Abstract
Background/aimsLong-term outcomes are understudied in patients with well-differentiated appendiceal neuroendocrine neoplasms (WD-ANENs). We aimed to evaluate the validity of currently applied criteria for completion prophylactic right hemicolectomy (pRHC) and determine its association with patient outcomes, including health-related quality of life (HRQoL).MethodsEligible patients from five European referral centers were divided between those who underwent appendectomy alone and those who underwent completion pRHC. HRQoL EORTC-QLC-C30 questionnaires and cross-sectional imaging data were prospectively collected. Age- and sex-matched healthy controls were recruited for HRQoL analysis’ validation.ResultsWe included 166 patients (119 women [71.2%]: mean age at baseline: 31 ± 16 years). Mean follow-up was 50.9 ± 54 months. Most patients (152 [92%]) had tumors ≤20 mm in size. Fifty-eight patients (34.9%) underwent pRHC that in final analysis was regarded as an overtreatment in 38/58 (65.5%). In multivariable analysis, tumor size >20 mm was the only independent predictor for lymph node (LN) involvement (p = 0.002). No mortality was reported, whereas 2-, 5- and 10-year recurrence-free survival in patients subjected to postoperative cross-sectional imaging (n = 136) was 98.5%, 97.8%, and 97.8%, respectively. Global HRQoL was not significantly impaired in patients with WD-ANEN compared with age- and sex-matched healthy individuals (median scores 0.83[0.08−1] vs 0.83[0.4−1], respectively; p = 0.929). Among patients with WD-ANEN impaired social functioning (p = 0.016), diarrhea (p = 0.003) and financial difficulties (0.024) were more frequently reported in the pRHC group.ConclusionsWD-ANEN is a low-malignant neoplasm with unconfirmed associated mortality, low recurrence rate, and overall preserved HRQoL. pRHC comes at a price of excessive surgery, functional HRQoL issues, and diarrhea. The value per se of a prophylactic surgical approach to patients with WD-ANENs <20 mm is challenged.
Highlights
A worldwide increase in the incidence of neuroendocrine neoplasms (NENs) has recently been described, including appendiceal NENs (ANENs) [1]
ANEN standard treatment is surgery, which can be limited to the initial appendectomy or completed with a prophylactic right hemicolectomy, with the latter potentially been associated with adverse consequences in terms of long-term health-related quality of life (HRQoL)
We assessed in a multicenter setting, outcomes of patients with WD-ANENs after completion prophylactic right hemicolectomy (pRHC) compared with appendectomy alone
Summary
A worldwide increase in the incidence of neuroendocrine neoplasms (NENs) has recently been described, including appendiceal NENs (ANENs) [1]. Contemporary guidelines suggest a rather aggressive surgical approach with completion pRHC as a treatment option for patients diagnosed with tumors ≥20 mm or tumors 10–20 mm in the presence of certain histopathological parameters [5, 6]. A more conservative strategy with an active surveillance approach following appendectomy to WD-ANEN patients with histopathological risk parameters has only lately been suggested pending confirmation from prolonged follow-up data [2, 12, 13]
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