Supporting the

Neuroendocrine Cancer Community

Goblet Cell Adenocarcinoma of the Appendix (GCA) 

Mar 20, 2024

Some of you may have seen an article published in the Mail Online (19th March) about a young mum, diagnosed with Goblet Cell Adenocarcinoma of the Appendix.
 
Our thoughts are with this young woman and her family. However, we would like to reduce any confusion or distress certain inaccuracies, within this article, may have caused.
We have contacted the reporter to discuss the content.

 We would like to make it clear that we were not approached or contacted prior to the article being published. 

We would also like to confirm that our information – available on our website here – makes it clear that Goblet Cell Carcinoma of the Appendix should be considered as a completely different entity to Neuroendocrine Cancer of the Appendix.  

 . . . and that we do NOT state that chemo and immunotherapy is offered when there is a higher risk of the cancer returning”.   

In fact, we follow expert UK and International guidance in stating that: 

In advanced disease (where there is evidence of spread within the abdomen) referral to a Peritoneal Cancer specialist centre for consideration of Cyto-reductive Surgery +/- HIPEC should occur.” 

Goblet cell adenocarcinoma of the appendix is a subtype of appendiceal adenocarcinoma, which is itself a rare form of cancer. Cancers of the appendix represent less than 1% of all gastrointestinal malignancies, and according to Hoehn et al (2021) there are 5 main subtypes: 

  • neuroendocrine neoplasms (NENs) – which include neuroendocrine tumours (NETs) and neuroendocrine carcinomas (NECs) 
  • mucinous neoplasms,  
  • goblet cell adenocarcinomas (GCAs),  
  • colonic-type (non-mucinous) adenocarcinomas,  
  • signet ring cell adenocarcinomas 

 

Formerly called goblet cell carcinoids”, in 2019, the World Health Organisation classification, renamed them as goblet cell adenocarcinomas (GCAs), to distinguish them as a separate tumor entity to other forms of appendiceal cancers. 

NB Carcinoid is also an outdated term for neuroendocrine neoplasms but is slowly being replaced! 

Goblet cell adenocarcinomas (GCAs) are complex as they demonstrate a mixed histology, and though they may display some neuroendocrine features, what is more significant, in terms of the behaviour, treatment and prognosis of disease, is the mucin-secreting gland formation –  as seen in typical adenocarcinomas. They are histologically distinct from NENs: as 

 “Despite previous goblet cell carcinoid terminology, goblet cell adenocarcinoma is considered a variant of adenocarcinoma and is distinct from neuroendocrine neoplasm”  

Lollie & Wang (2022) 

 In terms of diagnosis – GCA – like many cancers of the appendix, is more commonly a post-appendicectomy finding : however, experts note that – unlike neuroendocrine cancers of the appendix: 

  • The serotonin, chromogranin A, and imaging used for NETs (e.g., DOTATATE PET/CT) are typically not helpful for GCAs.  
  • Whereas tumour markers, as used in adenocarcinoma care namely CEA, CA 19-9, and CA-125, may be more informative – and FDG-PET (rather than Dotatate imaging), alongside CT and MRI, may be more helpful in GCA staging and surveillance.

 

The key aim of treatment is centred around promoting the best possible care and quality of life – by ensuring access to appropriate treatment, management of symptoms and addressing what’s most important to you (as someone diagnosed with GCA). 

Treatment options will depend on the type, position, and size of your cancer – and whether (and to where) it has spread. It will also depend on whether you have any other health concerns and/or illnesses and your general health and fitness.  

Options may include surgery, however, in advanced disease – where complete resection may not be possible, specialist centre referral* should be made so that cyto-reductive surgery +/- HIPEC may be considered. 

In inoperable, recurrent, or metastatic disease, palliative (that is, non-curative) chemotherapy may be offered to try to gain disease control and /or provide symptom relief. The chemotherapy used would be similar to that used in colorectal cancer. Clinical trial therapy may also be considered. 

*Both Basingstoke and The Christie Hospitals are recognised Peritoneal Cancer Centres – specialising in cyto-reductive surgery +/- HIPEC – further information about these services and the treatments they provide can be found by visiting their respective websites: 

  

References 

  • Hoehn et al.,(2021) Current Management of Appendiceal Neoplasms. Am Soc Clin Oncol Educ Book 41, 118-132. 
  • Palmer et al (2022) Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry. Front Oncol.:12:915028. 
  • Lollie & Wang (2022) Goblet cell adenocarcinoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/appendixgobletcellcarcinoid.html  
  • Wang HL (2022). Goblet cell adenocarcinoma of the appendix: Diagnosis, prognosis and nomenclature. Journal of Clinical and Translational Pathology. 
  • Köhler et al (2023) Neoplasms of the Appendix. Dtsch Arztebl Int.:120(31-32):519-525 
  • UKINETs Bitesize Guidance (2016) : https://www.ukinets.org/wp-content/uploads/UKINETS-bitesize-guidance-GCC-1.pdf