Sharing Mayo Clinic: Rare tumor triggers life-threatening medical ordeal

Featured News

Sharing Mayo Clinic: Rare tumor triggers life-threatening medical ordeal

By
Joel Streed

January 19, 2020

Diagnosed with a rare neuroendocrine tumor while she was pregnant, Dr. Staci Tanouye and her husband, Dr. Amit Merchea, turned to a team of Mayo Clinic specialists to safeguard Dr. Tanouye's health and that of their baby. Under that expert care, their son was safely delivered and the tumor successfully removed.

When Staci Tanouye, M.D., and her husband, Amit Merchea, M.D., finished their medical training at Mayo Clinic in Rochester, the couple moved to Jacksonville, Florida, and dove into their careers. Dr. Tanouye pursued private practice in obstetrics and gynecology, and Dr. Merchea joined the Division of Colon and Rectal Surgery at Mayo Clinic in Florida.

After they had lived in Jacksonville for several years, the physicians were plunged into a two-year medical odyssey. The ordeal, which at times put Dr. Tanouye's life at risk, took the combined effort of a team of Mayo Clinic specialists to unravel and resolve.

Distressing symptoms

In October 2015, Drs. Tanouye and Merchea went to Germany, where Dr. Merchea attended a medical conference. It was their first extended time away from their 18-month-old son, Niam. Almost immediately upon landing in Dusseldorf, Dr. Tanouye felt sudden chest pressure, heart palpitations and a throbbing thunderclap headache. "I was in so much pain, I couldn't talk," she says. "I curled up in a ball in the airport until the symptoms subsided."

Her symptoms came back later during the trip, sending Dr. Tanouye to a hospital. Physicians diagnosed her with a spontaneous cerebral spinal fluid leak. After four days in the hospital, they recommended she travel back to the U.S. and lie flat on the plane ride home. That trip included another bout of symptoms, and the couple went straight to Mayo Clinic upon their arrival in Jacksonville.

"Every day was a struggle. I had to cut back at work. I couldn't even walk up the stairs in our house to put our son to bed."
Staci Tanouye, M.D.

Dr. Tanouye was diagnosed with reversible cerebral vasoconstriction syndrome — a condition that involves spasms of the brain vessels. A year later, she was diagnosed with an abnormally fast heartbeat — a disorder known as supraventricular tachycardia. She was treated with medication and cardiac ablation.

After that, Dr. Tanouye's symptoms eased long enough for the couple to decide to have another child in 2018. "I work with maternal-fetal medicine specialists, so I was well-educated about what to expect during pregnancy with my diagnosis," Dr. Tanouye says.

During her first trimester of pregnancy, Dr. Tanouye's symptoms, including extremely high blood pressure, returned with full force. Dr. Tanouye had a bad feeling and told her husband she feared she'd either have a stroke or die from her condition. "Every day was a struggle," she says. "I had to cut back at work. I couldn't even walk up the stairs in our house to put our son to bed."

Dangerous tumor

Dr. Tanouye was in and out of the emergency department with severe headaches and high blood pressure. Her care team changed her medications. But at 28 weeks of pregnancy, she was hospitalized at Baptist Medical Center Jacksonville for almost four weeks with high blood pressure that spiked to 260/140, along with an alarmingly slow heart rate. For the first time, the baby became distressed, and his heart rate also decreased. "I could only focus on surviving minute by minute," Dr. Tanouye says. "I left the worrying to Amit."

A maternal-fetal medicine specialist suggested testing for a pheochromocytoma — a rare type of tumor, usually noncancerous, that develops in an adrenal gland. The tumor releases hormones that cause high blood pressure. If left untreated, a pheochromocytoma can result in life-threatening damage to other body systems, especially the cardiovascular system.

The test found that Dr. Tanouye likely had a pheochromocytoma. Then an MRI revealed a 7-centimeter paraganglioma behind the head of Dr. Tanouye's pancreas. Paragangliomas and pheochromocytomas both are types of tumors known as neuroendocrine tumors. Paragangliomas begin in certain nerve cells and, as with pheochromocytomas, they may produce hormones that can cause serious medical problems.

Mae Sheikh-Ali, M.D., an endocrinologist at Baptist Medical Center who had trained at Mayo Clinic in Florida, contacted William Young Jr., M.D., in the Division of Endocrinology, Diabetes, Metabolism, & Nutrition at Mayo Clinic in Rochester, to discuss Dr. Tanouye's diagnosis.

At the same time, Dr. Merchea reached out to his mentors in Rochester, Geoffrey Thompson, M.D., Division of Breast, Endocrine, Metabolic and Gastrointestinal Surgery; and David Larson, M.D., Division of Colon and Rectal Surgery. Dr. Tanouye also contacted her mentor in Rochester, Carl Rose, M.D., Division of Maternal and Fetal Medicine.

"I emailed him: 'Interesting patient; need help with a consult. It's me,'" she says. "Dr. Rose called me and sent all the posters and papers he'd published on this tumor type," Dr. Tanouye says. "He emailed colleagues in Anesthesiology and Surgery to assemble a team to care for me in Rochester. In a very short time, a team had been assembled, stabilizing medications were prescribed, a plan was put in place to transfer me safely from Florida to Minnesota, and dates were set for a C-section and tumor resection."

Seamless care

The couple decided to pursue care in Rochester due to the many uncertainties about Dr. Tanouye's condition. "At Mayo Clinic, we do complicated things every day," Dr. Merchea says. "We make the uncertain familiar. Rochester was the safest place to be." It didn't hurt that Dr. Thompson has more experience with pheochromocytoma and paraganglioma than anyone else in the world.

Dr. Tanouye's symptoms would have been exacerbated by flying, so the couple made the 1,350- mile trip in a minivan, with her reclining in the vehicle. They arrived in Rochester on Dec. 4, 2018, and embarked on two weeks of consultations. A C-section was scheduled for Dec. 18 at 36 weeks' gestation.

"As I was wheeled to surgery, several of my former gynecologic oncology colleagues were waiting outside the OR to wish me luck. It was emotional because they're like family — my Mayo family," Dr. Tanouye says. "It meant so much to know they were operating nearby and available in case anything happened during my delivery."

"Mayo Clinic is the absolute best health care system in the world. The teamwork and our colleagues saved my life."
Staci Tanouye, M.D.

Kiran Merchea was born weighing 6 pounds, 9 ounces. He and his mother were discharged after one night in the hospital. Four weeks later, Dr. Tanouye returned to have the tumor removed. Dr. Thompson says paragangliomas can be tricky to remove because they can wrap around major blood vessels. Despite the potential complications, Dr. Thompson and his team are comfortable managing patients with these tumors due to the team's surgical expertise and the number of patients that they've treated.

The morning after surgery, Dr. Tanouye walked farther than she had in months. With the tumor removed, her symptoms disappeared. But she was found to have a genetic mutation that predisposes her to tumor recurrence, so she'll have annual checkups throughout her life.

Looking back, Dr. Tanouye is grateful for the seamless care she received at Mayo. "Mayo Clinic is the absolute best health care system in the world," she says. "The teamwork and our colleagues saved my life."

Dr. Merchea says the experience has given him a new understanding of what families go through in complex medical journeys and has inspired him to look for ways to help them understand and cope.

The medical odyssey also changed Dr. Tanouye. She quit her busy OB-GYN practice. "I worked long hours and was often on call or at the hospital," she says. "After this ordeal, my family became much more important to me." She took a new job, practicing gynecology part time. She says it gives her better work-life balance. "I realized that my career is miniscule compared to everything else in life."

Note: A version of this story previously was published in Mayo Clinic Alumni Magazine.

HELPFUL LINKS