Mixed adeno-neuroendocrine carcinoma (MANEC) of the gastroenteropancreatic (GEP) tract: A multicentre retrospective study

Melissa Frizziero; Xin Wang; Bipasha Chakrabarty; Alexa Childs; TV Luong; Thomas Walter; M Elshafie; T Shah; Paul Fulford; A Minicozzi; Was Mansoor; Tim Meyer; Richard Hubner; Juan Valle; Mairead Mcnamara

Abstract

Background: MANEC is a rare entity and evidence on its prognosis and management is limited. Methods: Demographic/clinicopathological/survival data of consecutive patients (pts) with a diagnosis of MANEC (2010 WHO criteria) from 4 European centres were retrospectively reviewed. Results: Fifty-three pts were identified (01/06-03/17); median (med) age: 62 yrs (range 34-89), male: 70%, ECOG PS 0-1: 60%, with primary tumours from small/large bowel in 34 (64%), oesophagus/stomach: 13 (24.5%), pancreas/biliary tract: 5 (9.5%), unknown (UNK): 1 (2%). Forty percent had an adult comorbidity evaluation (ACE)-27 score of 0. The neuroendocrine (NE) component (predominant histology in 40%) was poorly-differentiated (PD) in 45 (85%) [Ki-67≥55%: 58%]. Most frequently-expressed immunohistochemical (IHC) markers were synaptophysin (100%), chromogranin A (CgA) (58.5%) and CDX2 (51%). Histology was PD NE in 64% from recurrent/metastatic sites (n = 14 pts). Of 28 (53%) pts with localised disease (LA), 26 (93%) had curative surgery (7 had neoadjuvant chemo-radiotherapy (CT-RT), 6 adjuvant CT, 1 peri-operative CT), 1 (3.5%) had definitive CT-RT and 1 (3.5%) had UNK management; 16 (57%) recurred. Forty-one pts (77%) were treated for advanced (adv) disease: 20 (49%) platinum-based CT, 3 (7%) irinotecan-based CT, 1 (2.4%) gemcitabine, 3 (7%) UNK CT regimen, 1 (2.4%) RT, 1 (2.4%) CT-RT, 11 (27%) best supportive care (BSC), and 1 (2.4%) UNK management. Med follow-up time was 10.4 months (mo) (95% Confidence Interval (CI) 5.15-13.09). Med overall survival (OS) for all pts was 18.6 mo (95% CI 11.4-40). Med recurrence-free survival and OS in pts with LA was 19.4 mo (95%CI 5.8-30.9) and 21 mo (95%CI 12.1-40). Med progression free survival (PFS) and OS in pts with adv disease was 4.6 mo (95%CI 3.3-6.7) and 13.6 mo (95%CI 8.8-33.1). On univariable analysis, ACE-27 score (0 vs ≥ 1) was prognostic for better PFS and OS (both p < 0.05); IHC negativity for CgA and active treatment (vs BSC) were prognostic for better PFS (both p < 0.05). Conclusions: PD NE histology in MANECs was predominant in both diagnostic and recurrent/metastatic tumour samples. Active treatments were offered to most pts but more effective therapy is clearly needed.

Original language	English
Publication status	Published - Sept 2017
Event	ESMO 2017 Congress - Madrid, Madrid, Spain Duration: 8 Sept 2017 → 12 Sept 2017

Conference

Conference	ESMO 2017 Congress
Country/Territory	Spain
City	Madrid
Period	8/09/17 → 12/09/17

Keywords

MANEC
retrospective study
Treatment

Research Beacons, Institutes and Platforms

Manchester Cancer Research Centre

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

https://doi.org/10.1093/annonc/mdx368.038

Cite this

Frizziero, M., Wang, X., Chakrabarty, B., Childs, A., Luong, TV., Walter, T., Elshafie, M., Shah, T., Fulford, P., Minicozzi, A., Mansoor, W., Meyer, T., Hubner, R., Valle, J., & Mcnamara, M. (2017). Mixed adeno-neuroendocrine carcinoma (MANEC) of the gastroenteropancreatic (GEP) tract: A multicentre retrospective study. Abstract from ESMO 2017 Congress, Madrid, Spain. https://doi.org/10.1093/annonc/mdx368.038

@conference{dbb88f8f364044daa94d5c1c02834b22,

title = "Mixed adeno-neuroendocrine carcinoma (MANEC) of the gastroenteropancreatic (GEP) tract: A multicentre retrospective study",

abstract = "Background: MANEC is a rare entity and evidence on its prognosis and management is limited. Methods: Demographic/clinicopathological/survival data of consecutive patients (pts) with a diagnosis of MANEC (2010 WHO criteria) from 4 European centres were retrospectively reviewed. Results: Fifty-three pts were identified (01/06-03/17); median (med) age: 62 yrs (range 34-89), male: 70\%, ECOG PS 0-1: 60\%, with primary tumours from small/large bowel in 34 (64\%), oesophagus/stomach: 13 (24.5\%), pancreas/biliary tract: 5 (9.5\%), unknown (UNK): 1 (2\%). Forty percent had an adult comorbidity evaluation (ACE)-27 score of 0. The neuroendocrine (NE) component (predominant histology in 40\%) was poorly-differentiated (PD) in 45 (85\%) [Ki-67≥55\%: 58\%]. Most frequently-expressed immunohistochemical (IHC) markers were synaptophysin (100\%), chromogranin A (CgA) (58.5\%) and CDX2 (51\%). Histology was PD NE in 64\% from recurrent/metastatic sites (n = 14 pts). Of 28 (53\%) pts with localised disease (LA), 26 (93\%) had curative surgery (7 had neoadjuvant chemo-radiotherapy (CT-RT), 6 adjuvant CT, 1 peri-operative CT), 1 (3.5\%) had definitive CT-RT and 1 (3.5\%) had UNK management; 16 (57\%) recurred. Forty-one pts (77\%) were treated for advanced (adv) disease: 20 (49\%) platinum-based CT, 3 (7\%) irinotecan-based CT, 1 (2.4\%) gemcitabine, 3 (7\%) UNK CT regimen, 1 (2.4\%) RT, 1 (2.4\%) CT-RT, 11 (27\%) best supportive care (BSC), and 1 (2.4\%) UNK management. Med follow-up time was 10.4 months (mo) (95\% Confidence Interval (CI) 5.15-13.09). Med overall survival (OS) for all pts was 18.6 mo (95\% CI 11.4-40). Med recurrence-free survival and OS in pts with LA was 19.4 mo (95\%CI 5.8-30.9) and 21 mo (95\%CI 12.1-40). Med progression free survival (PFS) and OS in pts with adv disease was 4.6 mo (95\%CI 3.3-6.7) and 13.6 mo (95\%CI 8.8-33.1). On univariable analysis, ACE-27 score (0 vs ≥ 1) was prognostic for better PFS and OS (both p < 0.05); IHC negativity for CgA and active treatment (vs BSC) were prognostic for better PFS (both p < 0.05). Conclusions: PD NE histology in MANECs was predominant in both diagnostic and recurrent/metastatic tumour samples. Active treatments were offered to most pts but more effective therapy is clearly needed. ",

keywords = "MANEC, retrospective study, Treatment",

author = "Melissa Frizziero and Xin Wang and Bipasha Chakrabarty and Alexa Childs and TV Luong and Thomas Walter and M Elshafie and T Shah and Paul Fulford and A Minicozzi and Was Mansoor and Tim Meyer and Richard Hubner and Juan Valle and Mairead Mcnamara",

year = "2017",

month = sep,

language = "English",

note = "ESMO 2017 Congress ; Conference date: 08-09-2017 Through 12-09-2017",

}

Frizziero, M, Wang, X, Chakrabarty, B, Childs, A, Luong, TV, Walter, T, Elshafie, M, Shah, T, Fulford, P, Minicozzi, A, Mansoor, W, Meyer, T, Hubner, R, Valle, J & Mcnamara, M 2017, 'Mixed adeno-neuroendocrine carcinoma (MANEC) of the gastroenteropancreatic (GEP) tract: A multicentre retrospective study', ESMO 2017 Congress, Madrid, Spain, 8/09/17 - 12/09/17. <https://doi.org/10.1093/annonc/mdx368.038>

TY - CONF

T1 - Mixed adeno-neuroendocrine carcinoma (MANEC) of the gastroenteropancreatic (GEP) tract

T2 - ESMO 2017 Congress

AU - Frizziero, Melissa

AU - Wang, Xin

AU - Chakrabarty, Bipasha

AU - Childs, Alexa

AU - Luong, TV

AU - Walter, Thomas

AU - Elshafie, M

AU - Shah, T

AU - Fulford, Paul

AU - Minicozzi, A

AU - Mansoor, Was

AU - Meyer, Tim

AU - Hubner, Richard

AU - Valle, Juan

AU - Mcnamara, Mairead

PY - 2017/9

Y1 - 2017/9

N2 - Background: MANEC is a rare entity and evidence on its prognosis and management is limited. Methods: Demographic/clinicopathological/survival data of consecutive patients (pts) with a diagnosis of MANEC (2010 WHO criteria) from 4 European centres were retrospectively reviewed. Results: Fifty-three pts were identified (01/06-03/17); median (med) age: 62 yrs (range 34-89), male: 70%, ECOG PS 0-1: 60%, with primary tumours from small/large bowel in 34 (64%), oesophagus/stomach: 13 (24.5%), pancreas/biliary tract: 5 (9.5%), unknown (UNK): 1 (2%). Forty percent had an adult comorbidity evaluation (ACE)-27 score of 0. The neuroendocrine (NE) component (predominant histology in 40%) was poorly-differentiated (PD) in 45 (85%) [Ki-67≥55%: 58%]. Most frequently-expressed immunohistochemical (IHC) markers were synaptophysin (100%), chromogranin A (CgA) (58.5%) and CDX2 (51%). Histology was PD NE in 64% from recurrent/metastatic sites (n = 14 pts). Of 28 (53%) pts with localised disease (LA), 26 (93%) had curative surgery (7 had neoadjuvant chemo-radiotherapy (CT-RT), 6 adjuvant CT, 1 peri-operative CT), 1 (3.5%) had definitive CT-RT and 1 (3.5%) had UNK management; 16 (57%) recurred. Forty-one pts (77%) were treated for advanced (adv) disease: 20 (49%) platinum-based CT, 3 (7%) irinotecan-based CT, 1 (2.4%) gemcitabine, 3 (7%) UNK CT regimen, 1 (2.4%) RT, 1 (2.4%) CT-RT, 11 (27%) best supportive care (BSC), and 1 (2.4%) UNK management. Med follow-up time was 10.4 months (mo) (95% Confidence Interval (CI) 5.15-13.09). Med overall survival (OS) for all pts was 18.6 mo (95% CI 11.4-40). Med recurrence-free survival and OS in pts with LA was 19.4 mo (95%CI 5.8-30.9) and 21 mo (95%CI 12.1-40). Med progression free survival (PFS) and OS in pts with adv disease was 4.6 mo (95%CI 3.3-6.7) and 13.6 mo (95%CI 8.8-33.1). On univariable analysis, ACE-27 score (0 vs ≥ 1) was prognostic for better PFS and OS (both p < 0.05); IHC negativity for CgA and active treatment (vs BSC) were prognostic for better PFS (both p < 0.05). Conclusions: PD NE histology in MANECs was predominant in both diagnostic and recurrent/metastatic tumour samples. Active treatments were offered to most pts but more effective therapy is clearly needed.

AB - Background: MANEC is a rare entity and evidence on its prognosis and management is limited. Methods: Demographic/clinicopathological/survival data of consecutive patients (pts) with a diagnosis of MANEC (2010 WHO criteria) from 4 European centres were retrospectively reviewed. Results: Fifty-three pts were identified (01/06-03/17); median (med) age: 62 yrs (range 34-89), male: 70%, ECOG PS 0-1: 60%, with primary tumours from small/large bowel in 34 (64%), oesophagus/stomach: 13 (24.5%), pancreas/biliary tract: 5 (9.5%), unknown (UNK): 1 (2%). Forty percent had an adult comorbidity evaluation (ACE)-27 score of 0. The neuroendocrine (NE) component (predominant histology in 40%) was poorly-differentiated (PD) in 45 (85%) [Ki-67≥55%: 58%]. Most frequently-expressed immunohistochemical (IHC) markers were synaptophysin (100%), chromogranin A (CgA) (58.5%) and CDX2 (51%). Histology was PD NE in 64% from recurrent/metastatic sites (n = 14 pts). Of 28 (53%) pts with localised disease (LA), 26 (93%) had curative surgery (7 had neoadjuvant chemo-radiotherapy (CT-RT), 6 adjuvant CT, 1 peri-operative CT), 1 (3.5%) had definitive CT-RT and 1 (3.5%) had UNK management; 16 (57%) recurred. Forty-one pts (77%) were treated for advanced (adv) disease: 20 (49%) platinum-based CT, 3 (7%) irinotecan-based CT, 1 (2.4%) gemcitabine, 3 (7%) UNK CT regimen, 1 (2.4%) RT, 1 (2.4%) CT-RT, 11 (27%) best supportive care (BSC), and 1 (2.4%) UNK management. Med follow-up time was 10.4 months (mo) (95% Confidence Interval (CI) 5.15-13.09). Med overall survival (OS) for all pts was 18.6 mo (95% CI 11.4-40). Med recurrence-free survival and OS in pts with LA was 19.4 mo (95%CI 5.8-30.9) and 21 mo (95%CI 12.1-40). Med progression free survival (PFS) and OS in pts with adv disease was 4.6 mo (95%CI 3.3-6.7) and 13.6 mo (95%CI 8.8-33.1). On univariable analysis, ACE-27 score (0 vs ≥ 1) was prognostic for better PFS and OS (both p < 0.05); IHC negativity for CgA and active treatment (vs BSC) were prognostic for better PFS (both p < 0.05). Conclusions: PD NE histology in MANECs was predominant in both diagnostic and recurrent/metastatic tumour samples. Active treatments were offered to most pts but more effective therapy is clearly needed.

KW - MANEC

KW - retrospective study

KW - Treatment

M3 - Abstract

Y2 - 8 September 2017 through 12 September 2017

ER -