Postpartum related intrahepatic cholangiocarcinoma with fusion and severe hyperbilirubinemia with response to FGFR inhibitor pemigatinib: case report and review.

Background: Cholangiocarcinoma during postpartum or pregnancy is a rare presentation. There are limited cases reported in the literature. Diagnosis can be delayed as presenting signs and symptoms may be attributed to pregnancy or postpartum state.

Case Description: We present the case of a 33-year-old postpartum woman with intrahepatic cholangiocarcinoma with severe hyperbilirubinemia who was found to have fibroblast growth factor receptor 2 ()-adenosylhomocysteinase like 1 () fusion on next-generation sequencing (NGS). She initially was treated with two doses of gemcitabine and cisplatin with increasing hyperbilirubinemia requiring hold of further chemotherapy. NGS showed fusion, and she was started on the FGFR inhibitor pemigatinib, with dramatically decreasing bilirubin within 10 days. She eventually normalized her bilirubin values and had partial response on follow-up imaging.

Conclusions: This is the first report, to our knowledge of response to an FGFR inhibitor in the postpartum setting, as well to show response in the setting of life-threatening hyperbilirubinemia. Our patient did not tolerate standard chemotherapy, likely due to liver dysfunction, but responded to pemigatinib, suggesting that the liver dysfunction was driven by her disease. This case underscores the need to include NGS as part of initial workup to identify important therapeutic targets and increase available lines of therapy, including those patients who are postpartum or pregnant.