ArticleJournal of surgical case reports2026
Malignant transformation of choledochal cyst 38 years after cystoduodenostomy mimicking Lemmel's syndrome: a rare diagnostic pitfall.
Article in Journal of surgical case reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Malignant transformation of choledochal cysts after internal drainage is a known risk, but presentation nearly four decades later is exceptional. We report a case of cholangiocarcinoma developing 38 years after cystoduodenostomy in a 39-year-old female. Diagnosis was confounded by a periampullary diverticulum, mimicking Lemmel's syndrome. Multimodal imaging suggested a hilar mass, but endoscopy revealed a large diverticulum. Exploratory laparotomy and frozen section analysis confirmed adenocarcinoma arising from the remnant cyst. The patient underwent complete cyst excision, lymphadenectomy, and Roux-en-Y hepaticojejunostomy. This case underscores two critical lessons: the carcinogenic risk of internal drainage persists indefinitely, necessitating lifelong surveillance; and secondary malignancies can anatomically mimic benign conditions like Lemmel's syndrome, requiring a high index of suspicion and meticulous surgical management.
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