Abstract
BackgroundDue to its rarity and high heterogeneity, neither established guidelines nor prospective data are currently available for using chemotherapy in the treatment of appendiceal cancer. This study was to determine the use of chemotherapy and its potential associations with survival in patients with different histological types of the cancer.MethodsPatients with histologically different appendiceal cancers diagnosed during 1998–2016 were selected from the Surveillance, Epidemiology, and End Results (SEER) database. The role and effect of chemotherapy were examined in the treatment of the disease. The Kaplan-Meier method was applied to construct survival curves and significance was examined by Log-rank test. Cox proportional hazard models were used to analyze the impact of chemotherapy and other variables on survival in these patients.ResultsA total of 8733 appendiceal cancer patients were identified from the database. Chemotherapy was administrated at highly variable rates in different histological types of appendiceal cancer. As high as 64.0% signet ring cell carcinoma (SRCC), 46.4% of mucinous adenocarcinomas (MAC), 40.6% of non-mucinous adenocarcinoma (NMAC) and 43.9% of mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) were treated with chemotherapy, whereas only 14.7% of goblet cell carcinoma (GCC), 5% neuroendocrine tumors (NETs) and 1.6% carcinomas (NEC) received chemotherapy. In all patients combined, chemotherapy significantly improved overall survival during the entire study period and cancer-specific survival was improved during in cases from 2012–2016. Further multivariate analysis showed that both cancer-specific and overall survival was significantly improved with chemotherapy in patients with MAC, NMAC and SRCC, but not for patients with GCC, MiNENs, NETs and NECs. Number (> 12) of lymph node sampled was associated with survival of patients with most histological types of cancer under study. Other prognostic factors related to individual histological types were identified.ConclusionsChemotherapy is administrated at highly variable rates in different histological types of appendiceal cancer. Efficacy of chemotherapy in the treatment of these cancers has been improved in recent years and is significantly associated with better survival for patients with NMAC, MAC, and SRCC. Adequate lymph node sampling may result in a survival benefit for most of these patients.
Highlights
Appendiceal cancer is a rare and highly heterogeneous malignancy and its incidence is on the rise [1]
This cancer includes a wide spectrum of histological types including: mucinous adenocarcinoma (MAC), non-mucinous adenocarcinoma (NMAC), signet-ring cell adenocarcinomas (SRCC), mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs), goblet cell carcinoid (GCC) neuroendocrine tumors (NETs), neuroendocrine carcinomas (NECs), and others [2]
CI confidence interval; HR hazard ratio; SRCC signet ring cell carcinoma; MAC mucinous adenocarcinomas; NMAC non-mucinous adenocarcinoma; MiNENs mixed neuroendocrine non-neuroendocrine neoplasms; GCC goblet cell carcinoma; NETs neuroendocrine tumors; NECs, neuroendocrine carcinomas patients died of the disease or from all causes during the study period (Table 2)
Summary
Appendiceal cancer is a rare and highly heterogeneous malignancy and its incidence is on the rise [1] This cancer includes a wide spectrum of histological types including: mucinous adenocarcinoma (MAC), non-mucinous adenocarcinoma (NMAC), signet-ring cell adenocarcinomas (SRCC), mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs), goblet cell carcinoid (GCC) neuroendocrine tumors (NETs), neuroendocrine carcinomas (NECs), and others [2]. These histological types display dramatically different biological phenotypes and indicate different prognoses [2, 3]. The rarity and high heterogeneity make it difficult to examine the effect of chemotherapy in treatment of appendiceal cancer in systematic studies [5, 6]. This study was to determine the use of chemotherapy and its potential associations with survival in patients with different histological types of the cancer
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