• CRS/HIPEC demonstrates promising survival in appendix goblet cell carcinoma patients. • Patients with and without high peritoneal burden have similar survival outcomes. • A CC-0 cytoreduction is a critical independent prognostic factor. • High CC-0 rates can be achieved at high-volume CRS/HIPEC centers. • Many goblet cell carcinoma patients are self-referred for CRS/HIPEC. CRS/HIPEC demonstrates promising survival in appendix goblet cell carcinoma patients. Patients with and without high peritoneal burden have similar survival outcomes. A CC-0 cytoreduction is a critical independent prognostic factor. High CC-0 rates can be achieved at high-volume CRS/HIPEC centers. Many goblet cell carcinoma patients are self-referred for CRS/HIPEC. Goblet cell adenocarcinoma (GCA) is a rare, aggressive type of appendix cancer (AC) often presenting with peritoneal disease (PD). While cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is standard for other AC subtypes, its role in GCA, particularly with high peritoneal cancer index (PCI), remains controversial. We conducted a retrospective analysis of two prospective databases, including patients with GCA undergoing first CRS/HIPEC. We described clinical characteristics and compared outcomes between PD patients with high (≥20) and low PCI. Progression-free (PFS) and overall survival (OS) were analyzed using the Kaplan Meier method. Prognostic factors were evaluated via multivariable Cox regression. Of 183 patients, 30 (16.4%) were non-metastatic (NM) and 153 (83.6%) had PD. In PD, median PCI was 20 (interquartile range IQR: 13-28). Complete cytoreduction (CC-0) was achieved in 61.4% of PD patients. Major complications (Clavien Dindo III-IV) occurred in 10% of NM and 33% of PD, with higher rates in PCI≥20 (p=0.011). After median follow-up of 59 months, no NM recurred or died and 62.7% of PD patients recurred (77.1% intraperitoneal). Five-year PFS and OS for PD were 11.2% and 29.5%, respectively. Incomplete cytoreduction independently predicted worse PFS (hazard ratio HR 1.7, p=0.009) and OS (HR 2.3, p<0.001), while PCI≥20 did not. Five-year OS was similar in patients with high and low PCI (28.3% vs 32.0%, p=0.551). Complete CRS/HIPEC offers promising survival in GCA with PD. NM patients at risk of PD show excellent survival. Further studies are warranted to optimize selection criteria.
Scardino et al. (Sun,) studied this question.