# Goblet cell adenocarcinoma of the appendix

Source: https://onco.cc/cancers/goblet-cell-adenocarcinoma/  
OnCo record `goblet-cell-adenocarcinoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Goblet cell adenocarcinoma is a rare appendix cancer whose cells mix mucus-filled goblet cells with neuroendocrine features, once called goblet cell carcinoid but now classed and treated as an adenocarcinoma. It is removed by right hemicolectomy, given bowel-cancer chemotherapy when it is high grade or has spread, and not treated with the somatostatin drugs used for true neuroendocrine tumours.

## Summary

Goblet cell adenocarcinoma is an amphicrine tumour: its cells contain both mucin, like intestinal goblet cells, and neuroendocrine granules, and it grows in a concentric, infiltrative pattern through the appendiceal wall, often without a visible mass, so it is usually discovered after appendicectomy for appendicitis. Because of its neuroendocrine component it was long called goblet cell carcinoid and lumped with appendiceal neuroendocrine tumours, but it spreads like a carcinoma, to the peritoneum and, in women, to the ovaries, and does not express somatostatin receptors or respond to somatostatin analogues. The 2019 WHO classification renamed it goblet cell adenocarcinoma and grades it by the proportion of tubular or clustered goblet cell growth against poorly cohesive or signet ring growth (grades 1 to 3), replacing the earlier Tang classification; grade and stage determine survival, which is long for grade 1 tumours confined to the appendix and short for grade 3 tumours with peritoneal spread. The genetics are distinct from both appendiceal adenocarcinoma and neuroendocrine tumours, with mutations in chromatin-remodelling and Wnt pathway genes and few KRAS mutations.

Right hemicolectomy with lymphadenectomy is recommended for almost all patients because nodal spread is common even with small tumours, and bilateral oophorectomy is considered in postmenopausal women. Adjuvant chemotherapy with FOLFOX or CAPOX is used for node-positive or grade 2 to 3 disease by analogy with colon cancer, and peritoneal metastases are treated with cytoreductive surgery and HIPEC in selected patients, with outcomes between those of low-grade pseudomyxoma peritonei and signet ring cell carcinoma. Metastatic disease receives colorectal chemotherapy regimens; platinum-etoposide, the treatment for neuroendocrine carcinoma, is not appropriate. Because the disease is so rare, care is best delivered in a peritoneal surface oncology centre with pathology review.

## Fields

- Kind: Cancer
- Last checked: 2026-09-18
- Also known as: Goblet cell carcinoid (obsolete); Adenocarcinoma ex goblet cell carcinoid (obsolete); GCA; Crypt cell carcinoma; Mixed adenoneuroendocrine carcinoma of the appendix (obsolete)
- Tags: subtype-page; gastrointestinal
- Group: gastrointestinal
- Burden: A rare tumour almost unique to the appendix, typically found in people in their fifties and sixties after appendicectomy; behaviour ranges from indolent to aggressive according to grade.
- Subtypes: Grade 1 goblet cell adenocarcinoma (tubular or clustered pattern, indolent); Grade 2 goblet cell adenocarcinoma; Grade 3 goblet cell adenocarcinoma (poorly cohesive or signet ring pattern, aggressive); Goblet cell adenocarcinoma confined to the appendix (right hemicolectomy); Goblet cell adenocarcinoma with peritoneal or ovarian metastases (cytoreduction and HIPEC in selected patients)
- Biomarkers: WHO 2019 grade (proportion of low-grade tubular pattern); TNM stage after right hemicolectomy; Synaptophysin and chromogranin (focal) with mucin stains (diagnosis); Somatostatin receptor status (negative; not a target); CEA, CA 19-9 and CA-125 for surveillance; Peritoneal cancer index where peritoneal disease is present

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/goblet-cell-adenocarcinoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#overview [3 state-of-the-art points]
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#what-it-is [5 subtypes]
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#finding-it [6 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#treating-it [4 settings, 4 decisions with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#evidence [2 key papers, 4 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#science
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/goblet-cell-adenocarcinoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/goblet-cell-adenocarcinoma/#living-with-it [16 questions, 2 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/goblet-cell-adenocarcinoma/coming/ [3 medicines, 4 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/goblet-cell-adenocarcinoma/data/ [16 connected records]

## Standard of care

- Diagnosis: Expert pathology review with WHO 2019 grading; CT of chest, abdomen and pelvis; colonoscopy; tumour markers; no somatostatin receptor imaging is needed. ([Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [CT (computed tomography)](https://onco.cc/technologies/ct/), [Endoscopy (EGD, EUS, ERCP)](https://onco.cc/terms/endoscopy/))
- Localised disease: Right hemicolectomy with lymphadenectomy for all grades; oophorectomy considered in postmenopausal women; adjuvant FOLFOX or CAPOX for node-positive or grade 2 to 3 disease. ([Colectomy](https://onco.cc/terms/colectomy/), [Lymphadenectomy (lymph node dissection)](https://onco.cc/terms/lymphadenectomy/), [FOLFOX (5-FU, leucovorin, oxaliplatin)](https://onco.cc/drugs/folfox/), [CAPOX (capecitabine, oxaliplatin)](https://onco.cc/drugs/capox/))
- Peritoneal metastases: Cytoreductive surgery with HIPEC in fit patients with limited disease, with perioperative systemic chemotherapy. ([HIPEC / PIPAC (intraperitoneal chemotherapy)](https://onco.cc/technologies/hipec/), [HIPEC (hyperthermic intraperitoneal chemotherapy)](https://onco.cc/terms/hipec-procedure/), [Peritoneal metastasis](https://onco.cc/terms/peritoneal-metastasis/), [FOLFOX (5-FU, leucovorin, oxaliplatin)](https://onco.cc/drugs/folfox/))
- Metastatic disease: FOLFOX or CAPOX, then FOLFIRI, as for colorectal adenocarcinoma; somatostatin analogues and platinum-etoposide are not used. ([FOLFOX (5-FU, leucovorin, oxaliplatin)](https://onco.cc/drugs/folfox/), [CAPOX (capecitabine, oxaliplatin)](https://onco.cc/drugs/capox/), [FOLFIRI (5-FU, leucovorin, irinotecan)](https://onco.cc/drugs/folfiri/))

## State of the art

- The 2019 WHO reclassification ended decades of confusion with neuroendocrine tumours and aligned treatment with adenocarcinoma.
- Right hemicolectomy is standard because nodal spread is common.
- Grade now predicts outcome and steers adjuvant therapy.

## Open problems

- No prospective trial has ever been conducted; all treatment is extrapolated.
- The benefit of adjuvant chemotherapy in grade 1 node-negative disease is unknown.
- Whether HIPEC helps in goblet cell adenocarcinoma specifically is defined only by small series.
- Diagnosis is delayed by the absence of a mass and by old terminology.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Goblet_cell_carcinoid
- WHO Classification of Tumours: Digestive System (2019): https://publications.iarc.who.int/579
- Wikipedia: https://en.wikipedia.org/wiki/Goblet_cell_carcinoid

## Connected records

- cancers: [Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell)](https://onco.cc/cancers/appendiceal-adenocarcinoma/), [Appendiceal cancer and pseudomyxoma peritonei](https://onco.cc/cancers/appendiceal/), [Low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei](https://onco.cc/cancers/low-grade-appendiceal-mucinous-neoplasm/), [Small intestinal neuroendocrine tumours](https://onco.cc/cancers/small-intestinal-net/)
- technologies: [CT (computed tomography)](https://onco.cc/technologies/ct/), [Cytotoxic chemotherapy](https://onco.cc/technologies/cytotoxic-chemotherapy/), [HIPEC / PIPAC (intraperitoneal chemotherapy)](https://onco.cc/technologies/hipec/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/)
- drugs: [CAPOX (capecitabine, oxaliplatin)](https://onco.cc/drugs/capox/), [FOLFIRI (5-FU, leucovorin, irinotecan)](https://onco.cc/drugs/folfiri/), [FOLFOX (5-FU, leucovorin, oxaliplatin)](https://onco.cc/drugs/folfox/)
- terms: [Colectomy](https://onco.cc/terms/colectomy/), [Endoscopy (EGD, EUS, ERCP)](https://onco.cc/terms/endoscopy/), [HIPEC (hyperthermic intraperitoneal chemotherapy)](https://onco.cc/terms/hipec-procedure/), [Lymphadenectomy (lymph node dissection)](https://onco.cc/terms/lymphadenectomy/), [Peritoneal metastasis](https://onco.cc/terms/peritoneal-metastasis/)
- key papers: [The 2019 WHO classification of tumours of the digestive system](https://onco.cc/key-papers/paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020/), [The Chicago Consensus on peritoneal surface malignancies: management of appendiceal neoplasms](https://onco.cc/key-papers/paper-chicago-consensus-appendiceal-neoplasms-cancer-2020/)

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JSON: https://onco.cc/api/v1/entities/goblet-cell-adenocarcinoma.json