Primary Peritoneal Hepatoid Adenocarcinoma Patients Treated by Complete Cytoreductive Surgery (CRS) and Hyperthermic...

Hepatoid adenocarcinoma (HAC) is an extremely rare disease, which could develop from any thoraco-abdominal organ and which exhibits features mimicking hepatocellular carcinoma (HCC). Its diagnosis is thus highly challenging, so is the treatment of that disease. So far, 12 cases have been reported in the literature as issued from the peritoneum. These primary peritoneal HAC were associated with a dismal prognosis and heterogenous management. Two additional cases were described here, managed in a multidisciplinary way as rare peritoneal surface malignancies in an expert center, following the strategy based on a comprehensive tumor burden extension assessment and a radical approach combining iterative complete cytoreductive surgeries followed by hyperthermic intra-peritoneal chemotherapy (HIPEC) and limited systemic chemotherapy sequences. In particular, the choline PET-CT scan guided surgical exploration to reach a complete resection. The oncologic outcomes were promising with a first patient dying 111 months after the diagnosis and a second patient still alive at 43 months.