Cancer Res Treat.  2016 Apr;48(2):596-604. 10.4143/crt.2015.029.

Appendiceal Neuroendocrine, Goblet and Signet-Ring Cell Tumors: A Spectrum of Diseases with Different Patterns of Presentation and Outcome

• Shaib W
• Krishna K
• Kim S
• Goodman M
• Rock J
• Chen Z
• Brutcher E
• Staley CI
• Maithel SK
• Abdel-Missih S
• El-Rayes B
• Bekaii-Saab T

• KMID: 2454337
• DOI: http://doi.org/10.4143/crt.2015.029

Abstract

PURPOSE
Appendiceal tumors are a heterogeneous group of diseases that include typical neuroendocrine tumors (TNET), goblet cell carcinoids (GCC), and atypical GCC. Atypical GCC are classified into signet-ring cell cancers (SRCC) and poorly differentiated appendiceal adenocarcinoids. The prognosis and management of these diseases is unclear because there are no prospective studies. The aim of this study is to assess the characteristics and outcome of appendiceal TNET, GCC, and SRCC patients.
MATERIALS AND METHODS
Appendiceal TNET, GCC, and SRCC patients diagnosed between 1973 and 2011 were identified in the Surveillance Epidemiology and End Results (SEER) database. Demographics, type of surgery, and clinicopathologic characteristics were collected. Survival functions were estimated by the Kaplan-Meier method, and log-rank test was used to assess the difference in overall survival (OS) among the three histologies.
RESULTS
The SEER database yielded 1,021 TNET patients, 1,582 with GCC, and 534 SRCC patients. TNET presented at a younger age (p < 0.001). Patients with SRCC presented with advanced stage disease (p < 0.001). The median OS (mOS) for GCC and TNET patients was not reached; mOS for SRCC was 24 months. Multivariate analysis stratified for stage revealed significantly longer survival for TNET and GCC than SRCC (p < 0.001).
CONCLUSION
This is the largest report to date for appendiceal neuroendocrine tumor patients, suggesting a spectrum of diseases with different characteristics and outcomes. In this report, we present a treatment approach for this complex spectrum of disease, based on the experience of Ohio State and Emory Universities investigators.

Keyword

Appendiceal neoplasms; Neuroendocrine tumors; Goblet cells; Signet ring; Survival; Disease management

MeSH Terms

Appendiceal Neoplasms
Carcinoid Tumor
Demography
Disease Management
Epidemiology
Goblet Cells
Humans
Methods
Multivariate Analysis
Neuroendocrine Tumors
Ohio
Prognosis
Prospective Studies
Research Personnel

Figure

• 

  Fig. 1. Kaplan-Meier curves of localized appendiceal cancers comparing survival in different histologies. Localized typical neuroendocrine tumor (TNET) (hazard ratio [HR], 0.26; 95% confidence interval [CI], 0.15 to 0.46; p < 0.001) and goblet cell carcinoid (GCC) tumors (HR, 0.42; 95% CI, 0.26 to 0.69; p=0.001) have survival advantage, as compared with signet-ring cell cancer (SRCC) histology.

• 

  Fig. 2. Kaplan-Meier curves of regional disease comparing survival in different histologies. Regional typical neuroendocrine tumor (TNET) has survival advantage over goblet cell carcinoid (GCC) tumors (hazard ratio [HR], 0.37; 95% confidence interval [CI], 0.24 to 0.59; p < 0.001), which has a better survival, in turn, as compared to signet-ring cell cancer (SRCC) histology (HR, 0.29; 95% CI, 0.21 to 0.40; p < 0.001).

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  Fig. 3. Kaplan-Meier curves of distant disease comparing survival in different histologies. Distant typical neuroendocrine tumor (TNET) has survival advantage over goblet cell carcinoid (GCC) tumors (hazard ratio [HR], 0.61; 95% confidence interval [CI], 0.44 to 0.83; p=0.002), which has a better survival, in turn, as compared to signet-ring cell cancer (SRCC) histology (HR, 0.73; 95% CI, 0.60 to 0.90; p=0.003).

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  Fig. 4. Summary of the proposed treatment algorithm for typical NET (A), goblet cell carcinoid (B), and signet-ring cell cancer (SRCC) (C) tumors. Chemotherapy is fluropyrimidine based. NET, neuroendocrine tumor; HIPEC, hyperthermic intraperitoneal chemotherapy. a)HIPEC treatment treatment is controversial in SRCC.

Reference

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