Neuroendocrine tumors

Carcinoid: The tumor that killed Steve Jobs

 

Resources

To learn more about carcinoid cancer and carcinoid syndrome, visit The Carcinoid Cancer Foundation at www.carcinoid.org.


Special to The Miami Herald

In 2007, Albert Garcia, now 59, went to his doctor for a screening colonoscopy. What he learned is that he had cancer in his large intestine. But lucky for him, it was determined to be a carcinoid, not a carcinoma.

A carcinoma starts in the cells that form a particular organ. For example, breast cancer grows in the breast ducts (or lobules) and colon cancer arises from the cells lining the colon wall.

“But carcinoids stem from very specialized cells,” says Dr. Seza Gulec, director of surgical oncology, Jackson North Medical Center/FIU.

Your body has a well-organized nervous system that includes the brain, cerebellum, spinal cord and nerve tissue. But it also has a more diffuse system made up of individual cells or clusters of cells found throughout the body.

“This is the neuroendocrine system and it’s unique,” says Gulec, who has seen Garcia since he moved to Wellington about a year ago.

Through the production of hormones, these cells fine-tune how organs function. And it’s these neuroendocrine cells that give rise to carcinoid tumors.

Today, these tumors are synonymous with a low-grade neuroendocrine tumor that can be found just about anywhere in the body, says Dr. Pasquale Benedetto, the Leonard M. Miller Professor of Medicine at Sylvester Cancer Center.

Carcinoid, which means “carcinoma-like,” was first characterized in 1907 by Siegfried Oberndorfer, a German pathologist.

“Under the microscope, carcinoids look like carcinomas but they behave better than most cancers,” Gulec says.

No one knows what causes these tumors, which can be either benign or malignant. They are relatively indolent, or slow growing. But over time, they, like any cancer, can squeeze out healthy cells and cause problems in the organs they occupy.

Carcinoids recently garnered attention when Apple co-founder Steve Jobs was diagnosed with a neuroendocrine tumor in the pancreas that metastasized to the liver. It proved fatal.

“You can’t ignore a carcinoid, absolutely not,” Gulec says.

According to the American Cancer Society, about 8,000 neuroendocrine tumors are diagnosed each year in the gastrointestinal tracts (the stomach, intestine, appendix, colon or rectum) of patients in the United States. These account for about 90 percent of all neuroendocrine tumors.

These tumors can also start in the lungs, pancreas and ovaries, although a small number develop in other organs. The incidence of carcinoid tumors has been increasing but doctors don’t know why, according to the ACA.

Often these tumors are hard to find and diagnosed only incidentally, says Christina Gomez, a medical oncologist at Holy Cross Hospital’s Michael and Dianne Bienes Comprehensive Cancer Center.

For example, you, like Garcia, may be having a routine colonoscopy or a chest CT scan when a carcinoid tumor in your intestine or lungs is identified. Or you might suffer appendicitis only to discover the blockage is caused by a carcinoid tumor. Many more are only found post mortem.

After a carcinoid tumor is noted by your pathologist, your doctor may run tests to determine the cancer’s rate of growth. As cells divide, they give off a protein that can be stained and seen under the microscope. The more of this protein that is present in the tissue sample, the more cell division and the faster the tumor growth.

A scan using radioactivity may also be used to look for concentrated areas of neuroendocrine cells throughout the body, which may suggest tumors.

“If you get hot spots, you also can often determine if there’s metastasis,” Benedetto says.

In theory, carcinoid cancers can grow wherever there are neuroendocrine cells so they can metastasize. Tumors of the pancreas and GI tract most often spread to the liver. Tumors in other locations reach to the liver, lungs, bones and lymph nodes.

The most common metastasis is to the lymph nodes and then the liver, Gulec says.

Also, neuroendocrine cells produce hormones. When a carcinoid tumor develops, it can give off excess serotonin that can cause carcinoid syndrome. The syndrome is characterized by flushing, wheezing, feeling both hot and cold, diarrhea, abdominal cramps and, after a time, heart problems.

For those diagnosed with carcinoid syndrome and carcinoid tumors, there’s good news.

“You have treatment options,” Gomez says.

For about the past nine months, Judith Meckler, 75, of Boynton Beach, hasn’t been herself. She’s been feeling “major exhaustion” and suffered vomiting and severe diarrhea resulting in a weight loss of 25 pounds.

A pathologist determined she has a carcinoid tumor in her pancreas that has spread to her liver, says her daughter Lori, who lives in Sunrise.

One treatment option for carcinoid patients is surgery.

Although most doctors try to avoid surgery for late-stage cancers that have spread to the liver and other organs, with carcinoids they still may operate.

“We know the tumor has a very slow growth rate and we can change the course of the disease by removing as much of the tumor as possible,” says Gulec.

Benedetto recalls a patient who had a tumor in his ileum with metastasis to his liver and the lymph nodes in his abdomen. The surgeon resectioned it all during the course of one surgery and to this day the patient returns for checkups but remains disease free.

Another option to fight tumors is the use of ocreotide (Sandostatin) injections. Ocreotide is a drug that binds with receptors on cancerous cell walls, helping to prevent them from dividing, growing and producing serotonin.

“This is the one and only effective drug for patients with carcinoid syndrome,” Gulec says.

For tumors that have metastasized to the liver, like those of both Garcia and Meckler, doctors may use chemotherapies that block the blood flow to specific areas of the kidney to starve the tumors, Gomez says.

Or they might insert a probe into the liver tumors to break them up with radio frequency, or more recently, zap them with microwaves.

“It’s just like the microwave in your kitchen that heats from the inside out,” Gulec says.

An alternative is to inject radioactive beads through the blood vessels in the leg. The radioactivity collects in the liver tumors to irradiate and kill them.

“This internal radiation provides high precision with much less damage to the normal liver tissue” Gulec says.

After having a second surgery for liver metastasis, Garcia has good news.

“For five years, I’ve been disease free,” he says. But he still has to return to his doctor every six months for follow-up tests.

Judith Meckler is undergoing additional tests, including scans, to help find any additional tumors and determine a treatment plan.

“When I found out her pancreatic tumors were carcinoids and learned more about this cancer, I felt a little more optimistic about the outcome,” says her daughter, Lori.

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