Primary Cardiac Tumors: A Clarion Call for Cardio-Oncology

Patrice Wendling

May 22, 2020

Life expectancy remains strikingly poor for patients with primary cardiac malignancies, a large national analysis shows, reinforcing the need for a multidisciplinary cardio-oncology team to evaluate and treat these tumors from the start.

Among 747 patients with a primary malignant cardiac tumor in the National Cancer Database, survival was 81.2% at 30 days and fell to 45.3% at 1 year and just 11.5% at 5 years.

Cardiac hemangiosarcomas were the most lethal, with 5-year survival rates of 6.5% vs 15.1% for all other primary cardiac tumors (< .001).

"These tumors are rare but important," and "it's disappointing that results are so dismal," study author Ibrahim Sultan, MD, University of Pittsburgh, told theheart.org | Medscape Cardiology.

Moreover, outcomes have not improved dramatically over the last 10 years, he noted. For example, 1-, 3-, and 5-year survival rates were 50%, 24%, and 19%, respectively, from 2000 to 2011 in a 551-patient SEER registry analysis.

Part of the challenge is the rarity of primary cardiac malignancies. Of the 100,317 cardiac tumors identified between 2004 to 2016 in the present series, 826 (0.8%) were primary cardiac malignant tumors. The remainder were tumors metastatic to the heart from another source.

Over the study period, "there was a slow trend for increased diagnosis, from about 7.6% to 10%, but to land in the right team is critical," Sultan said.

This disease typically strikes young people during their working, productive years and has no known causes or associations, according to Michael J. Reardon, MD, who is a world expert on primary cardiac malignancies and a cardiac surgeon at Houston Methodist Hospital.

"The other thing that's heartrending about this is that most physicians have never seen a primary cardiac sarcoma or they've seen one or two in their life. And a lot of these patients tell me they see their doctor and they're just told there's nothing we can do, just go home and die," he told theheart.org | Medscape Cardiology.

"So getting to specialized centers where cardio-oncology is practiced, where there's specialized teams, is essential," Reardon said. "It's still a devastating disease but there is treatment and there are ways to give these people hope and help them."

For the 747 patients (median age, 53 years) with follow-up data, treatment centers were academic (50.9%), community cancer program (35.8%), or an integrated network (13.3%).

Surgery was part of the treatment paradigm for 442 (72.3%) patients, including 187 with surgery alone, 168 with surgery plus chemotherapy, 37 with surgery plus radiation, and 50 with surgery and combined chemotherapy and radiation.

Five-year survival estimates were higher in patients who underwent surgery (P < .0001) and in those with stage III disease who received postoperative chemotherapy (P = .0095).

"However, significant selection bias was likely the case in these patients," the authors reported in the Journal of the American College of Cardiology.

On logistic regression, independent predictors of mortality were advanced age, higher comorbidity index, hemangiosarcoma histology, and stage IV disease.

To improve treatment success rates in these patients, Reardon and Chiara Lestuzzi, MD, National Cancer Institute of Aviano, Italy, write in an accompanying editorial that what is needed is to increase the rate of patients undergoing surgery and to obtain a R0 resection.

Of interest, only 26% of tumors in the series were resected with uninvolved margins. Patients with positive margins has significantly reduced long-term survival compared with those with negative margins.

In case of incomplete surgery, "a multimodal approach is necessary," the editorialists say.

For large tumors, neoadjuvant therapy may reduce tumor burden, improving resectability for large tumors. This approach doubled the negative margin resection rate and survival in a prospective study of right-sided tumors. Neoadjuvant radiotherapy may also be used in selected cases, without significant cardiac adverse effects, Reardon and Lestuzzi noted.

Also, adjuvant chemotherapy and/or radiotherapy after resection appears to reduce the risk of local recurrence, mostly when the margins are infiltrated.

"Right now our primary drugs are Adriamycin and ifosfamide," Reardon told Medscape. "Our second-line drugs are generally gemcitabine and Taxol and we often use those for maintenance because they're easier.

"The newer drugs such as immunotherapy are things that, of course, we remain quite interested in because we need better biologic treatment," he said. "We're currently taking all of our tumors and doing RNA and DNA analysis looking at the basic biology and trying to come up with better biologic approaches for this disease."

That level of sophistication is not the norm. Most cardiac tumors are first detected by general cardiologists, the editorialists note. "The most frequent approach is to send the patient to the cardiac surgeon; the diagnosis is made by the pathologists after surgery (which is often incomplete, as seen before), and only then the patient is referred to an oncologist."

The new series, they say, reinforces that the best option is a multidisciplinary team that includes experienced cardio-oncologists, cardiac surgeons, imaging specialists, and sarcoma oncologists.

"When it's impossible to find all specialists in hospitals close enough to create a cardiac tumor team, the role of cardio-oncologists is of utmost importance," Reardon and Lestuzzi write.

Cardio-oncologists may facilitate communication between specialists and play an essential role in planning radiotherapy on the heart and in the follow-up for both local relapse and cardiac adverse effects of antineoplastic therapies.

Sultan has received institutional research support from Atricure and Medtronic (no personal renumeration). Reardon and Lestuzzi report having no relevant c onflicts of interest.

J Am Coll Cardiol. 2020;75:2338-2347 and 2348-2351. Abstract, Editorial

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