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Medullary carcinoma is a very rare form of bowel cancer in which sheets of poorly formed cells are packed with immune cells; almost all cases are mismatch-repair deficient and it occurs mostly in older women on the right side. Despite its ugly appearance it does at least as well as ordinary bowel cancer, and because of its immune features it is a natural candidate for immunotherapy.
The WHO classification lists medullary carcinoma as a distinct colorectal adenocarcinoma type with solid sheets of poorly differentiated cells, vesicular nuclei and a prominent intraepithelial lymphocytic infiltrate, nearly always mismatch-repair deficient (Nagtegaal 2020). In SEER it was extremely rare (5 to 8 per 10,000 colon cancers), most common in the proximal colon, twice as common in women who presented at a lower stage with a trend to favourable prognosis, and no case was reliably identified in the rectum or appendix (International Journal of Oncology 2010). Loss of MLH1 and PMS2 was found in more than 80 percent of 18 cases, most were negative for CK7, CK20 and CDX2, and cadherin-17 and SATB2 were expressed in 89 percent, which matters when it presents as a carcinoma of unknown primary (Archives of Pathology 2014). In 105 cases the microenvironment showed strong interferon-gamma-induced immunoregulatory gene expression, including IDO-1, distinct from other microsatellite-unstable carcinomas (Modern Pathology 2016).
How it differs from its parent: it is the extreme of the mismatch-repair deficient, right-sided, immune-rich colon cancer that the MSI-high page describes, with a distinctive marker profile (CDX2-negative) that can mislead the pathologist.
How common: 5 to 8 per 10,000 colon cancers (International Journal of Oncology 2010).
Treatment: as the colorectal and MSI-high pages by stage, with checkpoint inhibitors for mismatch-repair deficient disease; there is no trial in the type.
About 5 to 8 cases for every 10,000 colon cancers, a mean annual incidence of 3.47 per 10 million population in SEER 1973 to 2006; mean age 69.3, twice as common in women, 74 percent in the proximal colon (International Journal of Oncology 2010).
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), Mucinous adenocarcinoma of the colon and rectum, Signet ring cell carcinoma of the colon and rectum, 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), Goblet cell adenocarcinoma of the appendix, Localised small bowel adenocarcinoma (stage I to III, resected), Advanced and metastatic small bowel adenocarcinoma
Treated as MSI-high colorectal cancer by stage, with checkpoint inhibitors for mismatch-repair deficient disease.
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Query for this cancer: (TITLE:"Medullary carcinoma of the colon" OR ABSTRACT:"Medullary carcinoma of the colon" OR TITLE:"Medullary carcinoma of the large intestine" OR ABSTRACT:"Medullary carcinoma of the large intestine" OR TITLE:"Medullary colorectal carcinoma" OR ABSTRACT:"Medullary colorectal carcinoma" OR TITLE:"Medullary adenocarcinoma of the colon" OR ABSTRACT:"Medullary adenocarcinoma of the colon") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Medullary carcinoma of the colon, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Persistent headache with extreme tiredness, nausea, dizziness on standing or low blood pressure. Vomiting, severe weakness or collapse is adrenal crisis.
No pharmacokinetic interactions expected (antibody). See the irAE guide for toxicity management.
Immunotherapy can attack hormone-producing glands: most often the thyroid (usually ending in an under-active thyroid needing lifelong tablets), and less often the pituitary (hypophysitis) or adrenal glands, which can be life-threatening if missed.
See all on the product pages:NivolumabPembrolizumab·Printable cards in the navigator
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