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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis | Expert pathology review with WHO 2019 grading; CT of chest, abdomen and pelvis; colonoscopy; tumour markers; no somatostatin receptor imaging is needed. | not mapped |
| 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. | not mapped |
| Peritoneal metastases | Cytoreductive surgery with HIPEC in fit patients with limited disease, with perioperative systemic chemotherapy. | not mapped |
| Metastatic disease | FOLFOX or CAPOX, then FOLFIRI, as for colorectal adenocarcinoma; somatostatin analogues and platinum-etoposide are not used. | not mapped |