Have you ever heard, “Only surgery or liver transplant can cure your Fibrolamellar Carcinoma?” Maybe you’ve been told “surgery must be done as soon as possible” because “there are no systemic therapies that are proven to work.” Worse still, have you been told “your Fibrolamellar Carcinoma s not resectable/transplantable, so you are not curable.” If you have, I am sorry. These statements are scary, out of date, and oversimplified. They do not reflect our current understanding and research on Fibrolamellar Carcinoma.
New and exciting systemic therapies developed by FibroFighters Foundation and others, as well as promising data from 240 FLC patients have revealed important insights into the role and timing of systemic therapy and surgery in treating Fibrolamellar Carcinoma.
Our research from the American Association of Cancer Research and the American Society of Clinical Research has made it clear that FLC has a subset (15%) of small (< 10cm) Stage I or II tumors, confined to the liver, with no signs of spread to lymph nodes or in the blood vessels, what we called “localized disease,” that can be cured with adequate surgery. 100% of such patients were alive 10 years from surgery with no other treatment. For practical purposes, this behaves like a different disease than higher stage FLC. For these other 85% of patients who have “regional,” or “metastatic” (stage III or IV) disease, immediate surgery after diagnosis relapsed on average 13 months and 4 months from surgery respectively, and did less well in terms of survival and relapse then those who got systemic therapy after surgery (adjuvant), before surgery (neoadjuvant) or both.
These results underline the idea that, with the exception of localized disease, FLC is a “systemic disease.” This means it is present in the body at a microscopic level that cannot be seen on MRI or CT. However, it can be detected with more sophisticated testing, such as ‘circulating tumor DNA,’ i.e, evidence of tumor cells in blood, such as “Signatera.” These microscopic cancer cells need to be eliminated as much as possible before the stress of major surgery to prevent the disease from recurring quickly.
Our data showed that many of the patients whose disease was thought to be “unresectable” can be converted to ‘resectable”! In fact, even the group of advanced stage IV patients that never had surgery, but did have newer systemic regimens, such as “GEMOX-LEN” or “Nivo-GEM-LEN” did just as well or better, then those who had surgery only. Both of these findings offer new hope to patients who are deemed ‘unresectable’ at diagnosis.
The obvious question is: “When should surgery be done if not initially?” Our data show that the best outcomes come after 6-12 months of pre-op multidisciplinary care with these newer systemic therapies, interventional radiology, and/or radiation. Additionally, delaying surgery until there is convincing evidence that the tumor is responding both on imaging and microscopically in the blood is crucial. This approach has proven prudent for patients; we have not seen new tumor growth prior to surgery using this approach.
In summary, if you have a low-stage, localized FLC, get surgery immediately, and you should be cured. If higher stage, multidisciplinary care and newer systemic therapies pre-op offer the best chances for relapse prevention and long-term control. Even if your are told your tumor is ‘unresectable / untransplantable’ do not lose hope! This same approach can convert you to a surgical/transplantable patient or give a much longer sustained control than previously thought.
To learn more, contact paulkentmd@fibrofighters.org