Dr. Goldenberg said provide nuanced insights about physical appearance not possible based solely on photographs or illustrations.

“I’m quite proud of this project,” he said. “Unlike a textbook, which is just chapters of facts, the structure of each chapter here has been done taking the modern learner into account. Young men and women graduating medical school and residency now learn differently than us older folks. We used to pore over a textbook, highlight, and read again. That’s not the way it’s done today.”

Besides writing and teaching, Dr. Goldenberg also treats patients who have head and neck tumors, as well as a variety of other ailments.

“Thyroid cancer is overwhelmingly a woman’s disease,” he said, noting that while thyroid cancer, in general, has tripled in incidence in the last 30 years—for a variety of reasons including hormonal variants and increasing obesity —the incidence of MTC has barely increased at all.

Read more about MTC epidemiology

On the other hand, he said, “overall, the prognosis for a patient with medullary thyroid cancer is not as good as people who have papillary or follicular thyroid cancer. MTC can get aggressive and nasty. But that all depends on the stage that it’s found and whether it’s sporadic, familial, or part of a syndrome. All of those things factor into the prognosis.”

Dr. Goldenberg said 70%-75% of medullary thyroid cancer patients have sporadic cancer. Papillary cancer accounts for about 85% of all thyroid cancers, followed by follicular cancer, with 10% of all cases. Anaplastic cancer is the least common (about 1% of cases) and the most deadly type, with a very low cure rate.

“With most direct cancers, the use of thyroid hormone replacement suppresses the return of the cancer,” Dr. Goldenberg said. “In medullary thyroid cancer, because it’s a completely different cell causing it, it has no effect on suppression of the cancer. They take it purely because they need the hormone to survive.” 

Treatment options for MTC are typically surgical in nature, Dr. Goldenberg said. Unlike the more common types of thyroid cancer, MTC does not respond to radioactive iodine.

“Depending on whether it’s sporadic, small or hereditary, or as part of a syndrome, we remove the whole thyroid gland, and also adjacent lymph nodes because medullary thyroid cancer spreads to the lymph nodes relatively quickly,” he said. “In some cases, we have to surgically remove other parts of the anatomy in the vicinity if they are invaded by the cancer.”

Patients whose thyroid cancers have metastasized to the rest of the body may require treatment with TKIs, which target the mutation that most MTCs harbor. Rarely, patients are given chemotherapy, cytotoxic agents, or radiotherapy.

Not much can be done to avoid getting MTC, because, if hereditary, the disease is passed on genetically, in an autosomal dominant fashion.

“However, what patients can do is know that their children may get this disease,” Dr. Goldenberg said. “Sometimes, [children] will undergo a prophylactic thyroidectomy—in infancy, other times in adolescence—depending on the type of MEN syndrome they have.”

Some of those syndromes “come with a 100% chance of getting medullary thyroid cancer,” he said. “Removing the thyroid before it happens is the way to go. But again, this is the minority.”