Hepatic and peri-hepatic cytoreductive surgery in low-grade appendiceal mucinous neoplasms

Abstract

Background

Low-grade appendiceal mucinous neoplasm (LAMN) is a rare tumor that carries a risk of rupture causing pseudomyxoma peritonei (PMP). With PMP, intraperitoneal surfaces and organs become involved, including the liver capsule, porta hepatis, and peri-hepatic regions. Extensive porta hepatis and peri-hepatic involvement in PMP is often cited as a contraindication for cytoreduction. We present a retrospective review of successful cytoreductive surgery (CRS) in patients with LAMN involvement of the porta hepatis and peri-hepatic tissues.

Methods

A retrospective review identified patients over a 3-year period with a diagnosis of LAMN with porta hepatis and peri-hepatic involvement. Peri-operative records were reviewed for all patients who met study criteria.

Results

We identified 41 patients with LAMN and porta hepatis and/or peri-hepatic involvement who underwent CRS with successful debulking of these regions. Non-anatomic hepatic parenchymal resection was required in 19 patients. Median peritoneal carcinoma index was 28, and median length of stay was 13.5 days. Clavien-Dindo Grade ≥ 3 complications were seen following 36 % of cases, with no liver-specific morbidity and no peri-operative mortalities.

Discussion

Peri-hepatic or portal involvement by PMP can be safely addressed during CRS and should not preclude attempts at complete cytoreduction in appropriate patients who may achieve long-term disease control.