Study shows how to achieve optimal treatment and improve outcomes for rare cancer patients

Newswise — (Baltimore, MD) -- The Institute for Cancer Care at Mercy Medical Center is pleased to announce the publication of an in-depth analysis on appendiceal goblet cell carcinomas managed with CRS/HIPEC (Cytoreductive Surgery, Hyperthermic Intraperitoneal Chemotherapy) in the Annals of Surgical Oncology. These very rare tumors are often misclassified and mismanaged. Despite their aggressive nature, a 5-year overall survival of 67% can be achieved with CRS/HIPEC in select patients. This important work will improve timely referrals to specialized CRS/HIPEC centers to provide optimal treatment and achieve the best outcomes for these patients.

Mercy surgical oncologists Drs. Armando Sardi and Vadim Gushchin were among the paper's authors.

Background

Appendiceal goblet cell adenocarcinoma (GCA) is often misclassified and mistreated due to mixed histologic features. In general, cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) is standard of care for peritoneal carcinomatosis (PC) from mucinous appendiceal tumors; however, in PC from GCA, data are limited and the role of CRS/HIPEC is controversial. We report outcomes in PC from appendiceal GCA treated with CRS/HIPEC.

Patients and Methods

A prospective institutional database of 391 CRS/HIPEC patients with appendiceal carcinomatosis from 1998 to 2018 was reviewed. Twenty-seven patients with GCA were identified. Perioperative variables were described. Survival was estimated using the Kaplan–Meier method.

Results

GCA occurred in 7% (27/391) of appendiceal CRS/HIPEC patients. Seven (26%) cases were aborted. Two patients underwent a second CRS/HIPEC for peritoneal recurrence. Median age at diagnosis was 53 years (range 39–72 years), and 12 (60%) were female. All underwent previous surgery. Seven (35%) had prior chemotherapy and received a median of 5 cycles (range 3–8). Median PCI was 6 (range 1–39). Complete cytoreduction was achieved in 95% (19/20). Grade III complications occurred in three (15%) patients, and no perioperative deaths occurred. Median follow-up was 97 months. Overall survival at 1, 3 and 5 years was 100%, 74% and 67%, respectively. Progression-free survival at 1, 3, and 5 years was 94%, 67% and 59%, respectively.

Conclusion

CRS/HIPEC should be considered as the main treatment option for patients with PC from appendiceal GCA. When performed at a CRS/HIPEC specialty center, 5-year OS of 67% can be achieved.