7.04 Goblet Cell Neuroendocrine Carcinomatosis treated with Cytoreductive Surgery and HIPEC

R. W. Randle1, K. F. Griffith1, K. R. Swett2, J. H. Stewart1, P. Shen1, E. A. Levine1, K. I. Votanopoulos1  1Wake Forest University Baptist Medical Center,Surgery,Winston-Salem, NORTH CAROLINA, USA 2Wake Forest University Baptist Medical Center,Biostatistics,Winston-Salem, NORTH CAROLINA, USA

Introduction:  Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) is an aggressive treatment for patients suffering with peritoneal carcinomatosis.  It is commonly applied to low-grade mucinous tumors of the appendix disseminated throughout the peritoneal cavity, yet some high volume centers have extended this therapy to carcinomatosis from a variety of more aggressive primary malignancies.  Therefore we decided to review our experience with CRS-HIPEC for patients with carcinomatosis from goblet cell neuroendocrine carcinomas.

Methods:  Patients with carcinomatosis and final pathology confirming goblet cell features were identified in a prospectively maintained database of 1069 CRS-HIPEC procedures performed between 1991 and 2013.  Patient demographics, disease characteristics, morbidity, mortality, and survival were reviewed.  

Results:  A total of 25 patients with goblet cell neuroendocrine carcinomatosis underwent CRS-HIPEC during the study period.  Tumors originated in the appendix in 23 (92%) patients and in the colon in 2 (8%).  Patients were generally young (mean age 53 years) and otherwise healthy (84% without comorbidities) with good performance status (92% ECOG 0 or 1).  The mean number of visceral resections was 3.6, and complete cytoreduction of all macroscopic disease was accomplished in 36% prior to HIPEC.  The 30-day major morbidity and mortality were 36% and 8%, respectively.  Median overall survival for all patients was 16.5 months.  In univariate analysis, significant predictors of decreased survival included worse performance status (hazard ration [HR] 2.2, 95% confidence interval [CI] 1.1–4.4, p=0.03) and nodal involvement (HR 9.6, 95% CI 1.2–73.8, p=0.03).  Despite similar volume of peritoneal disease, patients with negative nodes had better survival than those with positive nodes (median overall survival 32.7 months vs. 9.9 months), respectively (p=0.01).  While complete cytoreduction was associated with longer survival following CRS-HIPEC in all patients (R0/R1 median overall survival 28.5 months vs. R2 median overall survival 9.9 months, p=0.19) and in those with nodal disease (R0/R1 median overall survival 16.5 months vs. R2 median overall survival 8.5 months, p=0.07), neither observed difference reached statistical significance.

Conclusion:  CRS-HIPEC may improve survival in patients with node negative goblet cell neuroendocrine carcinomatosis when a complete cytoreduction is achieved.  Patients with disease not amenable to complete cytoreduction should not be offered CRS-HIPEC.