Pancreatic intraductal tubulopapillary neoplasms (ITPNs) are rare variants of intraductal papillary neoplasms. Unlike an IPMN, an ITPN does not produce mucin.
Incidence/prevalence is unclear. Small series show an even male:female distribution and an average age of presentation of 58 years old 1.
Nonspecific. Presents similarly to other pancreatic tumours, with signs/symptoms depending on the location of the tumour. They demonstrate high-grade atypia.
According to the WHO criteria, ITPN is a subtype of intraductal tubular neoplasms (ITN). Histologically, these tumours are distinct from pancreatic intraepithelial neoplasia, intraductal papillary mucinous neoplasm (IPMN), and intraductal variant of pancreatic acinar cell carcinoma.
Tumor cells form tubulopapillae and contain little cytoplasmic
mucin. There is also absence of acinar differentiation.
- positive for cytokeratin 7 and/or cytokeratin 19
- negative for trypsin, MUC2, MUC5AC, and fascin.
- aberrant expression of b-catenin is not observed.
Like a main-duct IPMN, an ITPN demonstrates a dilated and irregular main pancreatic duct, but without an abundance of low attenuation mucin.
- a "two-tone duct" or "cork-in-bottle" sign has been suggested 2
- a soft-tissue attenuation "cork" in the main pancreatic duct plugs the lower attenuation pancreatic fluid
- main pancreatic duct distal to the tumour is dilated
- intraductal tumour is isoattenuating or mildly hyperattenuating relative to surrounding pancreatic parenchyma
- the tumour is relatively hypoattenuating after contrast administration
- T1: tumour is mildly hypointense
- T2: tumour is mildly hyperintense
- MRCP: tumour fills main pancreatic duct like a "cork"
Treatment and prognosis
Because of the rarity of the tumour, treatment and prognosis are not well established. In one series, 8/10 patients are still alive after pancreaticoduodenectomy.
- 1. Yamaguchi H, Shimizu M, Ban S et-al. Intraductal tubulopapillary neoplasms of the pancreas distinct from pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms. Am. J. Surg. Pathol. 2009;33 (8): 1164-72. doi:10.1097/PAS.0b013e3181a162e5 - Pubmed citation
- 2. Motosugi U, Yamaguchi H, Furukawa T et-al. Imaging studies of intraductal tubulopapillary neoplasms of the pancreas: 2-tone duct sign and cork-of-wine-bottle sign as indicators of intraductal tumor growth. J Comput Assist Tomogr. 2012;36 (6): 710-7. doi:10.1097/RCT.0b013e31826d1fc8 - Pubmed citation