Appendiceal neuroendocrine, goblet and signet-ring cell tumors: A spectrum of diseases with different patterns of presentation and outcome

Walid Shaib, Kavya Krishna, Sungjin Kim, Michael Goodman, Jonathan Rock, Zhengjia Chen, Edith Brutcher, Charles Staley, Shishir K. Maithel, Samih Abdel-Missih, Bassel F. El-Rayes, Tanios Bekaii-Saab

Research output: Contribution to journalArticle

9 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)596-604
Number of pages9
JournalCancer Research and Treatment
Volume48
Issue number2
DOIs
StatePublished - Apr 1 2016
Externally publishedYes

Fingerprint

Neuroendocrine Tumors
Carcinoid Tumor
Neoplasms
Survival
Epidemiology
Databases
Neuroendocrine Cells
Disease Management
Histology
Multivariate Analysis
Research Personnel
Demography
Prospective Studies

Keywords

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

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Appendiceal neuroendocrine, goblet and signet-ring cell tumors : A spectrum of diseases with different patterns of presentation and outcome. / Shaib, Walid; Krishna, Kavya; Kim, Sungjin; Goodman, Michael; Rock, Jonathan; Chen, Zhengjia; Brutcher, Edith; Staley, Charles; Maithel, Shishir K.; Abdel-Missih, Samih; El-Rayes, Bassel F.; Bekaii-Saab, Tanios.

In: Cancer Research and Treatment, Vol. 48, No. 2, 01.04.2016, p. 596-604.

Research output: Contribution to journalArticle

Shaib, W, Krishna, K, Kim, S, Goodman, M, Rock, J, Chen, Z, Brutcher, E, Staley, C, Maithel, SK, Abdel-Missih, S, El-Rayes, BF & Bekaii-Saab, T 2016, 'Appendiceal neuroendocrine, goblet and signet-ring cell tumors: A spectrum of diseases with different patterns of presentation and outcome', Cancer Research and Treatment, vol. 48, no. 2, pp. 596-604. https://doi.org/10.4143/crt.2015.029
Shaib, Walid ; Krishna, Kavya ; Kim, Sungjin ; Goodman, Michael ; Rock, Jonathan ; Chen, Zhengjia ; Brutcher, Edith ; Staley, Charles ; Maithel, Shishir K. ; Abdel-Missih, Samih ; El-Rayes, Bassel F. ; Bekaii-Saab, Tanios. / Appendiceal neuroendocrine, goblet and signet-ring cell tumors : A spectrum of diseases with different patterns of presentation and outcome. In: Cancer Research and Treatment. 2016 ; Vol. 48, No. 2. pp. 596-604.
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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.",
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T2 - A spectrum of diseases with different patterns of presentation and outcome

AU - Shaib, Walid

AU - Krishna, Kavya

AU - Kim, Sungjin

AU - Goodman, Michael

AU - Rock, Jonathan

AU - Chen, Zhengjia

AU - Brutcher, Edith

AU - Staley, Charles

AU - Maithel, Shishir K.

AU - Abdel-Missih, Samih

AU - El-Rayes, Bassel F.

AU - Bekaii-Saab, Tanios

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Y1 - 2016/4/1

N2 - 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.

AB - 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.

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KW - Disease management

KW - Goblet cells

KW - Neuroendocrine tumors

KW - Signet ring

KW - Survival

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