What is intraductal papillary mucinous neoplasm

Intraductal papillary-mucinous neoplasia

1 definition

In the intraductal papillary-mucinous neoplasia, short IPMN, is a primarily intraductally growing epithelial pancreatic tumor that consists of mucinous cells. IPMN are usually built heterogeneously and show different degrees of dysplasia and malignancy.

2 epidemiology

With 20-30% of all cystic pancreatic tumors, intraductal papillary-mucinous neoplasia is the most common entity of cystic pancreatic neoplasms. Men are more frequently affected than women (approx. 3: 2). The average age is 60 to 70 years.

3 pathology

IPMNs that arise in the main pancreatic duct are referred to as "main duct type" (MDT); tumors that are located in the secondary ducts are correspondingly called "branch duct type" (BDT). This differentiation makes sense because several studies have shown that the BDT shows less aggressive behavior, i.e. it grows less invasively than the MDT. However, a combination of both forms is possible.

About 80% of the IPMNs are found in the head of the pancreas. Involvement of the papilla vateri and the periampullary duodenum is possible. The BDT variant arises mainly in the uncinate process.

4 pathohistology

Pathohistologically, the non-invasive IPMNs with minor, moderate or severe dysplasias (adenoma, borderline or carcinoma in situ type) are differentiated from the invasive IPMNs. The latter are defined by the invasive carcinoma arising from them, which clinically corresponds to ductal pancreatic carcinoma or mucinous adenocarcinoma.

5 symptoms

The clinical symptoms of IPMN include:

If the cyst is already larger, there may be pressure in the epigastrium and / or a feeling of fullness.

6 diagnostics

7 therapy

Surgical resection is the main pillar of the therapeutic strategy in IPMN of the pancreas. The goal is a surgical R0 resection. Like ductal adenocarcinomas, IPMN is mainly located in the head of the pancreas, so partial pancreaticoduodenectomy (Kausch-Whipple operation) is the most common procedure. If the pancreatic parenchyma is diffuse, a total pancreatectomy should be considered, depending on the general condition of the patient.

7.1 Consensus recommendations

The therapeutic approach with BDT is more demanding than with MDT. In this case, careful consideration must be given between the malignant potential of the tumor and the risk of surgery. For this purpose, the "International consensus guidelines for the treatment of branch duct IPMN" were drawn up in 2006. The guidelines suggest that patients be asymptomatic Not to be operated on, if

  • the neoplasm is <3 cm,
  • does not contain any nodular solid matter,
  • there is no dilatation of the large pancreatic ducts.

In contrast, the guidelines recommend surgical resection

  • existing symptoms
  • with a size> 3 cm
  • Presence of a nodular, solid mass
  • Dilatation of the great pancreatic ducts

Branch duct type IPMNs that are not surgically resected should be periodically monitored radiographically to ensure that they do not change any further. The imaging can be done by CT, EUS or MRCP. If BDT-IPMNs are smaller than 1 cm in diameter, an annual check is sufficient. Patients with a larger BDT-IPNM, on the other hand, should undergo quarterly check-ups.

7.2 Fukuoka criteria

The so-called revised Fukuoka criteria have also existed since 2012, which no longer make the therapy recommendation mainly dependent on participation in the main and secondary aisle. A distinction is made between:

  • Fukuoka-positive IPMNs with high-risk stigmata
  • IPMNs with problematic properties ("worrisome features") and
  • Fukuoka-negative or inconspicuous IPMNs.

Fukuoka-positive IPMNs have a significantly increased risk of malignancy. This includes IPMN

  • of the head of the pancreas, which have led to obstructive jaundice
  • with a contrast enhancing intramural nodule> 5 mm
  • with an expansion of the main pancreatic duct to> 10 mm (so-called "main-duct IPMN")

IPMNs with problematic properties are IPMNs with:

  • cystic lesions> 3 cm
  • thickened, contrast-enhancing cyst wall
  • Expansion of the main pancreatic duct to 5-9 mm
  • abrupt change in pancreatic duct width and distal atrophy
  • Detection of lymph nodes
  • CA 19-9 increase in serum
  • Cyst growth of at least 5 mm in 2 years
  • Pancreatitis

8 forecast

Intraductal papillary-mucinous neoplasia generally has a better prognosis than ductal adenocarcinoma of the pancreas. In addition, the distinction between non-invasive and invasive forms is of great prognostic importance.

Overall, patients with a non-invasive IPMN have a very good prognosis (5-year survival rate of 77%), while patients with invasively growing IPMN after radical resection have a much poorer prognosis (5-year survival rate of 43%). The "branch duct type" IPMN has a better prognosis than the "main duct type".

In about 10% of cases, a relapse is to be expected after surgical resection. The cause of death in papillary-mucinous carcinomas with peripancreatic invasion is usually a distant metastasis.

9 sources

  • Yamaguchi K, Kanemitsu S, Hatori T, Maguchi H, Shimizu Y, Tada M, et al. "Pancreatic ductal adenocarcinoma derived from IPMN and pancreatic ductal adenocarcinoma concomitant with IPMN", Pancreas 2011; 40: 571-80
  • Tanaka M, Chari S, Adsay V, Fernandez-del Castillo C, Falconi M, Shimizu M, et al. "International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas". Pancreatology 2006; 6: 17-32
  • Tanaka M, Fernandez-del Castillo C, Kamisawa T, Jang JY, Levy P, Ohtsuka T, et al. ‘Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas’, Pancreatology 2017; 17: 738-53
  • Tanaka M, Fernandez-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, et al. ‘International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas’, Pancreatology 2012; 12: 183-97
  • Grützmann, Robert; Post, Stefan; Saeger, Hans Detlev; Niedergethmann, Marco: Intraductal papillary-mucinous neoplasia of the pancreas. Current status of diagnostics, therapy and prognosis. Dtsch Arztebl Int 2011; 108 (46): 788-94; DOI: 10.3238 / arztebl.2011.0788