Peritoneal Carcinomatosis in Gastro-Entero-Pancreatic Neuroendocrine Neoplasms: Clinical Impact and Effectiveness of the Available Therapeutic Options

Neuroendocrinology. 2020;110(6):517-524. doi: 10.1159/000503144. Epub 2019 Sep 5.

Abstract

Background: Peritoneal carcinomatosis (PC) can affect the quality of life of patients with gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NENs). Peritoneal disease control by medical therapies in these patients has been poorly investigated Objectives: To describe, in a consecutive series of GEP-NENs, the clinical impact of PC and to report the effectiveness of available treatments in PC control.

Methods: A retrospective, monocenter analysis was performed of 135 GEP-NENs (1993-2016) with at least a 12-month follow-up. Peritoneal disease progression was defined as detection of a significant increase in size or appearance of new implants by imaging.

Results: A total of 62.9% of cases had diffuse PC (involving at least 2 abdominal quadrants). According to WHO 2017 classification, cases were 42.3% neuroendocrine tumors NET-G1, 45.5% NET-G2, 6.5% NET-G3, 4.9% neuroendocrine carcinomas NEC-G3, and 0.8% mixed neuroendocrine-nonneuroendocrine neoplasms. Bowel obstruction occurred in 30 (22.2%) patients mainly depending on size of peritoneal implants (HR: 1.10; 95% CI: 1.02-1.20; p = 0.01). Patients with diffuse PC treated with peptide receptor radionuclide therapy (PRRT) showed peritoneal progression in 37.5% of cases, and bowel obstruction or ascites in 28.1%. Better peritoneal disease control was observed in cases receiving somatostatin analogs at first-line therapy, probably due to a less aggressive disease behavior for these patients.

Conclusions: Bowel obstruction is not uncommon in GEP-NENs with PC. PRRT should be adopted with caution in GEP-NENs with diffuse PC, but larger series are needed to confirm these data.

Keywords: Bowel obstruction; Disease control; Neuroendocrine neoplasms; Peptide receptor radionuclide therapy; Peritoneal carcinomatosis.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Digestive System Neoplasms* / complications
  • Digestive System Neoplasms* / drug therapy
  • Digestive System Neoplasms* / pathology
  • Digestive System Neoplasms* / radiotherapy
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Humans
  • Intestinal Obstruction* / etiology
  • Intestinal Obstruction* / pathology
  • Intestinal Obstruction* / therapy
  • Male
  • Middle Aged
  • Neuroendocrine Tumors* / complications
  • Neuroendocrine Tumors* / drug therapy
  • Neuroendocrine Tumors* / pathology
  • Neuroendocrine Tumors* / radiotherapy
  • Outcome Assessment, Health Care*
  • Peritoneal Neoplasms* / complications
  • Peritoneal Neoplasms* / drug therapy
  • Peritoneal Neoplasms* / pathology
  • Peritoneal Neoplasms* / radiotherapy
  • Radioisotopes / therapeutic use*
  • Receptors, Peptide*
  • Retrospective Studies
  • Somatostatin / analysis
  • Somatostatin / therapeutic use*

Substances

  • Radioisotopes
  • Receptors, Peptide
  • Somatostatin