{"entity":{"id":"goblet-cell-adenocarcinoma","kind":"cancer","name":"Goblet cell adenocarcinoma of the appendix","aka":["Goblet cell carcinoid (obsolete)","Adenocarcinoma ex goblet cell carcinoid (obsolete)","GCA","Crypt cell carcinoma","Mixed adenoneuroendocrine carcinoma of the appendix (obsolete)"],"tldr":"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.\n\nRight 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.","asOf":"2026-09-18","wikipedia":"https://en.wikipedia.org/wiki/Goblet_cell_carcinoid","links":[{"label":"WHO Classification of Tumours: Digestive System (2019)","url":"https://publications.iarc.who.int/579"},{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Goblet_cell_carcinoid"}],"tags":["subtype-page","gastrointestinal"],"related":["appendiceal-adenocarcinoma","low-grade-appendiceal-mucinous-neoplasm","appendiceal","small-intestinal-net"],"cancers":[],"sections":[],"technologies":["hipec","histopathology-ihc","ct","cytotoxic-chemotherapy"],"targets":[],"drugs":["folfox","capox","folfiri"],"companies":[],"institutions":[],"pathways":[],"terms":["colectomy","lymphadenectomy","hipec-procedure","peritoneal-metastasis","endoscopy"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":["paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020","paper-chicago-consensus-appendiceal-neoplasms-cancer-2020"],"journals":[],"dependsOn":[],"notes":[],"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"],"standardOfCare":[{"setting":"Diagnosis","approach":"Expert pathology review with WHO 2019 grading; CT of chest, abdomen and pelvis; colonoscopy; tumour markers; no somatostatin receptor imaging is needed.","refs":["histopathology-ihc","ct","endoscopy"],"guideline":{"version":"NCCN Guidelines: Colon Cancer (appendiceal section)","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1428"}},{"setting":"Localised disease","approach":"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.","refs":["colectomy","lymphadenectomy","folfox","capox"],"guideline":{"version":"NCCN Guidelines: Colon Cancer (appendiceal section)","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1428"}},{"setting":"Peritoneal metastases","approach":"Cytoreductive surgery with HIPEC in fit patients with limited disease, with perioperative systemic chemotherapy.","refs":["hipec","hipec-procedure","peritoneal-metastasis","folfox"],"guideline":{"version":"NCCN Guidelines: Colon Cancer (appendiceal section)","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1428"}},{"setting":"Metastatic disease","approach":"FOLFOX or CAPOX, then FOLFIRI, as for colorectal adenocarcinoma; somatostatin analogues and platinum-etoposide are not used.","refs":["folfox","capox","folfiri"],"guideline":{"version":"NCCN Guidelines: Colon Cancer (appendiceal section)","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1428"}}],"stateOfArt":["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."],"history":[{"year":1974,"title":"Goblet cell carcinoid of the appendix described as a distinct tumour","refs":[]},{"year":2008,"title":"Tang classification separates typical goblet cell carcinoid from adenocarcinoma ex goblet cell carcinoid","refs":[]},{"year":2019,"title":"WHO renames the tumour goblet cell adenocarcinoma with a three-tier grade","refs":[]},{"year":2021,"title":"Molecular studies show a genotype distinct from appendiceal adenocarcinoma and neuroendocrine tumours","refs":[]}],"pipeline":["folfox","hipec"],"openProblems":["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."],"parent":"appendiceal"},"route":"/cancers/goblet-cell-adenocarcinoma/","neighbours":{"cancer":[{"id":"appendiceal-adenocarcinoma","kind":"cancer","name":"Appendiceal adenocarcinoma (mucinous and non-mucinous, including signet ring cell)","route":"/cancers/appendiceal-adenocarcinoma/"},{"id":"appendiceal","kind":"cancer","name":"Appendiceal cancer and pseudomyxoma peritonei","route":"/cancers/appendiceal/"},{"id":"low-grade-appendiceal-mucinous-neoplasm","kind":"cancer","name":"Low-grade appendiceal mucinous neoplasm and pseudomyxoma peritonei","route":"/cancers/low-grade-appendiceal-mucinous-neoplasm/"},{"id":"small-intestinal-net","kind":"cancer","name":"Small intestinal neuroendocrine tumours","route":"/cancers/small-intestinal-net/"}],"technology":[{"id":"ct","kind":"technology","name":"CT (computed tomography)","route":"/technologies/ct/"},{"id":"cytotoxic-chemotherapy","kind":"technology","name":"Cytotoxic chemotherapy","route":"/technologies/cytotoxic-chemotherapy/"},{"id":"hipec","kind":"technology","name":"HIPEC / PIPAC (intraperitoneal chemotherapy)","route":"/technologies/hipec/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"drug":[{"id":"capox","kind":"drug","name":"CAPOX (capecitabine, oxaliplatin)","route":"/drugs/capox/"},{"id":"folfiri","kind":"drug","name":"FOLFIRI (5-FU, leucovorin, irinotecan)","route":"/drugs/folfiri/"},{"id":"folfox","kind":"drug","name":"FOLFOX (5-FU, leucovorin, oxaliplatin)","route":"/drugs/folfox/"}],"term":[{"id":"colectomy","kind":"term","name":"Colectomy","route":"/terms/colectomy/"},{"id":"endoscopy","kind":"term","name":"Endoscopy (EGD, EUS, ERCP)","route":"/terms/endoscopy/"},{"id":"hipec-procedure","kind":"term","name":"HIPEC (hyperthermic intraperitoneal chemotherapy)","route":"/terms/hipec-procedure/"},{"id":"lymphadenectomy","kind":"term","name":"Lymphadenectomy (lymph node dissection)","route":"/terms/lymphadenectomy/"},{"id":"peritoneal-metastasis","kind":"term","name":"Peritoneal metastasis","route":"/terms/peritoneal-metastasis/"}],"paper":[{"id":"paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020","kind":"paper","name":"The 2019 WHO classification of tumours of the digestive system","route":"/key-papers/paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020/"},{"id":"paper-chicago-consensus-appendiceal-neoplasms-cancer-2020","kind":"paper","name":"The Chicago Consensus on peritoneal surface malignancies: management of appendiceal neoplasms","route":"/key-papers/paper-chicago-consensus-appendiceal-neoplasms-cancer-2020/"}]}}