216:
carcinomatosis, PMCA) with a third category reserved for cases with intermediate features. In this classification system, cases of DPAM were characterized by peritoneal lesions composed of abundant extracellular mucin containing scant simple to focally proliferative mucinous epithelium with little cytologic atypia or mitotic activity (in other words, most cells looked fairly normal and there was no evidence of mitosis which would indicate that cells were rapidly dividing), with or without an associated appendiceal mucinous adenoma. Cases of PMCA were characterized by peritoneal lesions composed of more abundant mucinous epithelium with the architectural and cytologic features of carcinoma (irregular cells, evidence that cells were rapidly dividing, and other criteria), with or without an associated primary mucinous adenocarcinoma. Bradley et al. (2007) argued that continued use of non-malignant terms, i.e., adenoma, for those frequent cases with low-grade features (such as DPAM), is misleading because pseudomyxoma peritonei is a disease state that results from invasion of the abdominal cavity by cells with uncontrolled growth. Bradley states that an adenoma, by definition, is a tumor confined to the appendiceal mucosa with absolutely no evidence of invasion beyond the muscularis mucosae.
207:
for the condition). Due to the rarity of this disease, it is important to obtain an accurate diagnosis so that appropriate treatment may be obtained from a gastrointestinal cancer surgeon. Diagnostic tests may include CT scans, examination of tissue samples obtained through laparoscopy, and the evaluation of tumor markers. In most cases a colonoscopy is unsuitable as a diagnostic tool because in most cases appendix cancer invades the abdominal cavity but not the colon (however, spread inside the colon is occasionally reported). PET scans may be used to evaluate high-grade mucinous adenocarcinoma, but this test is not reliable for detecting low-grade tumors because those do not take up the dye which shows up on scans. New MRI procedures are being developed for disease monitoring, but standard MRIs are not typically used as a diagnostic tool. Diagnosis is confirmed through pathology.
224:, 2nd ed.) also use the term mucinous adenocarcinoma when referring to low-grade, well-differentiated tumors lacking high-grade features. Low-grade mucinous adenocarcinoma is used by the American Joint Committee on Cancer and World Health Organization and is nearly or completely synonymous with the DPAM designation. For low-grade mucinous adenocarcinoma, disease may be designated as "benign" because tumors do not invade deeply into tissue and rarely metastasize to parenchyma of organs; this designation may be misleading and confusing to the layperson because pseudomyxoma peritonei is not a harmless condition, fatal if untreated. High-grade or poorly differentiated mucinous adenocarcinoma has a generally poorer prognosis, though surgical treatment with heated intra-peritoneal chemotherapy (HIPEC) is yielding promising outcomes (see surgical treatment).
329:) may be infused directly into the abdominal cavity after cytoreductive surgery (surgery removing all visible disease to kill remaining microscopic cancerous tumors and free floating cells). The heated chemotherapy (HIPEC) is perfused throughout the abdominal cavity for an hour or two as the last step in the surgery, or ports are installed to allow circulation and/or drainage of the chemicals for one to five days after surgery, known as early postoperative intraperitoneal chemotherapy (EPIC). EPIC may be given in multiple cycles for several months after surgery.
279:, performed by surgical oncologists who specialize in treating PMP. Some surgeons also apply early post-operative intraperitonial chemotherapy (EPIC), adjunct to surgical cytoreduction and HIPEC. In situations where surgery is not required immediately, patients can be monitored via CT scans, tumor marker laboratory tests, and physical symptoms, to determine when, and if, surgery is warranted. Although some surgical procedures may be rather extensive, patients can and do recover from surgery, and the majority of these patients can and do live productive lives.
56:
353:
per million, and the prevalence being 22 persons per million. It is slightly more common in women than men (male:female ratio of approximately 1:1.3,), although the actual ratio is difficult to identify due to potential misdiagnoses and possibly inclusion bias in reported studies. The median age at presentation is typically about 50 years with a range of 20–25 years, but PMP may strike persons of any age.
29:
1100:
Kusamura S, Baratti D, Hutanu I, Gavazzi C, Morelli D, Iusco DR, et al. (August 2015). "The role of baseline inflammatory-based scores and serum tumor markers to risk stratify pseudomyxoma peritonei patients treated with cytoreduction (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC)".
352:
The overall incidence was previously estimated at 0.5 to 1 cases per 100,000 people per year. Recent research in Europe indicates that the previous estimate of 1-2 persons per million may be underestimating the actual rate by approximately half, with the real incidence being approximately 3.2 persons
206:
This disease is often discovered during surgery for other conditions, e.g., hernia repair, following which an experienced pathologist can confirm the diagnosis. Advanced stages may present as tumors palpable on the abdomen or distention of the belly ("jelly belly" is sometimes used as a slang term
149:
and most commonly from these cells in the appendix. The K-Ras and p53 genes may be involved in the oncogenesis. It may be diagnosed with a range of conditions. While the majority of these cases are associated with appendiceal carcinomas, other conditions may also be found, including disseminated
336:
treatment. Due to the increased availability of new chemotherapies developed for colorectal cancer patients, some patients have experienced stability in tumor growth with systemic chemotherapy. Systemic chemotherapy is generally reserved for patients with advanced disease, recurrent disease, or
219:
The term mucinous adenocarcinoma is used in different contexts depending on the reference material used by the pathologist for disease classification. For example, neoplasms characterized by high-grade features, invasive glands and or signet ring cells, are termed adenocarcinoma in pathology
625:
Ronnett BM, Zahn CM, Kurman RJ, Kass ME, Sugarbaker PH, Shmookler BM (December 1995). "Disseminated peritoneal adenomucinosis and peritoneal mucinous carcinomatosis. A clinicopathologic analysis of 109 cases with emphasis on distinguishing pathologic features, site of origin, prognosis, and
215:
There is substantial debate regarding histopathologic classification of pseudomyxoma peritonei. In 1995, Ronnett et al. proposed separating pseudomyxoma peritonei cases into two diagnostic categories: adenoma (disseminated peritoneal adenomucinosis, DPAM) or carcinoma (peritoneal mucinous
1297:
1282:
954:
Culliford AT, Brooks AD, Sharma S, Saltz LB, Schwartz GK, O'Reilly EM, et al. (December 2001). "Surgical debulking and intraperitoneal chemotherapy for established peritoneal metastases from colon and appendix cancer".
88:
of tissues and impede digestion or organ function, and if left untreated, the tumors and mucin they produce will fill the abdominal cavity. This will result in compression of organs and will destroy the function of the
108:
have also been implicated, although in most cases ovarian involvement is favored to be a metastasis from an appendiceal or other gastrointestinal source. Disease is typically classified as low- or high-grade (with
365:
in 1842. Werth in 1884 coined the term pseudomyxoma peritonei, describing it in association with a mucinous ovarian tumor. In 1901 Frankel described the first case associated with a cyst of the appendix.
452:"Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy"
854:"Factors influencing long-term survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei originating from appendiceal neoplasms"
1312:
2041:
154:
tumors (mucinous adenocarcinoma, mucinous cystadenoma, and mucinous cystadenocarcinoma), as well as other disease states. Other primary sites that have been reported include
129:
Signs and symptoms of pseudomyxoma peritonei may include abdominal or pelvic pain and/or bloating, distension, digestive disorders, weight changes, increased girth, and
93:, small intestine, stomach, or other organs. Prognosis with treatment in many cases is optimistic, but the disease is lethal if untreated, with death occurring via
1405:
2272:
286:
and any adjacent organs which appear to have tumor seeding. Since the mucus tends to pool at the bottom of the abdominal cavity, it is common to remove the
276:
2297:
2034:
2522:
2382:
752:
2571:
2527:
2027:
1880:
570:
1449:
1398:
282:
In debulking, the surgeon attempts to remove as much tumor as possible. CRS or cytoreductive surgery involves surgical removal of the
2134:
2592:
2430:
1930:
768:
Sugarbaker PH (January 2006). "New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?".
1934:
1767:
2532:
2335:
2292:
2263:
1555:
2243:
1872:
1391:
2561:
2235:
2148:
2058:
1661:
302:. Depending upon the spread of the tumor, other organs might be removed, including but not limited to the gallbladder,
259:
and hyperthermic intraperitoneal chemotherapy (HIPEC, also called intraperitoneal hyperthermic chemotherapy, IPHC) with
2501:
2277:
2253:
1919:
1836:
1469:
2239:
1220:
Sullivan MH, Sugarbaker PH (February 1995). "Treatment of pseudomyxoma peritonei in a geriatric patient population".
2517:
2258:
2112:
1976:
1958:
1868:
1819:
526:
Jacquemin G, Laloux P (April 2005). "Pseudomyxoma peritonei: review on a cluster of peritoneal mucinous diseases".
39:
of an abdomen showing pseudomyxoma peritonei with multiple peritoneal masses (arrow) with "scalloping effect" seen.
2425:
2354:
2050:
1953:
1876:
1713:
1539:
1502:
998:
Marmor S, Portschy PR, Tuttle TM, Virnig BA (April 2015). "The rise in appendiceal cancer incidence: 2000-2009".
491:
Young RH (May 2004). "Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms".
36:
2377:
2231:
1981:
1914:
1849:
1723:
1703:
1670:
1648:
1610:
1320:
251:
Treatment is variable, both due to its rarity and to its frequently slow-growing nature. Treatment ranges from
2566:
2315:
2084:
1459:
306:, and portions of the small intestine and/or stomach. For organs that cannot be removed safely (like the
2597:
2407:
2157:
2067:
1666:
1606:
1371:
688:
661:
Bradley RF, Cortina G, Geisinger KR (October 2007). "Pseudomyxoma peritonei: Review of the controversy".
2004:
1728:
379:
272:
2267:
852:
van Eden WJ, Kok NF, Snaebjornsson P, Jóźwiak K, Woensdregt K, Bottenberg PD, et al. (June 2019).
2412:
1832:
1691:
1628:
1464:
1444:
1968:
1620:
1454:
1301:
362:
341:
113:). When disease presents with low-grade histologic features the cancer rarely spreads through the
55:
2472:
2454:
2394:
2223:
2219:
1801:
1633:
1596:
1474:
1245:
1161:
1023:
980:
725:
551:
1492:
1043:"Estimating the Prevalence of Pseudomyxoma Peritonei in Europe Using a Novel Statistical Method"
2539:
2491:
2482:
2205:
2107:
2076:
1945:
1331:
1237:
1202:
1153:
1118:
1082:
1064:
1015:
972:
936:
885:
834:
785:
748:
717:
700:
Panarelli NC, Yantiss RK (October 2011). "Mucinous neoplasms of the appendix and peritoneum".
643:
593:
543:
508:
473:
432:
110:
44:
601:
2340:
2214:
2129:
2117:
1906:
1824:
1638:
1550:
1229:
1192:
1179:
Fernandes AC, Rocha GR, Oliveira AD, GuimarĂŁes MD, Carvalho SC, Chojniak R (February 2018).
1145:
1136:
Smeenk RM, Bruin SC, van
Velthuysen ML, Verwaal VJ (August 2008). "Pseudomyxoma peritonei".
1110:
1072:
1054:
1007:
964:
926:
916:
875:
865:
824:
816:
777:
709:
670:
635:
585:
535:
500:
463:
422:
412:
333:
252:
114:
2182:
2165:
1924:
1587:
450:
Chua TC, Moran BJ, Sugarbaker PH, Levine EA, Glehen O, Gilly FN, et al. (July 2012).
299:
291:
179:
175:
155:
101:
90:
1675:
344:
and tumor marker laboratory tests are used to monitor patients for disease progression.
2496:
2459:
2389:
2287:
2227:
2170:
2099:
2089:
1601:
1530:
1077:
1042:
1041:
Patrick-Brown TD, Carr NJ, Swanson DM, Larsen S, Mohamed F, Flatmark K (January 2021).
880:
853:
829:
804:
745:
Gynecologic pathology : a volume in the series
Foundations in diagnostic pathology
427:
400:
367:
191:
73:
1306:
931:
904:
781:
271:
The standard of care for mucinous adenocarcinoma with clinical condition PMP involves
2586:
2440:
2122:
639:
260:
1249:
1181:"Pseudomyxoma peritonei in a pediatric patient: A case report and literature review"
1165:
1027:
729:
555:
2402:
1859:
1811:
1755:
1656:
1360:
984:
539:
340:
This disease may recur following surgery and chemotherapy. Periodic post operative
322:
318:
1383:
1325:
1149:
905:"Prognostic indicators in peritoneal carcinomatosis from gastrointestinal cancer"
2467:
2420:
2363:
2307:
1797:
1572:
1484:
1197:
1180:
504:
326:
167:
146:
130:
118:
1336:
1114:
1059:
674:
2553:
1708:
1577:
1567:
1423:
1291:
1011:
968:
713:
283:
2019:
1068:
468:
451:
417:
2449:
2372:
2190:
1760:
1696:
1436:
1432:
1355:
805:"A new standard of care for the management of peritoneal surface malignancy"
256:
171:
1233:
1206:
1157:
1122:
1086:
1019:
976:
940:
889:
838:
789:
721:
597:
547:
512:
477:
436:
1241:
921:
647:
28:
2512:
1366:
820:
195:
151:
94:
85:
49:
1718:
1525:
1497:
870:
287:
163:
100:
This disease is most commonly caused by an appendiceal primary cancer (
81:
1274:
689:
http://surgpathcriteria.stanford.edu/gitumors/appendix-adenocarcinoma/
589:
1995:
1788:
1582:
1415:
1286:
303:
295:
222:
Surgical
Pathology of the GI Tract, Liver, Biliary Tract and Pancreas
187:
159:
1516:
307:
105:
77:
220:
literature. However, some pathologists (e.g., Odze and
Goldblum,
150:
peritoneal adenomucinosis (DPAM), peritoneal carcinomas, several
2509:
183:
142:
2023:
1387:
72:) is a clinical condition caused by cancerous cells (mucinous
337:
disease that has spread to the lymph nodes or distant sites.
332:
Systemic chemotherapy may be administered as additional or
803:
Mohamed F, Cecil T, Moran B, Sugarbaker P (April 2011).
571:"Appendiceal mucinous neoplasms: controversial issues"
310:), the surgeon strips off the tumor from the surface.
97:, bowel obstruction, or other types of complications.
1264:
2552:
2481:
2439:
2362:
2353:
2328:
2306:
2204:
2181:
2156:
2147:
2098:
2075:
2066:
2057:
1994:
1967:
1944:
1905:
1894:
1858:
1810:
1787:
1776:
1741:
1684:
1647:
1619:
1538:
1524:
1515:
1483:
1431:
1422:
1346:
1268:
43:
21:
277:hyperthermic intraperitoneal chemotherapy (HIPEC)
236:Diffuse expression of SATB2, CK20, CDX2, and mCEA
242:High-grade neoplasms may show loss of DPC4 (10%)
702:Archives of Pathology & Laboratory Medicine
578:Archives of Pathology & Laboratory Medicine
2035:
1399:
8:
141:The primary tumor appears to arise from the
909:International Seminars in Surgical Oncology
626:relationship to "pseudomyxoma peritonei"".
2359:
2153:
2072:
2063:
2042:
2028:
2020:
1902:
1784:
1535:
1521:
1428:
1406:
1392:
1384:
1265:
903:Harmon RL, Sugarbaker PH (February 2005).
747:(Second ed.). Elsevier. p. 865.
628:The American Journal of Surgical Pathology
54:
27:
18:
2298:Hereditary nonpolyposis colorectal cancer
1196:
1076:
1058:
930:
920:
879:
869:
828:
467:
426:
416:
370:died of pseudomyxoma peritonei in 1993.
2523:Intraductal papillary mucinous neoplasm
1185:Revista da Associação Médica Brasileira
391:
401:"Management of pseudomyxoma peritonei"
1103:European Journal of Surgical Oncology
7:
2572:Desmoplastic small round cell tumor
1881:Papillary serous cystadenocarcinoma
1000:Journal of Gastrointestinal Surgery
239:Sometimes patchy CK7; negative PAX8
2273:Polymerase proofreading-associated
1450:Combined small-cell lung carcinoma
14:
2135:Hereditary diffuse gastric cancer
405:World Journal of Gastroenterology
2431:Nodular regenerative hyperplasia
640:10.1097/00000478-199512000-00006
584:(6). Pathology Portal: 864–870.
493:Seminars in Diagnostic Pathology
361:The first case was described by
1768:Syringocystadenoma papilliferum
2533:Solid pseudopapillary neoplasm
2336:Gastrointestinal stromal tumor
2293:Familial adenomatous polyposis
1556:Familial adenomatous polyposis
540:10.1080/00015458.2005.11679685
399:Qu ZB, Liu LX (October 2006).
232:Immunohistochemical features:
1:
2341:Krukenberg tumor (metastatic)
1931:Paget's disease of the breast
1873:Pancreatic serous cystadenoma
782:10.1016/S1470-2045(05)70539-8
2562:Primary peritoneal carcinoma
2236:traditional serrated adenoma
1935:Extramammary Paget's disease
1662:Multiple endocrine neoplasia
1222:Journal of Surgical Oncology
1150:10.1067/j.cpsurg.2008.04.003
743:Nucci MR (3 February 2020).
663:Current Diagnostic Pathology
456:Journal of Clinical Oncology
2502:Pancreatic ductal carcinoma
1920:Pancreatic ductal carcinoma
1837:Mucinous cystadenocarcinoma
1470:Transitional cell carcinoma
1198:10.1590/1806-9282.64.02.195
1138:Current Problems in Surgery
1047:Annals of Surgical Oncology
957:Annals of Surgical Oncology
505:10.1053/j.semdp.2004.12.002
2614:
2518:Serous microcystic adenoma
2113:Signet ring cell carcinoma
2051:Digestive system neoplasia
1977:Medullary breast carcinoma
1959:Invasive lobular carcinoma
1869:Ovarian serous cystadenoma
1820:Signet ring cell carcinoma
1115:10.1016/j.ejso.2015.04.005
1060:10.1245/s10434-020-08655-8
675:10.1016/j.cdip.2007.05.013
104:); mucinous tumors of the
2426:Focal nodular hyperplasia
1954:Lobular carcinoma in situ
1877:Serous cystadenocarcinoma
1714:Clear-cell adenocarcinoma
1503:Bartholin gland carcinoma
1414:Glandular and epithelial
1012:10.1007/s11605-014-2726-7
969:10.1007/s10434-001-0787-9
714:10.5858/arpa.2011-0034-RA
321:(typically utilising the
37:Computed tomographic scan
35:
26:
2528:Mucinous cystic neoplasm
2378:Hepatocellular carcinoma
2232:sessile serrated adenoma
1982:Medullary thyroid cancer
1915:Mammary ductal carcinoma
1850:Mucoepidermoid carcinoma
1704:Adenoid cystic carcinoma
1671:Adrenocortical carcinoma
1611:Hepatocellular carcinoma
569:Misdraji J (June 2010).
469:10.1200/JCO.2011.39.7166
418:10.3748/wjg.v12.i38.6124
76:) that produce abundant
2593:Gastrointestinal cancer
2567:Peritoneal mesothelioma
2316:Squamous cell carcinoma
2085:Squamous cell carcinoma
1460:Squamous-cell carcinoma
528:Acta Chirurgica Belgica
2408:Hepatocellular adenoma
2196:Pseudomyxoma peritonei
1843:Pseudomyxoma peritonei
1667:Adrenocortical adenoma
1607:Hepatocellular adenoma
1234:10.1002/jso.2930580210
102:cancer of the appendix
66:Pseudomyxoma peritonei
22:Pseudomyxoma peritonei
2005:Acinic cell carcinoma
1729:Papillary hidradenoma
922:10.1186/1477-7800-2-3
380:Pseudomyxoma Survivor
273:cytoreductive surgery
261:cytoreductive surgery
2413:Cavernous hemangioma
2264:Familial adenomatous
2251:Polyposis syndromes:
1833:Mucinous cystadenoma
1692:Neuroendocrine tumor
1629:Renal cell carcinoma
1465:Basal-cell carcinoma
1445:Small-cell carcinoma
821:10.3747/co.v18i2.663
770:The Lancet. Oncology
228:Immunohistochemistry
1969:Medullary carcinoma
1455:Verrucous carcinoma
298:, and parts of the
84:. The tumors cause
2473:Gallbladder cancer
2455:Cholangiocarcinoma
2395:Liver angiosarcoma
2329:Upper and/or lower
2278:Serrated polyposis
1802:Cystadenocarcinoma
1634:Endometrioid tumor
1597:Cholangiocarcinoma
1485:Complex epithelial
1475:Inverted papilloma
1347:External resources
871:10.1002/bjs5.50134
363:Carl F. Rokitansky
125:Signs and symptoms
2580:
2579:
2548:
2547:
2540:Pancreatoblastoma
2492:exocrine pancreas
2349:
2348:
2324:
2323:
2143:
2142:
2108:Gastric carcinoma
2017:
2016:
2013:
2012:
1990:
1989:
1946:Lobular carcinoma
1890:
1889:
1737:
1736:
1511:
1510:
1381:
1380:
754:978-0-323-35909-2
708:(10): 1261–1268.
634:(12): 1390–1408.
590:10.5858/134.6.864
462:(20): 2449–2456.
411:(38): 6124–6127.
111:signet ring cells
63:
62:
16:Medical condition
2605:
2360:
2259:MUTYH-associated
2244:Cronkhite–Canada
2215:Colorectal polyp
2154:
2130:Linitis plastica
2118:Gastric lymphoma
2073:
2064:
2044:
2037:
2030:
2021:
1937:
1907:Ductal carcinoma
1903:
1883:
1839:
1825:Krukenberg tumor
1785:
1639:Renal oncocytoma
1551:Linitis plastica
1540:Gastrointestinal
1536:
1522:
1429:
1408:
1401:
1394:
1385:
1266:
1254:
1253:
1217:
1211:
1210:
1200:
1176:
1170:
1169:
1133:
1127:
1126:
1109:(8): 1097–1105.
1097:
1091:
1090:
1080:
1062:
1038:
1032:
1031:
995:
989:
988:
951:
945:
944:
934:
924:
900:
894:
893:
883:
873:
849:
843:
842:
832:
809:Current Oncology
800:
794:
793:
765:
759:
758:
740:
734:
733:
697:
691:
685:
679:
678:
658:
652:
651:
622:
616:
615:
613:
612:
606:
600:. Archived from
575:
566:
560:
559:
523:
517:
516:
488:
482:
481:
471:
447:
441:
440:
430:
420:
396:
323:chemotherapeutic
253:watchful waiting
115:lymphatic system
59:
58:
31:
19:
2613:
2612:
2608:
2607:
2606:
2604:
2603:
2602:
2583:
2582:
2581:
2576:
2544:
2477:
2435:
2345:
2320:
2302:
2200:
2177:
2166:Duodenal cancer
2158:Small intestine
2139:
2094:
2053:
2048:
2018:
2009:
1986:
1963:
1940:
1929:
1925:Comedocarcinoma
1898:
1896:
1886:
1867:
1854:
1831:
1806:
1780:
1778:
1772:
1743:
1733:
1680:
1643:
1615:
1588:Somatostatinoma
1531:adenocarcinomas
1529:
1507:
1493:Warthin's tumor
1479:
1418:
1412:
1382:
1377:
1376:
1342:
1341:
1277:
1263:
1258:
1257:
1219:
1218:
1214:
1178:
1177:
1173:
1135:
1134:
1130:
1099:
1098:
1094:
1040:
1039:
1035:
997:
996:
992:
963:(10): 787–795.
953:
952:
948:
902:
901:
897:
851:
850:
846:
802:
801:
797:
767:
766:
762:
755:
742:
741:
737:
699:
698:
694:
686:
682:
660:
659:
655:
624:
623:
619:
610:
608:
604:
573:
568:
567:
563:
525:
524:
520:
490:
489:
485:
449:
448:
444:
398:
397:
393:
388:
376:
359:
350:
316:
300:large intestine
292:fallopian tubes
269:
249:
230:
213:
204:
192:fallopian tubes
180:urinary bladder
176:small intestine
139:
127:
117:or through the
53:
17:
12:
11:
5:
2611:
2609:
2601:
2600:
2595:
2585:
2584:
2578:
2577:
2575:
2574:
2569:
2564:
2558:
2556:
2550:
2549:
2546:
2545:
2543:
2542:
2536:
2535:
2530:
2525:
2520:
2505:
2504:
2499:
2497:Adenocarcinoma
2487:
2485:
2479:
2478:
2476:
2475:
2463:
2462:
2460:Klatskin tumor
2457:
2445:
2443:
2437:
2436:
2434:
2433:
2428:
2416:
2415:
2410:
2398:
2397:
2392:
2390:Hepatoblastoma
2387:
2386:
2385:
2368:
2366:
2357:
2351:
2350:
2347:
2346:
2344:
2343:
2338:
2332:
2330:
2326:
2325:
2322:
2321:
2319:
2318:
2312:
2310:
2304:
2303:
2301:
2300:
2295:
2290:
2288:Adenocarcinoma
2281:
2280:
2275:
2270:
2261:
2256:
2247:
2246:
2210:
2208:
2202:
2201:
2199:
2198:
2193:
2187:
2185:
2179:
2178:
2176:
2175:
2174:
2173:
2171:Adenocarcinoma
2162:
2160:
2151:
2145:
2144:
2141:
2140:
2138:
2137:
2132:
2127:
2126:
2125:
2115:
2110:
2104:
2102:
2096:
2095:
2093:
2092:
2090:Adenocarcinoma
2087:
2081:
2079:
2070:
2061:
2055:
2054:
2049:
2047:
2046:
2039:
2032:
2024:
2015:
2014:
2011:
2010:
2008:
2007:
2001:
1999:
1992:
1991:
1988:
1987:
1985:
1984:
1979:
1973:
1971:
1965:
1964:
1962:
1961:
1956:
1950:
1948:
1942:
1941:
1939:
1938:
1927:
1922:
1917:
1911:
1909:
1900:
1892:
1891:
1888:
1887:
1885:
1884:
1864:
1862:
1856:
1855:
1853:
1852:
1847:
1846:
1845:
1829:
1828:
1827:
1816:
1814:
1808:
1807:
1805:
1804:
1794:
1792:
1782:
1774:
1773:
1771:
1770:
1765:
1764:
1763:
1758:
1747:
1745:
1744:skin appendage
1739:
1738:
1735:
1734:
1732:
1731:
1726:
1721:
1716:
1711:
1706:
1701:
1700:
1699:
1688:
1686:
1685:Other/multiple
1682:
1681:
1679:
1678:
1673:
1664:
1659:
1653:
1651:
1645:
1644:
1642:
1641:
1636:
1631:
1625:
1623:
1617:
1616:
1614:
1613:
1604:
1602:Klatskin tumor
1599:
1593:
1592:
1591:
1590:
1585:
1580:
1575:
1570:
1559:
1558:
1553:
1544:
1542:
1533:
1519:
1513:
1512:
1509:
1508:
1506:
1505:
1500:
1495:
1489:
1487:
1481:
1480:
1478:
1477:
1472:
1467:
1462:
1457:
1452:
1447:
1441:
1439:
1426:
1420:
1419:
1413:
1411:
1410:
1403:
1396:
1388:
1379:
1378:
1375:
1374:
1363:
1351:
1350:
1348:
1344:
1343:
1340:
1339:
1328:
1317:
1309:
1294:
1278:
1273:
1272:
1270:
1269:Classification
1262:
1261:External links
1259:
1256:
1255:
1228:(2): 121–124.
1212:
1191:(2): 195–199.
1171:
1144:(8): 527–575.
1128:
1092:
1053:(1): 252–257.
1033:
1006:(4): 743–750.
990:
946:
895:
864:(3): 376–386.
844:
815:(2): e84–e96.
795:
760:
753:
735:
692:
680:
669:(5): 410–416.
653:
617:
561:
534:(2): 127–133.
518:
499:(2): 134–150.
483:
442:
390:
389:
387:
384:
383:
382:
375:
372:
368:Audrey Hepburn
358:
355:
349:
346:
315:
312:
268:
265:
248:
245:
244:
243:
240:
237:
229:
226:
212:
211:Classification
209:
203:
200:
138:
135:
126:
123:
80:or gelatinous
74:adenocarcinoma
61:
60:
47:
41:
40:
33:
32:
24:
23:
15:
13:
10:
9:
6:
4:
3:
2:
2610:
2599:
2598:Rare diseases
2596:
2594:
2591:
2590:
2588:
2573:
2570:
2568:
2565:
2563:
2560:
2559:
2557:
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2541:
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2515:
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2507:
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2503:
2500:
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2484:
2480:
2474:
2471:
2469:
2465:
2464:
2461:
2458:
2456:
2453:
2451:
2447:
2446:
2444:
2442:
2441:Biliary tract
2438:
2432:
2429:
2427:
2424:
2422:
2418:
2417:
2414:
2411:
2409:
2406:
2404:
2400:
2399:
2396:
2393:
2391:
2388:
2384:
2383:Fibrolamellar
2381:
2380:
2379:
2376:
2374:
2370:
2369:
2367:
2365:
2361:
2358:
2356:
2352:
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2334:
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2331:
2327:
2317:
2314:
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2309:
2305:
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2286:
2283:
2282:
2279:
2276:
2274:
2271:
2269:
2265:
2262:
2260:
2257:
2255:
2252:
2249:
2248:
2245:
2241:
2240:Peutz–Jeghers
2237:
2233:
2229:
2225:
2221:
2218:
2216:
2212:
2211:
2209:
2207:
2203:
2197:
2194:
2192:
2189:
2188:
2186:
2184:
2180:
2172:
2169:
2168:
2167:
2164:
2163:
2161:
2159:
2155:
2152:
2150:
2146:
2136:
2133:
2131:
2128:
2124:
2123:MALT lymphoma
2121:
2120:
2119:
2116:
2114:
2111:
2109:
2106:
2105:
2103:
2101:
2097:
2091:
2088:
2086:
2083:
2082:
2080:
2078:
2074:
2071:
2069:
2065:
2062:
2060:
2056:
2052:
2045:
2040:
2038:
2033:
2031:
2026:
2025:
2022:
2006:
2003:
2002:
2000:
1997:
1993:
1983:
1980:
1978:
1975:
1974:
1972:
1970:
1966:
1960:
1957:
1955:
1952:
1951:
1949:
1947:
1943:
1936:
1932:
1928:
1926:
1923:
1921:
1918:
1916:
1913:
1912:
1910:
1908:
1904:
1901:
1899:and medullary
1893:
1882:
1878:
1874:
1870:
1866:
1865:
1863:
1861:
1857:
1851:
1848:
1844:
1841:
1840:
1838:
1834:
1830:
1826:
1823:
1822:
1821:
1818:
1817:
1815:
1813:
1809:
1803:
1799:
1796:
1795:
1793:
1790:
1786:
1783:
1775:
1769:
1766:
1762:
1759:
1757:
1754:
1753:
1752:
1749:
1748:
1746:
1740:
1730:
1727:
1725:
1722:
1720:
1717:
1715:
1712:
1710:
1707:
1705:
1702:
1698:
1695:
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1693:
1690:
1689:
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1677:
1674:
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1668:
1665:
1663:
1660:
1658:
1655:
1654:
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1650:
1646:
1640:
1637:
1635:
1632:
1630:
1627:
1626:
1624:
1622:
1618:
1612:
1608:
1605:
1603:
1600:
1598:
1595:
1594:
1589:
1586:
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1579:
1576:
1574:
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1569:
1566:
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1561:
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1557:
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1552:
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1543:
1541:
1537:
1534:
1532:
1527:
1523:
1520:
1518:
1514:
1504:
1501:
1499:
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1471:
1468:
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1438:
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1421:
1417:
1409:
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1397:
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1373:
1369:
1368:
1364:
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1357:
1353:
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1338:
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1323:
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1318:
1315:
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1308:
1304:
1303:
1299:
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1293:
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1288:
1284:
1280:
1279:
1276:
1271:
1267:
1260:
1251:
1247:
1243:
1239:
1235:
1231:
1227:
1223:
1216:
1213:
1208:
1204:
1199:
1194:
1190:
1186:
1182:
1175:
1172:
1167:
1163:
1159:
1155:
1151:
1147:
1143:
1139:
1132:
1129:
1124:
1120:
1116:
1112:
1108:
1104:
1096:
1093:
1088:
1084:
1079:
1074:
1070:
1066:
1061:
1056:
1052:
1048:
1044:
1037:
1034:
1029:
1025:
1021:
1017:
1013:
1009:
1005:
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986:
982:
978:
974:
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966:
962:
958:
950:
947:
942:
938:
933:
928:
923:
918:
914:
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906:
899:
896:
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867:
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859:
855:
848:
845:
840:
836:
831:
826:
822:
818:
814:
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806:
799:
796:
791:
787:
783:
779:
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771:
764:
761:
756:
750:
746:
739:
736:
731:
727:
723:
719:
715:
711:
707:
703:
696:
693:
690:
684:
681:
676:
672:
668:
664:
657:
654:
649:
645:
641:
637:
633:
629:
621:
618:
607:on 2013-03-20
603:
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591:
587:
583:
579:
572:
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562:
557:
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402:
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385:
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371:
369:
364:
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338:
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328:
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311:
309:
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297:
293:
289:
285:
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278:
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266:
264:
262:
258:
254:
246:
241:
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234:
233:
227:
225:
223:
217:
210:
208:
201:
199:
197:
193:
189:
185:
181:
177:
173:
169:
165:
161:
157:
153:
148:
144:
136:
134:
132:
124:
122:
120:
116:
112:
107:
103:
98:
96:
92:
87:
83:
79:
75:
71:
67:
57:
51:
48:
46:
42:
38:
34:
30:
25:
20:
2508:
2490:
2466:
2448:
2419:
2401:
2371:
2284:
2250:
2224:hyperplastic
2213:
2206:Colon/rectum
2195:
1842:
1756:Hidrocystoma
1750:
1676:HĂĽrthle cell
1657:Prolactinoma
1562:
1547:
1365:
1354:
1330:
1319:
1311:
1296:
1281:
1225:
1221:
1215:
1188:
1184:
1174:
1141:
1137:
1131:
1106:
1102:
1095:
1050:
1046:
1036:
1003:
999:
993:
960:
956:
949:
912:
908:
898:
861:
857:
847:
812:
808:
798:
776:(1): 69–76.
773:
769:
763:
744:
738:
705:
701:
695:
683:
666:
662:
656:
631:
627:
620:
609:. Retrieved
602:the original
581:
577:
564:
531:
527:
521:
496:
492:
486:
459:
455:
445:
408:
404:
394:
360:
351:
348:Epidemiology
339:
331:
319:Chemotherapy
317:
314:Chemotherapy
281:
270:
250:
231:
221:
218:
214:
205:
147:goblet cells
140:
128:
99:
69:
65:
64:
2468:gallbladder
2421:hyperplasia
1798:Cystadenoma
1751:sweat gland
1742:Adnexal and
1573:Glucagonoma
327:Mitomycin C
275:(CRS) with
168:gallbladder
145:expressing
131:infertility
119:bloodstream
2587:Categories
2554:Peritoneum
1781:and serous
1724:Cylindroma
1709:Oncocytoma
1621:Urogenital
1578:Gastrinoma
1568:Insulinoma
1424:Epithelium
1332:DiseasesDB
611:2013-04-21
386:References
284:peritoneum
194:, and the
172:bile ducts
2513:neoplasms
2450:bile duct
2373:malignant
2355:Accessory
2285:Neoplasm:
2268:Gardner's
2191:Carcinoid
2077:Esophagus
1779:mucinous,
1761:Syringoma
1697:Carcinoid
1649:Endocrine
1437:carcinoma
1433:Papilloma
1356:eMedicine
1069:1068-9265
257:debulking
247:Treatment
202:Diagnosis
45:Specialty
2483:Pancreas
2254:Juvenile
2228:juvenile
2183:Appendix
2059:GI tract
1897:lobular,
1812:Mucinous
1563:pancreas
1526:Adenomas
1367:Orphanet
1361:med/1795
1250:19571870
1207:29641675
1166:12923510
1158:18590843
1123:26026742
1087:32488520
1028:24206562
1020:25560182
977:11776492
941:15701175
915:(1): 3.
890:31183454
858:BJS Open
839:21505593
790:16389186
730:24335764
722:21970481
598:20524864
556:24386163
548:15906901
513:15807473
478:22614976
437:17036382
374:See also
342:CT scans
334:adjuvant
267:Surgical
196:pancreas
152:mucinous
95:cachexia
86:fibrosis
50:Oncology
2220:adenoma
2100:Stomach
1895:Ductal,
1791:general
1777:Cystic,
1719:Apudoma
1498:Thymoma
1326:D011553
1316:: M8480
1242:7844982
1078:7752784
985:5692764
881:6551418
830:3070715
648:7503361
428:4088104
357:History
288:ovaries
164:stomach
82:ascites
2510:cystic
2403:benign
1996:Acinar
1860:Serous
1789:Cystic
1583:VIPoma
1548:tract:
1517:Glands
1416:cancer
1248:
1240:
1205:
1164:
1156:
1121:
1085:
1075:
1067:
1026:
1018:
983:
975:
939:
932:549516
929:
888:
878:
837:
827:
788:
751:
728:
720:
687:e.g.,
646:
596:
554:
546:
511:
476:
435:
425:
325:agent
304:spleen
296:uterus
188:breast
160:rectum
52:
2364:Liver
2149:Lower
2068:Upper
1372:26790
1337:31498
1313:ICD-O
1307:197.6
1292:C78.6
1246:S2CID
1162:S2CID
1024:S2CID
981:S2CID
726:S2CID
605:(PDF)
574:(PDF)
552:S2CID
308:liver
156:colon
137:Cause
106:ovary
91:colon
78:mucin
2308:Anus
1998:cell
1321:MeSH
1302:9-CM
1238:PMID
1203:PMID
1154:PMID
1119:PMID
1083:PMID
1065:ISSN
1016:PMID
973:PMID
937:PMID
886:PMID
835:PMID
786:PMID
749:ISBN
718:PMID
644:PMID
594:PMID
544:PMID
509:PMID
474:PMID
433:PMID
184:lung
143:MUC2
1298:ICD
1283:ICD
1230:doi
1193:doi
1146:doi
1111:doi
1073:PMC
1055:doi
1008:doi
965:doi
927:PMC
917:doi
876:PMC
866:doi
825:PMC
817:doi
778:doi
710:doi
706:135
671:doi
636:doi
586:doi
582:134
536:doi
532:105
501:doi
464:doi
423:PMC
413:doi
255:to
70:PMP
2589::
2516::
2242:,
2238:,
2234:,
2230:,
2226:,
2222:,
1933:/
1879:/
1875:/
1871:/
1835:/
1370::
1359::
1335::
1324::
1305::
1290::
1287:10
1244:.
1236:.
1226:58
1224:.
1201:.
1189:64
1187:.
1183:.
1160:.
1152:.
1142:45
1140:.
1117:.
1107:41
1105:.
1081:.
1071:.
1063:.
1051:28
1049:.
1045:.
1022:.
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1004:19
1002:.
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959:.
935:.
925:.
911:.
907:.
884:.
874:.
860:.
856:.
833:.
823:.
813:18
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807:.
784:.
772:.
724:.
716:.
704:.
667:13
665:.
642:.
632:19
630:.
592:.
580:.
576:.
550:.
542:.
530:.
507:.
497:21
495:.
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460:30
458:.
454:.
431:.
421:.
409:12
407:.
403:.
294:,
290:,
263:.
198:.
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