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Correctable biliary atresia and cholangiocarcinoma: a case report of a 63-year-old patient
Masaki Nio1, Motoshi Wada2, Hideyuki Sasaki2
1Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1, Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan. mnio@ped-surg.med.tohoku.ac.jp.
Insights
Cholangiocarcinoma is an exceptionally rare cancer in biliary atresia survivors. Increased long-term survival necessitates vigilant follow-up for potential secondary malignancies in these patients.
Area of Science:
- Hepatobiliary surgery
- Pediatric surgery
- Surgical oncology
Background:
- Cancer development after biliary atresia surgery is increasing.
- Cholangiocarcinoma in native liver survivors of biliary atresia is extremely rare.
- This case highlights a rare occurrence in a long-term survivor.
Purpose of the Study:
- To report an extremely rare case of cholangiocarcinoma in a biliary atresia survivor.
- To emphasize the importance of long-term surveillance in biliary atresia patients.
Main Methods:
- A case presentation of a female patient with biliary atresia.
- The patient underwent multiple surgical procedures throughout her life.
- Diagnosis of cholangiocarcinoma was made at age 63.
Main Results:
- The patient developed cholangiocarcinoma at the porta hepatis.
- Chemotherapy was administered, but the cancer progressed.
- The patient died before reaching 64 years of age.
Conclusions:
- Cholangiocarcinoma is a very rare complication in biliary atresia patients.
- Close follow-up is crucial for biliary atresia survivors.
- The incidence of secondary malignancies may rise due to increased long-term survival rates.
Background:
Although cancer occurrence following surgery for biliary atresia has gradually increased, the development of cholangiocarcinoma in a native liver survivor of biliary atresia is extremely rare.
Case Presentation:
A 3-month-old female patient with the correctable type of biliary atresia underwent a cystoduodenostomy. At 16 years of age, she underwent multiple surgeries including lysis of intestinal adhesions, ileostomy, and gastrojejunostomy at another hospital. At 54 years of age, she underwent lithotomy at the porta hepatis, resection of the residual cystic bile duct with gallbladder, and hepaticojejunostomy in Roux-en-Y fashion. As she approached the age of 63, her computed tomography scan showed no liver tumors. In the following year, she developed cholangiocarcinoma at the porta hepatis and underwent chemotherapy. However, the cancer progressed, and she died before she reached the age of 64 years.
Conclusions:
Cholangiocarcinoma is extremely rare in patients with biliary atresia. However, physicians should follow up patients with biliary atresia as closely as possible, as malignant tumors secondary to biliary atresia may increase in number in the near future because of the growing number of long-term survivors with biliary atresia.
