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 - P L o S One
JF - P L o S One
IS - 2
M1 - e0117627
ER -