Clinical management of atypical carcinoid and large-cell neuroendocrine carcinoma: a multicentre study on behalf of the European Association of Thoracic Surgeons (ESTS) Neuroendocrine Tumours of the Lung Working Group†

Eur J Cardiothorac Surg. 2015 Jul;48(1):55-64. doi: 10.1093/ejcts/ezu404. Epub 2014 Nov 18.

Abstract

Objectives: In 2012, the European Society of Thoracic Surgeons (ESTS) created the Lung Neuroendocrine Tumors Working Group (NETs-WG) with the aim to develop scientific knowledge on clinical management of such rare neoplasms. This paper outlines the outcome and prognostic factors of two aggressive NETs: atypical carcinoids (ACs) and large-cell neuroendocrine carcinomas (LCNCs).

Methods: Using the ESTS NETs-WG database, we retrospectively collected data on 261 patients in seven institutions in Europe, between 1994 and 2011. We used a Cox regression model to evaluate variables affecting patient survival and disease-free survival. Univariate and multivariate analysis were also carried out.

Results: Five-year overall survival rates for ACs and LCNCs were 77 vs 28% (P < 0.001), respectively. We found that for ACs, age (P < 0.001), tumour size (P = 0.015) and sub-lobar surgical resection (P = 0.005) were independent negative prognostic factors; for LCNCs, only pTNM stage III tumours (P = 0.016) negatively affected outcome in the multivariate analysis. Local recurrences and distant metastases developed in 93 patients and were statistically more frequent in LCNCs (P = 0.02).

Conclusions: The biological aggressiveness of ACs and LCNCs has been demonstrated with this study. Our aim is to confirm these results with enhanced data collection through the ESTS NETs database.

Keywords: Atypical carcinoid; Large-cell neuroendocrine carcinoma; Lung; Metastasis; Neuroendocrine tumour; Recurrence; Surgery; Survival.

Publication types

  • Multicenter Study

MeSH terms

  • Aged
  • Carcinoid Tumor / diagnosis
  • Carcinoid Tumor / mortality
  • Carcinoid Tumor / pathology
  • Carcinoid Tumor / surgery*
  • Carcinoid Tumor / therapy
  • Carcinoma, Large Cell / diagnosis
  • Carcinoma, Large Cell / mortality
  • Carcinoma, Large Cell / pathology
  • Carcinoma, Large Cell / surgery*
  • Carcinoma, Large Cell / therapy
  • Carcinoma, Neuroendocrine / diagnosis
  • Carcinoma, Neuroendocrine / mortality
  • Carcinoma, Neuroendocrine / pathology
  • Carcinoma, Neuroendocrine / surgery*
  • Carcinoma, Neuroendocrine / therapy
  • Combined Modality Therapy
  • Female
  • Humans
  • Lung / pathology
  • Lung Neoplasms / diagnosis
  • Lung Neoplasms / mortality
  • Lung Neoplasms / pathology
  • Lung Neoplasms / surgery*
  • Lung Neoplasms / therapy
  • Male
  • Middle Aged
  • Prognosis
  • Proportional Hazards Models
  • Retrospective Studies
  • Survival Analysis