Intracholecystic papillary-tubular neoplasm in a patient with choledochal cyst: a link between choledochal cyst and gallbladder cancer?

Abstract

BackgroundIntracholecystic papillary-tubular neoplasms are rare precursor lesions of gallbladder cancer. They were proposed as a separate pathologic entity in 2012 by Adsay et al. for the unification of a variety of mass-forming precursor lesions including papillary adenomas, tubulopapillary adenomas, intestinal adenomas, and others. They are considered homologous to intrapapillary mucinous neoplasms of the pancreas and intrabiliary papillary neoplasms of the common bile duct. In contrast with the commoner flat-type precursor gallbladder cancer lesions, they follow a more indolent clinical course and probably different genetic pathways to carcinogenesis. They are largely uninvestigated with only a handful of studies providing biological and clinical information. Choledochal cysts are dilation of the common bile duct. Diagnosis is usually established during childhood, and only a minority of patients are diagnosed at adulthood. They are of major clinical importance as they are known predisposing factors for biliary carcinogenesis.Case presentationThe current report describes a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. The patient underwent excision of the extrahepatic biliary tree for a Todani I choledochal cyst, and histological examination of the specimen revealed an intracholecystic papillary-tubular neoplasm of the gallbladder. Authors describe diagnostic and clinical course of the patient alongside clinical and biological characteristics of these rare lesions.ConclusionsTo the best of our knowledge, this is the first report of a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. Those rare lesions shed light on different forms of gallbladder cancer carcinogenesis and its relationship with choledochal cysts and cholestasis.

A Case Report of Intraductal Papillary Neoplasm Associated with an Extrahepatic Bile Ducts

“…Approximately 70% of tumors are seen in the biliary tract and 30% in the gallbladder. 6 Bile duct cysts are divided into types according to the Todani anatomical classification. Type 1 is the most common type with a frequency of 50-90%, and Type 4 is the second most common type with a frequency of 15-42%.…”

“…Cholecystectomy is included in the surgical procedure because the risk of gallbladder cancer increases in surgical resection of all types. 6 In the follow-up of these patients abdominal ultrasound and parameters of cholestasis, cancer antigen 19-9 levels is recommended on annual basis. 15 We planned the postoperative follow-up with annual blood test and abdominal ultrasound, MRI/MRCP in a suspicious finding.…”

Intracholecystic Papillary Neoplasm of Gallbladder in a Middle-Aged Woman—A Rare Entity

“…7 ICPN has a better prognosis as compared to GB carcinoma. 8 Long-term follow-up is suggested after ICPN resection due to the field effect rendering a high incidence of biliary tract carcinoma. 1…”

Neoplasia Papilar Intracolecística. Reporte de un Caso y Revisión de la Evidencia Existente

“…Existen escasos reportes en la literatura, la mayor parte de los cuales son de casos aislados (Almagro, 1985;Arunabh et al, 1988;Bazira et al, 1998;Kushima et al, 1996;Nagahama et al, 1997;Kosemehmetoglu et al, 2011;Resende et al, 2012;Hashimoto et al, 2014;Michalinos et al, 2016;Páez Cumpa et al, 2017); de reportes de menos de 10 casos (Araki et al, 1988;Diaz-Plasencia et al, 1994;Bennett et al); y de series entre 16 y hasta 123 casos (Albores-Saavedra et al, 2005;Adsay et al;Argon et al, 2016), siendo esta última la mayor de todas (Adsay et al). No obstante ello, entre todas, no suman más de 250 en total, y es posible, que en algunos casos pueda existir duplicidad de información (Tabla I).…”