Invasive cholangiocarcinoma identified in congenital biliary dilatation in a 3-year-old boy
Nobuyuki Saikusa1, Shinichi Naito, Yasushi Iinuma
1Department of Pediatric Surgery, Niigata City General Hospital, Niigata City, Niigata 950-1197, Japan. saikusa_nobuyuki@med.kurume-u.ac.jp
Insights
This report details the youngest known case of a 3-year-old boy diagnosed with invasive cholangiocarcinoma (bile duct cancer) linked to congenital biliary dilatation. Despite initial negative findings, surgery confirmed cancer, highlighting the need for vigilance in pediatric cases.
Area of Science:
- Pediatric Gastroenterology
- Surgical Oncology
- Hepatobiliary Surgery
Background:
- Congenital biliary dilatation (CBD) is a rare condition.
- Cholangiocarcinoma (CC) is uncommon in pediatric patients.
- Pancreaticobiliary maljunction is a known risk factor for biliary tract cancers.
Observation:
- A 3-year-old boy presented with abdominal pain and vomiting.
- Imaging revealed a dilated extrahepatic bile duct, diagnosed as Todani's type 1a CBD.
- Intraoperative cholangiography showed pancreaticobiliary maljunction but no visible tumor.
Findings:
- Postoperative histopathology confirmed well-differentiated tubular adenocarcinoma with lymphovascular invasion.
- Carcinoma was present at both surgical margins, indicating residual disease.
- This represents the youngest reported case of CC associated with CBD.
Implications:
- Early diagnosis and management of CC in pediatric patients with CBD are crucial.
- Close follow-up is essential even after initial negative findings.
- This case underscores the importance of thorough histopathological examination in suspected biliary anomalies.
Abstract:
We herein report a 3-year-old male demonstrating invasive cholangiocarcinoma (CC) associated with congenital biliary dilatation (CBD). A 3-year-old Japanese boy was admitted to our hospital with abdominal pain and vomiting. Computed tomography and magnetic resonance imaging demonstrated a dilated extrahepatic bile duct. A diagnosis of Todani's type 1a CBD was made. Intraoperative cholangiography demonstrated the presence of pancreaticobiliary maljunction but could not reveal any tumor lesion in the bile duct. The excision of extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy were performed. On gross inspection, we could not find any tumor lesion in the resected specimen. However, the postoperative histopathologic examinations confirmed the presence of well-differentiated tubular adenocarcinoma with lymphovascular invasion. Most of the carcinoma remained within the mucosal layer, and the carcinoma was identified at both the distal and proximal surgical margins of the bile duct. We scheduled additional surgery to eradicate the residual carcinoma, but informed consent for the extra surgery could not be obtained. A close follow-up with abdominal computed tomography has been going on without either additional surgery or adjuvant chemotherapy about for 1 year. The patient has so far been clinically doing well without any obvious symptoms of recurrent disease. To our knowledge, this report is the youngest case of CC associated with CBD.