An 11-year-old male patient demonstrating cholangiocarcinoma associated with congenital biliary dilatation

Diagnosis and treatment of pancreaticobiliary maljunction in children

“…In the published literature, all pediatric patients who developed biliary tract cancer were reported to have PBM. [22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] This supports the hypothesis that epithelial changes of the gallbladder, leading to carcinogenesis, may occur in early childhood in these patients. We also speculate that patients with PBM have the possibility of producing precancerous lesions of the biliary tree early in childhood.…”

“…22 In a review of the literature, several investigators were found to have reported cholangiocarcinoma that developed in patients younger than 20 years old who had PBM and choledochal cysts. [22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] In the presence of PBM, free refl ux of pancreatic juice into the biliary tract can occur, and this may damage the biliary epithelium. In fact, epithelial hyperplasia of the biliary tract was frequently observed in patients with choledochal cysts with PBM.…”

Pancreaticobiliary maljunction and carcinogenesis to biliary and pancreatic malignancy

“…The average age of patients of biliary tract cancer with PBM is 10 years younger than that of cancer without PBM [5], and very young teenagers having carcinoma with PBM [22,23] were reported, which were not seen in cancer patients without PBM.…”

“…It is believed by some investigators [21][22][23] that the number of bile duct cancer cases exceeds the number of gallbladder cancer in the dilated bile duct cases of PBM, and that gallbladder cancer frequently occurs but bile duct cancer rarely occurs in PBM cases not associated with dilatation. But the last 10 years of JSPBM (Table 2) indicates that gallbladder cancer rate is higher than bile duct cancer (14.8% vs. 4.9%), even in the biliary dilatation cases (8.8% vs. 5.2%).…”

Controversies in choledochal malformation

“…This risk is probably due to ongoing chronic inflammation in the epithelium over many years, exacerbated by episodic cholangitis and stone formation. The youngest case appears to be an 11-year-old boy, reported by Tanaka et al [18] in 2006, who developed adenocarcinoma in a type 4 CM. Tanaka et al also identifed ten more cases from the literature in patients aged <20 years, the majority also arising in long-standing type 4 CMs.…”