Primary Peritoneal Hepatoid Adenocarcinoma Patients Treated by Complete Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC)

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.

Automated summary

Hepatoid adenocarcinoma (HAC) is a rare disease that can develop from any thoraco-abdominal organ and exhibits features similar to hepatocellular carcinoma (HCC). Diagnosis and treatment of this disease pose significant challenges. So far, there have been 12 reported cases of primary peritoneal HAC with poor prognosis and diverse management. This article presents two additional cases managed in a multidisciplinary manner at an expert center, using a comprehensive approach including complete cytoreductive surgeries, hyperthermic intra-peritoneal chemotherapy (HIPEC), and limited systemic chemotherapy sequences. The use of choline PET-CT scan guided surgical exploration resulted in complete resection. The oncologic outcomes were promising, with one patient surviving 111 months after diagnosis and the other still alive at 43 months.