{"entity":{"id":"pancreatic-undifferentiated-carcinoma-ogc","kind":"cancer","name":"Undifferentiated carcinoma of the pancreas with osteoclast-like giant cells","aka":["Undifferentiated carcinoma with osteoclast-like giant cells (1.4 percent of resected pancreatic cancers; five-year survival 59 percent)","Osteoclastic giant cell tumour of the pancreas","UC-OGC","Undifferentiated carcinoma with osteoclastic giant cells","Giant cell carcinoma of the pancreas"],"tldr":"Undifferentiated carcinoma with osteoclast-like giant cells is a rare form of pancreatic cancer studded with large bone-eating-type cells that are not themselves cancerous. It grows as a big, well-circumscribed mass, often within a cyst, spreads to nerves and lymph nodes far less often than ordinary pancreatic cancer, and about six in ten patients are alive five years after surgery.","summary":"What it is. A WHO variant of ductal adenocarcinoma (Nagtegaal 2020) in which undifferentiated, often spindled or pleomorphic carcinoma cells are mixed with non-neoplastic osteoclast-like giant cells that express the histiocytic marker CD68; the background spindle and giant carcinoma cells often show p53 and often lack cytokeratin, and osteoid was seen in 12 of 38 tumours (Muraki 2016). A 2021 review sets out its pathology and molecular features and the questions it raises (Luchini 2021). Undifferentiated (anaplastic) carcinoma without giant cells is a separate, aggressive variant that this record does not describe.\n\nHow it differs from its parent. In 38 resected cases against 725 resected ductal adenocarcinomas, the tumours were larger (mean 5.3 against 3.2 cm), nodular with pushing borders, arose in a mucinous cystic or IPMN-type lesion in 8 cases and showed prominent intraductal or intracystic growth in 61 percent; 76 percent also had an invasive ductal or tubular component. Despite their size, perineural invasion (31.6 against 85.5 percent) and nodal metastasis (22.6 against 64.0 percent) were uncommon, and five-year survival was 59.1 percent against 15.7 percent (Muraki 2016).\n\nHow common it is. 1.4 percent of the resected pancreatic cancers reviewed at one centre; no population count is published.\n\nHow it is treated. Resection, with adjuvant chemotherapy by extrapolation from ductal adenocarcinoma; there is no trial. The more protracted course than the literature had assumed is the main lesson of the 2016 series, and it argues for offering surgery even to large tumours.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Giant-cell_tumor_of_bone","links":[{"label":"Muraki, Am J Surg Pathol 2016: undifferentiated carcinoma with osteoclastic giant cells of the pancreas, 38 cases","url":"https://doi.org/10.1097/pas.0000000000000689"},{"label":"Luchini, Virchows Arch 2021: undifferentiated carcinoma of the pancreas with osteoclast-like giant cells, a review","url":"https://doi.org/10.1007/s00428-021-03021-9"},{"label":"Nagtegaal, Histopathology 2020: the 2019 WHO classification of tumours of the digestive system","url":"https://doi.org/10.1111/his.13975"}],"tags":["gi","pancreatic","subtype-page"],"related":["pancreatic","pancreatic-adenosquamous-carcinoma","mcn-associated-carcinoma","ipmn-associated-carcinoma","undifferentiated-pleomorphic-sarcoma"],"cancers":[],"sections":[],"technologies":["cytotoxic-chemotherapy","histopathology-ihc"],"targets":["kras","tp53"],"drugs":["folfirinox","gemcitabine-nab-paclitaxel"],"companies":[],"institutions":[],"pathways":["ras-mapk","pancreatic-cancer-signalling","emt"],"terms":["tumour-grade","whipple","resection-margins"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"gastrointestinal","burden":"1.4 percent of systematically reviewed resected pancreatic cancers in the largest series (38 of about 2,700 cases); slight female predominance (62.9 percent) and a mean age of 57.9 against 65.0 for ordinary ductal adenocarcinoma.","subtypes":["Undifferentiated carcinoma with osteoclast-like giant cells and an invasive ductal (PDAC) component (76 percent of cases)","Undifferentiated carcinoma with osteoclast-like giant cells arising in a mucinous cystic neoplasm or IPMN","Undifferentiated (anaplastic) carcinoma without giant cells (aggressive; treated as ductal adenocarcinoma)"],"biomarkers":["CD68-positive osteoclast-like giant cells (non-neoplastic) among cytokeratin-poor, p53-positive carcinoma cells","Associated ductal adenocarcinoma component or cystic precursor on histology","KRAS as in ductal adenocarcinoma"],"standardOfCare":[{"setting":"Resectable","approach":"Resection even of large tumours, given the low rates of nodal and perineural spread and the 59 percent five-year survival in the resected series; adjuvant chemotherapy by extrapolation from ductal adenocarcinoma.","refs":["whipple","distal-pancreatectomy","folfirinox"],"guideline":{"version":"NCCN Guidelines: Pancreatic Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1455"}},{"setting":"Advanced","approach":"Treated as pancreatic ductal adenocarcinoma: resection with adjuvant chemotherapy when removable, the chemotherapy rows of the parent page when not; the parent record carries the trials.","refs":["folfirinox","gemcitabine-nab-paclitaxel"],"guideline":{"version":"NCCN Guidelines: Pancreatic Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1455"}}],"stateOfArt":["The 2016 series of 38 cases overturned the assumption that this variant behaves like ductal adenocarcinoma."],"history":[{"year":2016,"title":"Muraki and colleagues report a more protracted course in 38 resected cases","refs":["tumour-grade"]},{"year":2021,"title":"Review of pathology and molecular features (Luchini)","refs":["histopathology-ihc"]}],"pipeline":[],"openProblems":["No trial; adjuvant treatment is extrapolated.","Whether the immune-rich microenvironment makes it a candidate for immunotherapy is unproven."],"parent":"pancreatic"},"route":"/cancers/pancreatic-undifferentiated-carcinoma-ogc/","neighbours":{"cancer":[{"id":"pancreatic-adenosquamous-carcinoma","kind":"cancer","name":"Adenosquamous carcinoma of the pancreas","route":"/cancers/pancreatic-adenosquamous-carcinoma/"},{"id":"ipmn-associated-carcinoma","kind":"cancer","name":"Invasive carcinoma arising in an intraductal papillary mucinous neoplasm (IPMN-associated carcinoma)","route":"/cancers/ipmn-associated-carcinoma/"},{"id":"mcn-associated-carcinoma","kind":"cancer","name":"Mucinous cystic neoplasm of the pancreas with associated invasive carcinoma (MCN-associated carcinoma)","route":"/cancers/mcn-associated-carcinoma/"},{"id":"pancreatic","kind":"cancer","name":"Pancreatic ductal adenocarcinoma","route":"/cancers/pancreatic/"},{"id":"undifferentiated-pleomorphic-sarcoma","kind":"cancer","name":"Undifferentiated pleomorphic sarcoma (UPS)","route":"/cancers/undifferentiated-pleomorphic-sarcoma/"}],"technology":[{"id":"cytotoxic-chemotherapy","kind":"technology","name":"Cytotoxic chemotherapy","route":"/technologies/cytotoxic-chemotherapy/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"target":[{"id":"kras","kind":"target","name":"KRAS","route":"/targets/kras/"},{"id":"tp53","kind":"target","name":"TP53","route":"/targets/tp53/"}],"drug":[{"id":"folfirinox","kind":"drug","name":"FOLFIRINOX / mFOLFIRINOX","route":"/drugs/folfirinox/"},{"id":"gemcitabine-nab-paclitaxel","kind":"drug","name":"Gemcitabine + nab-paclitaxel","route":"/drugs/gemcitabine-nab-paclitaxel/"}],"pathway":[{"id":"emt","kind":"pathway","name":"Epithelial-mesenchymal transition & drug efflux","route":"/pathways/emt/"},{"id":"pancreatic-cancer-signalling","kind":"pathway","name":"Pancreatic cancer (KEGG map)","route":"/pathways/pancreatic-cancer-signalling/"},{"id":"ras-mapk","kind":"pathway","name":"RAS / RAF / MEK / ERK (MAPK)","route":"/pathways/ras-mapk/"}],"term":[{"id":"distal-pancreatectomy","kind":"term","name":"Distal pancreatectomy (removal of the body and tail of the pancreas, usually with the spleen)","route":"/terms/distal-pancreatectomy/"},{"id":"tumour-grade","kind":"term","name":"Grade","route":"/terms/tumour-grade/"},{"id":"resection-margins","kind":"term","name":"Resection margins (R0 / R1 / R2)","route":"/terms/resection-margins/"},{"id":"whipple","kind":"term","name":"Whipple procedure (pancreaticoduodenectomy)","route":"/terms/whipple/"}]}}