Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients

Ingrid Holst Olsen; Nanna Holt; Seppo W Langer; Jane Preuss Hasselby; Henning Grønbæk; Jens Hillingsø; Masti Mahmoud; Morten Ladekarl; Lene H Iversen; Andreas Kjær; Birgitte H Federspiel; Ulrich Knigge

doi:10.1371/journal.pone.0117627

Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients

Ingrid Holst Olsen, Nanna Holt, Seppo W Langer, Jane Preuss Hasselby, Henning Grønbæk, Jens Hillingsø, Masti Mahmoud, Morten Ladekarl, Lene H Iversen, Andreas Kjær, Birgitte H Federspiel, Ulrich Knigge

13 Citations (Scopus)

Abstract

Background: Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features. Patients and Methods: Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013. Results: Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. at diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p <0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002). Conclusion: The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.

Original language	English
Article number	e0117627
Journal	P L o S One
Volume	10
Issue number	2
Pages (from-to)	1-17
Number of pages	17
ISSN	1932-6203
DOIs	https://doi.org/10.1371/journal.pone.0117627
Publication status	Published - 11 Feb 2015

Keywords

Adult
Aged
Carcinoid Tumor
Cohort Studies
Databases, Factual
Denmark
Female
Humans
Male
Middle Aged
Neoplasm Staging
Postoperative Period
Prognosis
Receptors, Somatostatin
Retrospective Studies
Survival Analysis

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@article{8a831c8d27f74a7ba95389f6b95a8de3,

title = "Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients",

abstract = "Background: Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features. Patients and Methods: Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013. Results: Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. at diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p <0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002). Conclusion: The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.",

keywords = "Adult, Aged, Carcinoid Tumor, Cohort Studies, Databases, Factual, Denmark, Female, Humans, Male, Middle Aged, Neoplasm Staging, Postoperative Period, Prognosis, Receptors, Somatostatin, Retrospective Studies, Survival Analysis",

author = "Olsen, {Ingrid Holst} and Nanna Holt and Langer, {Seppo W} and Hasselby, {Jane Preuss} and Henning Gr{\o}nb{\ae}k and Jens Hillings{\o} and Masti Mahmoud and Morten Ladekarl and Iversen, {Lene H} and Andreas Kj{\ae}r and Federspiel, {Birgitte H} and Ulrich Knigge",

year = "2015",

month = feb,

day = "11",

doi = "10.1371/journal.pone.0117627",

language = "English",

volume = "10",

pages = "1--17",

journal = "PLoS Computational Biology",

issn = "1932-6203",

publisher = "Public Library of Science",

number = "2",

}

TY - JOUR

T1 - Goblet cell carcinoids

T2 - characteristics of a Danish cohort of 83 patients

AU - Olsen, Ingrid Holst

AU - Holt, Nanna

AU - Langer, Seppo W

AU - Hasselby, Jane Preuss

AU - Grønbæk, Henning

AU - Hillingsø, Jens

AU - Mahmoud, Masti

AU - Ladekarl, Morten

AU - Iversen, Lene H

AU - Kjær, Andreas

AU - Federspiel, Birgitte H

AU - Knigge, Ulrich

PY - 2015/2/11

Y1 - 2015/2/11

N2 - Background: Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features. Patients and Methods: Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013. Results: Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. at diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p <0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002). Conclusion: The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.

AB - Background: Appendiceal goblet cell carcinoids (GCCs) exhibit neuroendocrine and adenocarcinoma features. Patients and Methods: Analysis of demography, pathology, prognostic markers, treatment and survival in 83 GCC patients (f/m: 56/27) diagnosed 1992-2013. Results: Median age for f/m was 59/58 years, respectively, and similar for localized and disseminated disease. at diagnosis 54 patients had localized appendiceal disease (f/m: 29/25). According to TNM 24% had Stage I, 70% had Stage II and 6% had Stage III. Twenty-nine patients had disseminated disease (f/m: 27/2). Chromogranin A, synaptophysin and p53 were positive in >90%. Serotonin was positive in 70%. Median Ki67 index was 32% (6-75%) and higher in Tang group C (50%) compared to group A (30%; p<0.0001), and group B (30%; p <0.004). All patients had surgery. Sixty-three (76%) had radical resections including all patients with localized disease. Median OS was 83 months. The 1-, 5- and 10-year survival rates were 90%, 58%, and 38%, respectively. For localized disease OS was 164 months and 1-, 5- and 10-year survival rates were 100%, 80%, and 55%, respectively. For disseminated disease OS was 19 months and 1-, 5- and 10-year survival rates were 73%, 18% and 6%, respectively. The 1-, 5- and 10 year-survival rates for f/m were 87%/96%, 49%/76% and 31%/57%, respectively (p = 0.02). According to the Tang classification group A, B, and C OS was 118, 83 and 20 months, respectively (p = 0.0002). Conclusion: The Tang classification was found to be a significant prognostic factor, while the Ki67 index was not. Localized GCCs occurred equally in males and females, while disseminated GCCs were mostly seen in females. Median age of patients with localized disease and disseminated disease was identical. Cox regression analysis found Stage IV, focally positive synaptophysin and non-radical surgery as strongest negative prognostic factors.

KW - Adult

KW - Aged

KW - Carcinoid Tumor

KW - Cohort Studies

KW - Databases, Factual

KW - Denmark

KW - Female

KW - Humans

KW - Male

KW - Middle Aged

KW - Neoplasm Staging

KW - Postoperative Period

KW - Prognosis

KW - Receptors, Somatostatin

KW - Retrospective Studies

KW - Survival Analysis

U2 - 10.1371/journal.pone.0117627

DO - 10.1371/journal.pone.0117627

M3 - Journal article

C2 - 25671432

SN - 1932-6203

VL - 10

SP - 1

EP - 17

JO - PLoS Computational Biology

JF - PLoS Computational Biology

IS - 2

M1 - e0117627

ER -

Goblet cell carcinoids: characteristics of a Danish cohort of 83 patients

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Keywords

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