Abstract

BackgroundAppendiceal neuroendocrine neoplasms are most often diagnosed incidentally during appendectomy. The need for subsequent right hemicolectomy (RHC) is determined based on the risk of regional lymph node (LN) involvement. Tumor size has historically been used as an indicator of this risk, but controversy remains regarding its cut off. Furthermore, the impact of RHC on survival is unclear.MethodsWe used the SEER database to identify patients diagnosed with appendiceal neuroendocrine tumors.ResultsOf 1731 patients, 38.0% had well-differentiated neuroendocrine tumors (WDNETs), 60.8% had mixed histology tumors (MHTs), and 1.2% had poorly differentiated neuroendocrine carcinomas (PDNECs). In patients with WDNETs and MHTs who had adequate lymphadenectomy, higher rates of LN involvement were noted for tumors size 11–20 mm than ≤10 mm (56.8% vs. 11.6%, p <0.001; 32.9% vs. 10.4%, p=0.004, respectively). The type of surgery did not affect OS in cases with MHTs with LN involvement (HR 1.00; 95% CI, 0.53–1.89; p =0.99). Patients with regionally advanced WDNET showed excellent survival and only 3 patients (out of 118) died from cancer within 10 years.Conclusions10 mm appears to be a more appropriate cutoff than 20 mm for predicting LN metastasis in appendiceal NETs. Cases with WDNETs and nodal involvement demonstrate overall excellent prognosis irrespective of type of surgery (i.e. RHC may not improve outcome). In MHTs with LN metastases, survival is markedly worse in spite of RHC. The role of adjuvant therapy should be evaluated in this subset.

Highlights

  • Neuroendocrine neoplasms are the most common malignancies in the appendix

  • In patients with well-differentiated neuroendocrine tumors (WDNET) and mixed histology tumor (MHT) who had adequate lymphadenectomy, higher rates of lymph node (LN) involvement were noted for tumors size 11–20 mm than ≤10 mm (56.8% vs. 11.6%, p

  • In MHTs with LN metastases, survival is markedly worse in spite of right hemicolectomy (RHC)

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Summary

Introduction

Neuroendocrine neoplasms are the most common malignancies in the appendix. This heterogeneous group of tumors encompasses a wide spectrum of histologic types with different behaviors. The 2010 World Health Organization (WHO) Classification of Tumors of the Digestive System categorizes neuroendocrine neoplasms as well-differentiated neuroendocrine tumors (WDNET), poorly differentiated neuroendocrine carcinomas (PDNEC), and mixed adenoneuroendocrine carcinomas (MANEC) [1]. These subtypes are characterized by different morphological, clinical, and prognostic features. MANEC is defined as a tumor with both neuroendocrine and epithelial components in which each component represents at least 30% of neoplastic tissue.

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