Adenocarcinoma Ex goblet cell: A retrospective experience.

Abstract

847 Background: Adenocarcinoma ex goblet cell tumors (AGCC) represent rare appendiceal neoplasms with neuroendocrine and adenocarcinoma features. They represent the most aggressive subset of goblet cell carcinoids (Group B and C) and are often categorized as chemotherapy unresponsive [Am J SurgPathol.2008;32(10):1429-43]. Despite this, most patients with AGCC are treated with colon cancer chemotherapy regimens. We explored outcomes in patients treated at our institution with these regimens. Methods: We performed a single center retrospective analysis of 22 biopsy-proven AGCC patients (13 men, 9 women, median age at diagnosis 54.5) at Vanderbilt who had undergone treatment for local or metastatic disease. PFS and OS were determined for each patient and stratified by features listed on Table 1. Patients who were lost to follow up were censored based on their last recorded clinic date. Results: 16 patients received chemotherapy in the metastatic setting and all 16 had peritoneal involvement; of these patients 4 underwent HIPEC. Median PFS and OS in HIPEC treated patients was 17 months and 33 months respectively. 8 patients received adjuvant chemotherapy with FOLFOX; 6 in the local setting and 2 in the metastatic setting. Patients with metastatic AGCC had a median PFS of 21.5 months and a median OS of 32.9 months. Patients with local disease had a median PFS of 32.8 months and an OS that was not reached (median duration of follow up 12.4 months). Conclusions: AGCC have a predilection for peritoneal spread. Patients who received either adjuvant FOLFOX post definitive surgery or FOLFOX/FOLFIRI in the metastatic setting post optimal cytoreduction had prolonged periods of PFS and OS. [Table: see text]