Cholangioblastic variant of intrahepatic cholangiocarcinoma in a young adult with situs inversus totalis.

The cholangioblastic variant of intrahepatic cholangiocarcinoma is a rare subset of cholangiocarcinoma. Only a few cases have been reported, typically in young people with large tumours originally diagnosed as neuroendocrine tumours. Here we report the first case of this rare malignancy in a young female with situs inversus totalis. Our patient initially presented with early satiety and worsening left-sided abdominal pain. Laboratory values, imaging and biopsies diagnosed a neuroendocrine tumour of primary hepatic origin. However, tumour staging with DOTATATE-PET and FDG PET/CT suggested incongruency with the biopsied diagnosis and pathology after upfront surgical resection revealed the cholangioblastic variant of intrahepatic cholangiocarcinoma.