Cargando…
Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management
Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopa...
Autores principales: | , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Bioscientifica Ltd
2018
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5801558/ https://www.ncbi.nlm.nih.gov/pubmed/29335251 http://dx.doi.org/10.1530/EC-17-0311 |
_version_ | 1783298367384715264 |
---|---|
author | Clift, Ashley K Kornasiewicz, Oskar Drymousis, Panagiotis Faiz, Omar Wasan, Harpreet S Kinross, James M Cecil, Thomas Frilling, Andrea |
author_facet | Clift, Ashley K Kornasiewicz, Oskar Drymousis, Panagiotis Faiz, Omar Wasan, Harpreet S Kinross, James M Cecil, Thomas Frilling, Andrea |
author_sort | Clift, Ashley K |
collection | PubMed |
description | Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopathological data were extracted from a prospectively maintained database. Survival analyses utilised Kaplan–Meier methodology. Twenty-one patients were identified (9 females). Median age at diagnosis was 55 years (range 32–77). There were 3, 6 and 9 grade 1, 2 and 3 tumours, respectively. One, 10, 5 and 5 patients had stage I, II, III and IV disease at diagnosis, respectively. There were 8, 10 and 3 Tang class A, B and C tumours, respectively. Index operation was appendectomy (n = 12), right hemicolectomy (n = 6) or resections including appendix/right colon, omentum and the gynaecological system (n = 3). Eight patients underwent completion right hemicolectomy. Surgery for recurrence included small bowel resection (n = 2), debulking with peritonectomy and heated intraperitoneal chemotherapy, and hysterectomy and bilateral salpingo-oophorectomy (all n = 1). Median follow-up was 30 months (range 2.5–123). One-, 3- and 5-year OS was 79.4, 60 and 60%, respectively. Mean OS (1-, 3-, and 5-year OS) for Tang class A, B and C tumours were 73.1 months (85.7, 85.7, 51.4%), 83.7 months (all 66.7%) and 28.5 months (66.7, 66.7%, not reached), respectively. Chromogranin A/B and (68)Ga-DOTATATE PET/CT were not useful in follow-up, but CEA, CA 19-9, CA 125 and (18)F-FDG PET/CT identified tumour recurrence. GCC must be clearly discriminated from relatively indolent appendiceal neuroendocrine neoplasms. (18)F-FDG PET/CT and CEA/CA19-9/CA 125 are useful in detecting recurrence of GCC. |
format | Online Article Text |
id | pubmed-5801558 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Bioscientifica Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-58015582018-02-12 Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management Clift, Ashley K Kornasiewicz, Oskar Drymousis, Panagiotis Faiz, Omar Wasan, Harpreet S Kinross, James M Cecil, Thomas Frilling, Andrea Endocr Connect Research Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopathological data were extracted from a prospectively maintained database. Survival analyses utilised Kaplan–Meier methodology. Twenty-one patients were identified (9 females). Median age at diagnosis was 55 years (range 32–77). There were 3, 6 and 9 grade 1, 2 and 3 tumours, respectively. One, 10, 5 and 5 patients had stage I, II, III and IV disease at diagnosis, respectively. There were 8, 10 and 3 Tang class A, B and C tumours, respectively. Index operation was appendectomy (n = 12), right hemicolectomy (n = 6) or resections including appendix/right colon, omentum and the gynaecological system (n = 3). Eight patients underwent completion right hemicolectomy. Surgery for recurrence included small bowel resection (n = 2), debulking with peritonectomy and heated intraperitoneal chemotherapy, and hysterectomy and bilateral salpingo-oophorectomy (all n = 1). Median follow-up was 30 months (range 2.5–123). One-, 3- and 5-year OS was 79.4, 60 and 60%, respectively. Mean OS (1-, 3-, and 5-year OS) for Tang class A, B and C tumours were 73.1 months (85.7, 85.7, 51.4%), 83.7 months (all 66.7%) and 28.5 months (66.7, 66.7%, not reached), respectively. Chromogranin A/B and (68)Ga-DOTATATE PET/CT were not useful in follow-up, but CEA, CA 19-9, CA 125 and (18)F-FDG PET/CT identified tumour recurrence. GCC must be clearly discriminated from relatively indolent appendiceal neuroendocrine neoplasms. (18)F-FDG PET/CT and CEA/CA19-9/CA 125 are useful in detecting recurrence of GCC. Bioscientifica Ltd 2018-01-15 /pmc/articles/PMC5801558/ /pubmed/29335251 http://dx.doi.org/10.1530/EC-17-0311 Text en © 2018 The authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (http://creativecommons.org/licenses/by-nc-nd/4.0/) . |
spellingShingle | Research Clift, Ashley K Kornasiewicz, Oskar Drymousis, Panagiotis Faiz, Omar Wasan, Harpreet S Kinross, James M Cecil, Thomas Frilling, Andrea Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
title | Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
title_full | Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
title_fullStr | Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
title_full_unstemmed | Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
title_short | Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
title_sort | goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5801558/ https://www.ncbi.nlm.nih.gov/pubmed/29335251 http://dx.doi.org/10.1530/EC-17-0311 |
work_keys_str_mv | AT cliftashleyk gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT kornasiewiczoskar gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT drymousispanagiotis gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT faizomar gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT wasanharpreets gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT kinrossjamesm gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT cecilthomas gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement AT frillingandrea gobletcellcarcinomasoftheappendixrarebutaggressiveneoplasmswithchallengingmanagement |