Clinical features and prognostic analysis of 14 cases of appendiceal mixed neuroendocrine-non-neuroendocrine tumors with peritoneal pseudomyxoma.

Abstract

Background: Appendiceal mixed neuroendocrine-non-neuroendocrine neoplasms (a-MiNENs) combined with peritoneal pseudomyxoma (PMP) represent an exceptionally rare and clinically challenging entity. This study aims to elucidate the clinical characteristics of this unique tumor subtype and identify critical prognostic factors, thereby providing insights into the management and outcomes of affected patients.

Methods: We conducted a retrospective analysis of 14 patients diagnosed with a-MiNEN and PMP at the Department of Myxoma, Aerospace Center Hospital, between January 2014 and September 2022. Data included demographics, symptoms, tumor grading, treatments, and outcomes.

Results: Pathological evaluation revealed a diverse spectrum of tumor grades and PMP subtypes: 5 cases were classified as G1 neuroendocrine tumors (NET), 4 as G2 NET, and 5 as G3 neuroendocrine carcinoma (NEC). Regarding PMP subtypes, 5 cases had disseminated peritoneal adenomucinosis (DPAM), 4 had peritoneal mucinous carcinomatosis (PMCA), and 5 had peritoneal mucinous carcinomatosis with signet ring cell carcinoma (PMCA-S). The median postoperative survival time was 28.55 months, with 1-year and 2-year survival rates of 80.0% and 66.7%, respectively. Patients aged < 60 years, male sex, absence of right hemicolectomy, postoperative peritoneal cancer index (PCI) ≥ 25, and perineural invasion demonstrated significantly lower 1-year and 2-year survival rates compared to their counterparts, with distinct separation observed in the Kaplan-Meier curves. These factors likely reflect biological differences in disease progression, inadequate surgical debulking, and aggressive tumor behavior.

Conclusions: a-MiNEN with PMP progresses rapidly. Young patients, male patients, those with absence of right hemicolectomy, postoperative PCI ≥ 25 and perineural invasion may face poorer outcomes. This study, one of the few to analyze this rare condition, underscores the need for targeted treatments and further validation in larger cohorts.