Abstract

BackgroundThe increasing incidence and heterogeneous behavior of intestinal neuroendocrine tumors (iNETs) pose a clinicopathological challenge. Our goal was to decribe the prognostic value of the new WHO 2010 grading and the AJCC/UICC TNM staging systems for iNETs. Moreover, outcomes of patients treated with somatostatin analogs were assessed.MethodsWe collected epidemiological and clinicopathological data from 93 patients with histologically proven iNETs including progression and survival outcomes. The WHO 2010 grading and the AJCC/UICC TNM staging systems were applied for all cases. RECIST criteria were used to define progression. Kaplan-Meier analyses for progression free survival (PFS) and overall survival (OS) were performed.ResultsMean follow-up was 58.6 months (4–213 months). WHO 2010 grading yielded PFS and disease-specific OS of 125.0 and 165.8 months for grade 1 (G1), 100.0 and 144.2 months for G2 and 15.0 and 15.8 months for G3 tumors (p = 0.004 and p = 0.001). Using AJCC staging, patients with stage I and II tumors had no progression and no deaths. Stage III and IV patients demonstrated PFS of 138.4 and 84.7 months (p = 0.003) and disease-specific OS of 210.0 and 112.8 months (p = 0.017). AJCC staging also provided informative PFS (91.2 vs. 50.0 months, p = 0.004) and OS (112.3 vs. 80.0 months, p = 0.005) measures with somatostatin analog use in stage IV patients.ConclusionOur findings underscore the complementarity of WHO 2010 and AJCC classifications in providing better estimates of iNETS disease outcomes and extend the evidence for somatostatin analog benefit in patients with metastatic disease.

Highlights

  • Neuroendocrine tumors (NETs), known as ‘‘carcinoid’’ tumors, are slowly growing neoplasms that were previously considered to be largely benign, retrospective data suggested that all NETs have malignant potential [1]

  • Preoperative imaging studies (CT and/or MRI) revealed that the most common initial radiographic findings were the presence of a small bowel lesion in 33.3% and a mesenteric mass associated with a bowel lesion in 22.6%

  • Distant metastatic disease was present in 43.0% (n = 40) of patients and the liver, alone or in combination with other sites, was the most frequently involved organ

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Summary

Introduction

Neuroendocrine tumors (NETs), known as ‘‘carcinoid’’ tumors, are slowly growing neoplasms that were previously considered to be largely benign, retrospective data suggested that all NETs have malignant potential [1]. Intestinal NETs (iNETs) arising in the small bowel, appendix and large bowel are typically discovered incidentally during surgery or imaging for unexplained symptoms [6,7,8]. When symptoms occur they tend to be nonspecific, often vague abdominal pain; carcinoid syndrome appears in just 20–30% of the patients, who almost invariably have metastases [9]. Surgery remains the only potentially curative therapy for patients with localized disease [5,9]. The increasing incidence and heterogeneous behavior of intestinal neuroendocrine tumors (iNETs) pose a clinicopathological challenge. Outcomes of patients treated with somatostatin analogs were assessed

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