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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.
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.
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.
Right-sided tumours behave differently from left-sided and rectal ones; the colon drains along its mesenteric vessels, the rectum into the mesorectum and pelvic side wall.
Same organ: Colon cancer (adenocarcinoma of the colon), Micropapillary adenocarcinoma of the colon and rectum, Adenoma-like adenocarcinoma of the colon and rectum, Lynch syndrome-associated colorectal cancer, Familial adenomatous polyposis-associated colorectal cancer, Mucinous adenocarcinoma of the colon and rectum, Signet ring cell carcinoma of the colon and rectum, Medullary carcinoma of the colon, Serrated adenocarcinoma of the colon and rectum, Peritoneal mesothelioma, Colorectal cancer, Rectal cancer, Mismatch-repair deficient (MSI-high) colorectal cancer, BRAF V600E-mutant colorectal cancer, HER2-amplified colorectal cancer, KRAS G12C-mutant colorectal cancer, Early-onset colorectal cancer (under 50), Anal cancer (squamous cell carcinoma), Appendiceal cancer and pseudomyxoma peritonei, Small intestine cancer (small bowel adenocarcinoma), Small intestinal neuroendocrine tumours, Anal high-grade squamous intraepithelial lesions (precursor), Localised anal squamous cell carcinoma (stage I to III), Metastatic and recurrent anal squamous cell carcinoma, Low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei, Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell), Localised small bowel adenocarcinoma (stage I to III, resected), Advanced and metastatic small bowel adenocarcinoma
Expert pathology review with WHO 2019 grading; CT of chest, abdomen and pelvis; colonoscopy; tumour markers; no somatostatin receptor imaging is needed.
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.
Cytoreductive surgery with HIPEC in fit patients with limited disease, with perioperative systemic chemotherapy.
FOLFOX or CAPOX, then FOLFIRI, as for colorectal adenocarcinoma; somatostatin analogues and platinum-etoposide are not used.
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The names on an appendix or small bowel pathology report, and hence which OnCo subtype page applies, follow this classification.
The standard-of-care rows on the appendiceal pages, particularly who is referred for cytoreductive surgery and who receives chemotherapy, follow this consensus and the NCCN appendiceal section.
Query for this cancer: (TITLE:"Goblet cell adenocarcinoma of the appendix" OR ABSTRACT:"Goblet cell adenocarcinoma of the appendix" OR TITLE:"Goblet cell carcinoid obsolete" OR ABSTRACT:"Goblet cell carcinoid obsolete" OR TITLE:"Adenocarcinoma ex goblet cell carcinoid obsolete" OR ABSTRACT:"Adenocarcinoma ex goblet cell carcinoid obsolete" OR TITLE:"GCA" OR ABSTRACT:"GCA" OR TITLE:"Crypt cell carcinoma" OR ABSTRACT:"Crypt cell carcinoma" OR TITLE:"Mixed adenoneuroendocrine carcinoma of the appendix obsolete" OR ABSTRACT:"Mixed adenoneuroendocrine carcinoma of the appendix obsolete") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Goblet cell adenocarcinoma of the appendix, not a curated reading list.
No targets or pathways are linked to this cancer yet. Browse the gene hub →
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
The bowel cancer regimens share low blood counts, tiredness, sickness and a sore mouth. Oxaliplatin adds cold-triggered tingling, irinotecan adds early and late diarrhoea, capecitabine adds hand-foot syndrome and needs a DPD test first, and cetuximab or panitumumab add an acne-like rash and low magnesium. The rule for all of them: ring the 24-hour number rather than wait.
See all on the product pages:CAPOX (capecitabine, oxaliplatin)FOLFIRI (5-FU, leucovorin, irinotecan)FOLFOX (5-FU, leucovorin, oxaliplatin)·Printable cards in the navigator
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