Advanced bile duct carcinoma in a 15-year-old patient with pancreaticobiliary maljunction and congenital biliary cystic disease.
We report a case of advanced bile duct carcinoma arising in a 15-year-old female with pancreaticobiliary maljunction and congenital biliary cystic disease. Pancreaticoduodenectomy and partial resection of the liver was performed. Surgical and histopathological findings indicated advanced tubular adenocarcinoma, classified as final stage IVb according to the General rules for surgical and pathological studies on cancer of the biliary tract proposed by the Japanese Society of Biliary Surgery, 5th edition, and stage IV according to the American Joint Committee on Cancer (AJCC)/International Union Against Cancer (UICC), 6th edition. She underwent chemotherapy with gemcitabine HCl after discharge. She died of cachexia 14 months after the surgery. Although it is well known that biliary malignancies arise frequently in patients with pancreaticobiliary maljunction, it is uncommon for advanced bile duct carcinoma to occur in a 15-year-old female. We should pay attention to the possibility of biliary malignancy in patients with pancreaticobiliary maljunction and congenital biliary cystic disease, even when the patients are juveniles.
Division of Hepato-Biliary Pancreatic Surgery, Department of Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Aoba-ku, Sendai, Japan.
Hiroyuki
H
Nakamura
Yu
Y
Katayose
Toshiki
T
Rikiyama
Tohru
T
Onogawa
Kuniharu
K
Yamamoto
Hiroshi
H
Yoshida
Hiroki
H
Hayashi
Hideo
H
Ohtsuka
Yutaka
Y
Hayashi
Shin-ichi
S
Egawa
Michiaki
M
Unno
D000230:Adenocarcinoma / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D000293:Adolescent; D001650:Bile Duct Neoplasms / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D001659:Biliary Tract / Q000002:abnormalities; D003560:Cysts / Q000151:congenital; D004066:Digestive System Diseases / Q000150:complications* / Q000151:congenital; D005260:Female; D006498:Hepatectomy; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary* / Q000628:therapy; D010179:Pancreas / Q000002:abnormalities; D016577:Pancreaticoduodenectomy