Regional lymph node involvement and outcomes in appendiceal neuroendocrine tumors: a SEER database analysis

Abstract

Background: Appendiceal 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.

Methods: We used the SEER database to identify patients diagnosed with appendiceal neuroendocrine tumors.

Results: Of 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.

Conclusions: 10 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.

Keywords: appendix; neuroendocrine; right hemicolectomy; survival; tumor size.

Figure 1. Patients included in our study

WDNET, well-differentiated neuroendocrine tumor; PDNEC, poorly differentiated neuroendocrine carcinoma; MHT, mixed histology tumor.

Figure 2. Cancer-specific survival rates of all included patients with appendiceal neuroendocrine malignancies by histologic subtype

WDNET, well-differentiated neuroendocrine tumor; PDNEC, poorly differentiated neuroendocrine carcinoma; MHT, mixed histology tumor.

Figure 3. Comparison ofcancer-specificsurvival of patients with well-differentiated neuroendocrine tumors (WDNETs) and no nodal metastases (n=368), those with nodal involvement who underwent appendectomy or cecectomy (n=28), and those with nodal involvement who underwent right hemicolectomy/ or greater (n=90) (date of diagnosis 1988–2012)

Figure 4. Comparison of cancer specific survival of patients with mixed histology tumor (MHT) and no nodal metastases (n=651), those with nodal involvement who underwent appendectomy or cecectomy (n=25), and those with nodal involvement who underwent right hemicolectomy/ or greater (n=86) (date of diagnosis 1988–2012)