Peritoneal carcinomatosis from small intestinal neuroendocrine tumors: Clinical course and genetic profiling

Surgery. 2014 Dec;156(6):1512-21; discussion 1521-2. doi: 10.1016/j.surg.2014.08.090. Epub 2014 Nov 11.

Abstract

Background: One-fifth of all patients with small-intestinal neuroendocrine tumors (SI-NETs) present with or develop peritoneal carcinomatosis (PC). Our aim was to determine the prognosis and genetic profiles of tumors in patients with PC compared with tumors in patients without PC.

Methods: We included SI-NET patients (cases with PC, n = 73, and controls without PC, n = 468) who underwent operation between 1985 and 2012. The Lyon prognostic index was used to correlate the amount of PC to survival. DNA samples from patients with (n = 8) and without (n = 7) PC were analyzed with a single-nucleotide polymorphism array (HumanOmni2.5 BeadChip, Illumina) to investigate genetic disparities between groups.

Results: Patients with PC had poorer survival (median 5.1 years) than controls (11.1 years). An advanced postoperative Lyon prognostic index was a negative prognostic marker for survival by multivariable analysis (P = .042). Patients with and without PC clustered differently based on loss of heterozygosity and copy number variation data from single-nucleotide polymorphism array of the primary tumors (P = .042).

Conclusion: SI-NET patients with PC have poor survival, which diminishes with increasing PC load after surgery. Clustering based on copy number variation and loss of heterozygosity data suggests different genotypes in primary tumors comparing patients with and without PC.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Carcinoma / genetics
  • Carcinoma / mortality
  • Carcinoma / secondary*
  • Cohort Studies
  • DNA Copy Number Variations
  • Disease-Free Survival
  • Female
  • Genetic Variation*
  • Humans
  • Intestinal Neoplasms / genetics*
  • Intestinal Neoplasms / mortality
  • Intestinal Neoplasms / pathology
  • Intestine, Small / pathology
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Neuroendocrine Tumors / genetics*
  • Neuroendocrine Tumors / mortality
  • Neuroendocrine Tumors / secondary
  • Peritoneal Neoplasms / genetics
  • Peritoneal Neoplasms / mortality
  • Peritoneal Neoplasms / secondary*
  • Prognosis
  • Reference Values
  • Retrospective Studies
  • Risk Assessment
  • Survival Analysis