Ciliated hepatic foregut cysts are rare congenital lesions derived from the
embryologic foregut. They are considered benign, and a review of 64 publis
hed cases revealed no instances of malignant transformation. We report a ca
se of squamous cell carcinoma arising in a ciliated hepatic foregut cyst in
a 51-year-old man. The tumor was found during a routine cholecystectomy an
d involved the adjacent mesentery and duodenal wall. There was histologic e
vidence of perineural and perivascular involvement. Despite an en bloc rese
ction of the tumor and contiguous areas of gross involvement, the patient d
ied 2 months later. Although aspiration of cyst contents is an accepted tre
atment for asymptomatic lesions, this case suggests that most ciliated hepa
tic foregut cysts should be excised, especially when radiologic studies yie
ld equivocal results.