His labored breathing and exhaustion prompted him to see his physician, who ordered a battery of tests. Aster’s diagnosis: A rare cancer called a neuroendocrine tumor was causing a condition called carcinoid heart disease, and causing his heart to fail.

With treatment at the Cedars-Sinai Neuroendocrine Tumor Multidisciplinary Clinic, Aster is now back on the trail, his bike, his board and the court.

“I went from wondering how long I have to live to understanding that this can be managed,” Aster said. “I feel better than I have felt in years.”

Aster’s symptoms came on slowly over several months: crushing fatigue, bloating, loss of appetite and swelling in his legs and feet. Blood tests ordered by his primary doctor were normal, but a CT scan showed tumors in his liver, which were eventually diagnosed as stage 4 neuroendocrine cancer.

“We received the diagnosis the day we dropped the youngest of our four kids off at college,” Orr said. “We had other plans, so we just had to reset.”

Unable to sleep the night they got the news, Orr—who Aster said was his caregiver and support throughout this difficult time and credits with helping save his life—spent hours online. She clicked her way to the Neuroendocrine Tumor Research Foundation. The organization, a resource for research funding, patient education and support, directed the couple to  the Neuroendocrine Cancer Foundation, which referred them to Cedars-Sinai and its multidisciplinary clinic.

“We were concerned about Ken’s neuroendocrine tumors, which had spread to his liver and were making it difficult for his stomach to work normally,” said Andrew Hendifar, MD, who leads the clinic and is a professor of Medicine at Cedars-Sinai. “But we knew from experience that his heart failure would have to be treated first. We didn’t have a minute to lose.”

Neuroendocrine tumors begin in neuroendocrine cells, which connect the nervous system with the hormone-producing endocrine system. They can occur anywhere in the body.

Aster’s began in his small bowel and produced a hormone called serotonin, which thickened two of his heart valves and caused them to leak. The condition is called carcinoid heart disease.

Prashanth Venkatesh, MD, assistant professor of Cardiology in the Smidt Heart Institute at Cedars-Sinai, is the cardiologist on the neuroendocrine team.

“The pulmonary valve, which sends blood from the heart to the lungs, is notoriously difficult to evaluate with standard imaging techniques because of its location in the chest and the relative rarity of pulmonary valve disease in adults,” Venkatesh said. “Significant pulmonary valve involvement in carcinoid heart disease is hence very easily missed.”

Venkatesh, a congenital heart specialist, often employs more extensive imaging techniques for patients born with structural heart defects. Aster’s results showed there was a leak in his pulmonary valve as well as a leak in his tricuspid valve. Both valves needed to be replaced.

“When there is a leaky valve, which we call regurgitation, some of the blood in the heart goes in the wrong direction,” said Raj Makkar, MD, vice president of Cardiovascular Innovation and Intervention at Cedars-Sinai and the Karsh Distinguished Chair in Interventional Cardiology in the Smidt Heart Institute. “With leaky pulmonary and tricuspid valves, the blood that is supposed to go into the lungs can leak back toward the upper chamber of the heart.”

Aster’s pulmonary valve was replaced via a minimally invasive procedure in which a catheter was threaded up through an artery in the groin to maneuver the valve into its proper place. Four weeks later, Makkar replaced Aster’s tricuspid valve using a similar procedure. 

“Patients with carcinoid heart disease are going through a lot because of the neuroendocrine tumor, and traditional valve replacement surgery puts them at higher risk for complications,” Makkar said. “We pioneered testing of transcatheter tricuspid valve replacement around five years ago, and this was the option we chose for Mr. Aster.”

To address the neuroendocrine tumors on Aster’s liver, Alexandra Gangi, MD, director of the Division of Surgical Oncology and co-director of the Neuroendocrine Tumor Multidisciplinary Clinc, chose another minimally invasive approach: tumor embolization.

“We accessed the tumors with a catheter, through the artery in the groin,” Gangi said. “This allows us to block off the blood flow to the specific arteries that feed the tumors. This starves the tumors, and results in shrinking and tumor control.”

So far, Aster has avoided surgery and chemotherapy because of the nature of neuroendocrine tumors and the expertise of his care team in sequencing his therapies based on his sypmtoms.

“I think the difference between metastatic neuroendocrine tumors and most other types of gastrointestinal cancers is that we’re playing the long game,” Gangi said. “We often do the least invasive thing and save more aggressive options for when we really need them. These tumors tend to recur, but prognosis is generally good and patients can live a long time with them.”

Additional treatment or surgical procedures might be on the horizon, but today, Aster is doing well.

“We feel very fortunate that we were able to identify this as early as we did and intervene in the way that we did,” Hendifar said. “A few years ago, even six months ago, Ken wouldn't have been a candidate for minimally invasive valve replacements. He would have required open-heart surgery, and I'm not sure if the outcome would have been as good.”

Aster describes his current health as “great.”

“I just started cycling again in January, 13 months after the second valve replacement,” Aster said. “Now I'm doing a 25-mile ride in the hills two or three times a week with no problem. My heart feels as normal as can be.”

Cedars-Sinai Health Sciences University is advancing groundbreaking research and educating future leaders in medicine, biomedical sciences and allied health sciences. Learn more about the university.

“These rising cancer rates are expected worldwide, and large-scale efforts need to be made to encourage lifestyle changes and develop screening programs to reduce them,” said Ju Dong Yang, MD, medical director of the Liver Cancer Program and professor of Medicine at Cedars-Sinai and co-corresponding author of the study.

Liver Cancer

Yang said that up to 70% of liver cancers could be prevented through lifestyle changes, because while hepatitis B and C were once leading causes of liver cancer in the U.S., they are being eclipsed.

“Metabolic dysfunction-associated steatotic liver disease, or MASLD, is emerging as a leading cause of liver cancer in Western countries, including the U.S.,” Yang said. “This is a buildup of fat in the liver that is associated with diabetes, obesity, and uncontrolled high cholesterol and high blood pressure.”

Taking steps to help control these risk factors is the best way to reduce risk, Yang said.

Yang said that only about 20% of people in the U.S. with cirrhosis from hepatitis and other chronic liver conditions receive liver cancer screening, which could help improve early detection and patient outcomes. Only about 30% of liver cancers are detected at an early enough stage for cure, Yang said.

Esophageal and Gastric Cancers

Lifestyle factors and limited screening also come into play in risk for esophageal and gastric cancers, said Alexandra Gangi, MD, director of the Division of Surgical Oncology in the Jim and Eleanor Randall Department of Surgery and director of the Gastrointestinal Tumor Program.

“These tumors cause few symptoms before the cancer has spread—and they tend to spread rapidly,” Gangi said. “To reduce risk, focus on preventable issues such as obesity, tobacco and alcohol use, and diet. And if you have other risk factors such as chronic acid reflux, gastritis or family history of these cancers, speak with your doctor about screening.”

Colorectal Cancer

Everyone should be screened for colorectal cancer by age 45—but those with a family history of the disease should begin sooner, said Alessio Pigazzi, MD, PhD, director of the Division of Colorectal Surgery. Results of that first screening, along with other risk factors, will help determine how often follow-up screenings are needed.

“Late diagnosis makes colorectal cancer highly lethal, while early diagnosis leads to very high cure rates,” Pigazzi said. “To reduce risk, everyone should eat a diet focused on whole foods that are high in fiber and low in sugars and animal fats, exercise regularly, keep up with screenings, and watch for colon cancer symptoms such as frequent rectal bleeding or unexplained changes in bowel habits.”

Pancreatic Cancer

Pancreatic cancer is another GI cancer usually diagnosed in advanced stages—with 50% of people diagnosed when the disease has already spread, said Arsen Osipov, MD, medical director of Pancreatic Cancer and Multidisciplinary Programs and Integration.

“There have been recent advances in early detection and treatment, including blood-based ‘liquid biopsy’ tests and structured screening programs for those with strong genetic risk,” Osipov said. “And multidisciplinary clinics, such as one pioneered at Cedars-Sinai, help accelerate diagnosis and treatment planning, and improve access to clinical trials and patient outcomes.”

To reduce pancreatic cancer risk, Osipov recommended paying attention to modifiable lifestyle factors similar to those for other GI cancers. He also suggested that people with chronic pancreatitis or family history of pancreatic cancer consider genetic counseling and participation in a high-risk surveillance program.

On the Horizon

Cedars-Sinai research is changing the landscape of GI cancer detection and treatment, said Katelyn Atkins, MD, PhD, interim chair and medical director of Radiation Oncology. Blood-based biomarkers such as circulating tumor DNA are improving early detection and allowing for tailored treatments and better detection of recurrence in colorectal cancer—and are now being leveraged for other GI sites, Atkins said.

“Improvements in radiation oncology techniques are allowing us to deliver the therapy more precisely and better protect nearby organs, making it safer to deliver more intense and effective treatment,” Atkins said. “There is also growing use of radiotherapy as an alternative to radical surgery in rectal, esophageal and gastroesophageal cancers.” 

Biomarker-driven treatment is also improving integration of radiotherapy into treatment regimens that also include surgery, chemotherapy and immunotherapy.  

“This shift toward more personalized, biology-driven treatment approaches helps us extend survival and preserve quality of life for patients with GI cancers,” said Robert Figlin, MD, interim director of Cedars-Sinai Cancer. “We will continue to work to improve treatment options and prevent cancer incidence in our community.”  

Cedars-Sinai Health Sciences University is advancing groundbreaking research and educating future leaders in medicine, biomedical sciences and allied health sciences. Learn more about the university.

Hyperthermic intraperitoneal chemotherapy (HIPEC) is a special type of treatment for patients with certain cancers that have spread to the abdominal cavity (peritoneum).

Unlike regular chemotherapy, which is often delivered through an intravenous (IV) drip and then circulates throughout the entire body, HIPEC is delivered during a surgical procedure. Also called “hot chemotherapy,” HIPEC involves delivering high doses of heated chemotherapy directly to the abdomen during the surgical procedure. The chemotherapy solution is heated to about 41 to 42 degrees Celsius (about 108 F).

“Bathing the peritoneal cavity with heated chemotherapeutic agents helps to target and kill cancer cells that might be too small to remove through surgery alone,” said Alexandra Gangi, MD, director of the Gastrointestinal Tumor and Cancer Regional Therapies programs at Cedars-Sinai Cancer and associate professor at the Jim and Eleanor Randall Department of Surgery.

Gangi recently spoke with the Cedars-Sinai Newsroom about how HIPEC works and who can benefit from the targeted surgical procedure.

What is HIPEC and who gets it?

HIPEC is a regional therapy used for patients with peritoneal disease, where cancer spreads to the peritoneum—the smooth membrane lining the abdominal wall and surrounding organs. When cancer from sites like the colon or the peritoneum itself spreads into the abdominal cavity, after removing visible tumors through surgery, heated chemotherapy is circulated in the abdomen to target and destroy remaining cancer cells at a cellular level.

Who is a good candidate for the HIPEC procedure?

Good candidates for HIPEC are patients with certain cancers that have spread within the peritoneum, a membrane that lines the inside of the abdomen and pelvis abdominal cavity. These patients have stage 4 cancer. This can include appendix cancer, colon and rectal cancer, mesothelioma, and primary peritoneal cancer. Some patients with gastric and ovarian cancer are eligible for the procedure, as well.

What does the HIPEC procedure involve?

The procedure typically begins with a cytoreductive surgery, where visible tumors are removed. Afterward, large catheters are placed into the abdominal cavity and the abdomen is closed. Heated chemotherapy is then circulated for 30 minutes to about an hour and a half at temperatures between 41 to 42 degrees Celsius—the solution is really hot.

Once that's done, we drain all the fluid from the belly and we open the patient back up. If there are any new surgical connections we may need to create, as in scenarios where we have removed some colon or intestine, we do that. We rinse the abdomen with saline, and then we close up the abdomen, and the patient goes to recovery.

How is HIPEC different from regular chemotherapy?

The procedure offers a more focused approach compared to regular chemotherapy, which circulates through your bloodstream throughout your body. Typically, patients won't see major fluctuations in their blood count, and they’re not necessarily considered immunocompromised. Unlike IV chemotherapy, which requires repeated sessions every few weeks, HIPEC is typically a one-time procedure, but can be repeated in some cases.

Systemic chemotherapy, which is given through an IV, relies on blood flow to reach tumors, but small tumors may not receive enough blood flow to be effectively treated this way. In HIPEC, chemotherapy is used after the visible disease is removed to kill any remaining cancer cells that exist at a cellular level since it only penetrates about three millimeters into the abdominal wall.

How does HIPEC improve survival rates?

For certain late-stage cancers, the survival rate is quite a bit lower, at around the 20% range. However, for certain mucinous tumors like pseudomyxoma peritonei, a type of appendix cancer, HIPEC can be life changing. The surgery significantly improves survival rate and can reduce the risk of recurrence. In some cases, survival without recurrence can be over 80%. It's a huge range but it is dependent on what type of cancer we're ultimately trying to treat and the patient's overall response to treatment.

What is the recovery process following the HIPEC procedure?

After surgery, some patients experience delayed return of bowel function, where the intestines take time to "wake up." This might lead to symptoms like reduced appetite, constipation or diarrhea, depending on what was removed during the surgery. Patients may also experience some discomfort or bloating. It’s best to avoid heavy lifting for about 6-8 weeks to allow proper healing. I typically advise patients that the first three months are kind of tough. And then you kind of start to feel more like your normal self. It’s a marathon, not a sprint.

Read more on the Cedars-Sinai Blog: The Need for Colorectal Cancer Screening in the Hispanic Community