Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States

Mixed acinar neuroendocrine carcinoma of the pancreas (MANEC-P) is an extremely rare malignancy with a poor prognosis. However, epidemiological estimates of MANEC-P remain unknown. This study aimed to estimate and compare the incidence, prevalence, and cancer-specific survival (CSS) of MANEC-P in th...

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Main Authors: Amro M. Abdelrahman, Jun Yin, Roberto Alva-Ruiz, Jennifer A. Yonkus, Jennifer L. Leiting, Isaac T. Lynch, Alessandro Fogliati, Nellie A. Campbell, Danielle M. Carlson, Lewis R. Roberts, Gregory J. Gores, Rory L. Smoot, Rondell P. Graham, Thorvardur R. Halfdanarson, Mark J. Truty
Format: Article
Language:English
Published: MDPI AG 2023-01-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/15/3/840
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author Amro M. Abdelrahman
Jun Yin
Roberto Alva-Ruiz
Jennifer A. Yonkus
Jennifer L. Leiting
Isaac T. Lynch
Alessandro Fogliati
Nellie A. Campbell
Danielle M. Carlson
Lewis R. Roberts
Gregory J. Gores
Rory L. Smoot
Rondell P. Graham
Thorvardur R. Halfdanarson
Mark J. Truty
author_facet Amro M. Abdelrahman
Jun Yin
Roberto Alva-Ruiz
Jennifer A. Yonkus
Jennifer L. Leiting
Isaac T. Lynch
Alessandro Fogliati
Nellie A. Campbell
Danielle M. Carlson
Lewis R. Roberts
Gregory J. Gores
Rory L. Smoot
Rondell P. Graham
Thorvardur R. Halfdanarson
Mark J. Truty
author_sort Amro M. Abdelrahman
collection DOAJ
description Mixed acinar neuroendocrine carcinoma of the pancreas (MANEC-P) is an extremely rare malignancy with a poor prognosis. However, epidemiological estimates of MANEC-P remain unknown. This study aimed to estimate and compare the incidence, prevalence, and cancer-specific survival (CSS) of MANEC-P in the United States (US). Patients with MANEC-P were identified through the Surveillance, Epidemiology, and End Results (SEER) and National Program of Cancer Registries databases between 2000–2017. The primary outcomes included age-adjusted incidence rate, limited-duration prevalence, and CSS. A total of 630 patients were identified for the incidence analysis and 149 for the prevalence and CSS analyses. The MANEC-P incidence rate was 0.011 per 100,000 individuals, which was the lowest among pancreatic cancer histologic subtypes. The incidence rate was significantly higher in men and Black races and peaked at 75–79 years of age. The incidence rate was the lowest in the midwestern region (0.009) and the highest in the northeastern US (0.013). The 17-year prevalence was 0.00005%, indicating that 189 patients were alive in the United States at the beginning of 2018. The median CSS of MANEC-P was estimated to be 41 (23, 69) months. In conclusion, MANEC-P is very rare, and its incidence rate has been steady in the US over the last two decades. MANEC-P has a poor prognosis and is the 5th leading cause of pancreatic cancer-related death in the US.
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spelling doaj.art-3f8332a3d41c4fbaa4d6634738c0e7032023-11-16T16:17:58ZengMDPI AGCancers2072-66942023-01-0115384010.3390/cancers15030840Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United StatesAmro M. Abdelrahman0Jun Yin1Roberto Alva-Ruiz2Jennifer A. Yonkus3Jennifer L. Leiting4Isaac T. Lynch5Alessandro Fogliati6Nellie A. Campbell7Danielle M. Carlson8Lewis R. Roberts9Gregory J. Gores10Rory L. Smoot11Rondell P. Graham12Thorvardur R. Halfdanarson13Mark J. Truty14Division of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Clinical Trials and Biostatistics, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USADivision of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USADivision of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USADivision of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USADivision of Anatomic Pathology, Mayo Clinic, Rochester, MN 55905, USADivision of Medical Oncology, Mayo Clinic, Rochester, MN 55905, USADivision of Hepatobiliary and Pancreas Surgery, Mayo Clinic, Rochester, MN 55905, USAMixed acinar neuroendocrine carcinoma of the pancreas (MANEC-P) is an extremely rare malignancy with a poor prognosis. However, epidemiological estimates of MANEC-P remain unknown. This study aimed to estimate and compare the incidence, prevalence, and cancer-specific survival (CSS) of MANEC-P in the United States (US). Patients with MANEC-P were identified through the Surveillance, Epidemiology, and End Results (SEER) and National Program of Cancer Registries databases between 2000–2017. The primary outcomes included age-adjusted incidence rate, limited-duration prevalence, and CSS. A total of 630 patients were identified for the incidence analysis and 149 for the prevalence and CSS analyses. The MANEC-P incidence rate was 0.011 per 100,000 individuals, which was the lowest among pancreatic cancer histologic subtypes. The incidence rate was significantly higher in men and Black races and peaked at 75–79 years of age. The incidence rate was the lowest in the midwestern region (0.009) and the highest in the northeastern US (0.013). The 17-year prevalence was 0.00005%, indicating that 189 patients were alive in the United States at the beginning of 2018. The median CSS of MANEC-P was estimated to be 41 (23, 69) months. In conclusion, MANEC-P is very rare, and its incidence rate has been steady in the US over the last two decades. MANEC-P has a poor prognosis and is the 5th leading cause of pancreatic cancer-related death in the US.https://www.mdpi.com/2072-6694/15/3/840MANEC-Pepidemiologyrare cancersmixed carcinomacancer surveillance and screening
spellingShingle Amro M. Abdelrahman
Jun Yin
Roberto Alva-Ruiz
Jennifer A. Yonkus
Jennifer L. Leiting
Isaac T. Lynch
Alessandro Fogliati
Nellie A. Campbell
Danielle M. Carlson
Lewis R. Roberts
Gregory J. Gores
Rory L. Smoot
Rondell P. Graham
Thorvardur R. Halfdanarson
Mark J. Truty
Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States
Cancers
MANEC-P
epidemiology
rare cancers
mixed carcinoma
cancer surveillance and screening
title Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States
title_full Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States
title_fullStr Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States
title_full_unstemmed Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States
title_short Mixed Acinar Neuroendocrine Carcinoma of the Pancreas: Comparative Population-Based Epidemiology of a Rare and Fatal Malignancy in The United States
title_sort mixed acinar neuroendocrine carcinoma of the pancreas comparative population based epidemiology of a rare and fatal malignancy in the united states
topic MANEC-P
epidemiology
rare cancers
mixed carcinoma
cancer surveillance and screening
url https://www.mdpi.com/2072-6694/15/3/840
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