Published online Sep 28, 2019. doi: 10.3748/wjg.v25.i36.5569
Peer-review started: July 17, 2019
First decision: August 18, 2019
Revised: August 29, 2019
Accepted: September 11, 2019
Article in press: September 11, 2019
Published online: September 28, 2019
Intraductal papillary neoplasm of the bile duct (IPNB) is a type of tumor that presents in the intra- or extrahepatic bile ducts. Cystic-type intrahepatic IPNB often mimics simple liver cysts, making the diagnosis difficult. Because the growth of IPNB is slow, careful follow-up and timely therapeutic intervention is recommended. There are few reports with a follow-up period longer than a decade; thus, we report the case of a patient with an IPNB that grew for over 13 years.
A 65-year-old man was diagnosed, 13 years prior with a cystic hepatic tumor with abnormal imaging findings. The targeted tumor biopsy results showed no malignancy. Biannual follow-up examinations were performed because of the potential for malignancy. The cystic lesions showed gradual enlargement over 11 years and a 4 mm papillary proliferation appeared on the cyst wall, which is compatible with IPNB. The tumor was observed for another 2 years because of the patient’s wishes. The imaging findings showed enlargement to 8 mm and a new 9 mm papillary proliferation of the cystic tumor. Contrast-enhanced ultrasonography showed hyperenhancement during the arterial phase in both cyst walls, indicating intraductal tumor progression in both tumors. Thus, liver segment 8 subsegmentectomy was performed. The pathological findings indicated that the tumors contained mucin, and high-grade atypia was observed in the papillary lesions, showing IPNB.
The development of IPNB should be monitored in patients with cystic lesions and ultrasonography are useful tool for the evaluation.
Core tip: Cystic lesions in the liver, such as intraductal papillary neoplasms of the bile duct, rarely yield malignant tumors; additionally, the development of a tumor over a decade is hardly ever observed. Here, we present the case of a patient with an intrahepatic intraductal papillary neoplasm of the bile duct that developed over 13 years from a cystic tumor of the liver. The tumor was diagnosed by contrast-enhanced ultrasonography with perfluorobutane and was completely surgically resected. Notably, the intracystic spread of the tumor was well represented by contrast-enhanced ultrasonography.