August 2020, Vol 1, No 1

Fred Koehl, who spoke at the 2020 Cholangiocarcinoma Foundation (CCF) Annual Conference, was a 62-year-old healthy man living in Atlanta, GA, who was “not ready to go” when he was diagnosed with extrahepatic cholangiocarcinoma (CCA) 7 years ago. Mr Koehl’s disease was diagnosed at an early stage (stage I), and after surgery to remove the tumor (including part of his pancreas), radiation, and 12 cycles of gemcitabine and cisplatin therapy, he was told he had “no evidence of disease.”

The 2020 Cholangiocarcinoma Foundation (CCF) Annual Conference was held virtually this year, as did all oncology conferences, because of the COVID-19 pandemic. More than 1400 people, including patients, caregivers, clinicians, researchers, and industry experts from 49 states and 42 countries, participated in the conference. This year, CCF invited patients and caregivers to share their experiences and concerns with fellow conference attendees.

Treatment with 6 months of adjuvant capecitabine chemotherapy is currently the standard of care in patients with resected extrahepatic cholangiocarcinoma (CCA). However, the benefit of treatment with adjuvant radiation therapy is not well-defined.

It is my great pleasure to launch the inaugural issue of CCA News, an important new publication highlighting the latest research and development in cholangiocarcinoma (CCA). I am proud that we now have a focused publication on biliary tract cancers and am humbled to serve as the first editor-in-chief.

Mutations in IDH1 are detected in approximately 13% of patients with intrahepatic cholangiocarcinoma (CCA). ClarIDHy was a global, phase 3, double-blind clinical trial in previously treated patients with advanced intrahepatic CCA with IDH1 mutation.

In patients with locally advanced or metastatic biliary tract cancer, treatment with second-line chemotherapy is challenging after disease progression from first-line gemcitabine plus cisplatin, although treatment with modified FOLFOX (mFOLFOX) has been proven to be superior to active symptom control in the ABC-06 trial. Irinotecan (Camptosar) is an active drug used in the treatment of various gastrointestinal cancers.

Varlitinib is a reversible small-molecule, pan human epidermal growth factor receptor (HER) inhibitor with low nanomolar potency against HER1 (EGFR), HER2, and HER4.

Futibatinib is a highly selective irreversible fibroblast growth factor receptor (FGFR)1-4 inhibitor, administered as a continuous once-daily oral regimen. The FOENIX-CCA2 phase 2 clinical trial was initiated after the results from a phase 1 dose-escalation/expansion study showed the tolerability and preliminary efficacy of futibatinib in patients with intrahepatic cholangiocarcinoma (CCA) and FGFR2 fusions.

Genetic alterations in the fibroblast growth factor receptor (FGFR) pathway are emerging as promising therapeutic targets in patients with cholangiocarcinoma (CCA). A retrospective chart review, led by Lipika Goyal, MD, MPhil, Medical Oncologist, Tucker Gosnell Center for Gastrointestinal Cancers, Massachusetts General Hospital, Boston, was performed in patients with CCA who had an FGFR alteration found by tumor molecular profiling as part of routine care.¹ Dr Goyal presented this study at the 2020 ASCO annual meeting.