Primary Bile Duct Cancer and von Recklinghausen Disease

Excerpt

To the editor: The association of neurofibromatosis (von Recklinghausen disease) with malignant tumors of neural crest origin is well documented, but common cancers like colon, lung, and breast cancer are underreported (1, 2). Neurofibromas and neurogenic tumors of the gastrointestinal tract are found in 10% to 25% of patients (3). We report two cases of bile duct cancer, an uncommon type of adenocarcinoma, in patients with neurofibromatosis.

A 50-year-old man with known neurofibromatosis was hospitalized because of painless jaundice. His total serum bilirubin level was 23.7 mg/dL, and alkaline phosphatase level 1033 IU/L. Endoscopic retrograde cholangiopancreatography showed hilar obstruction, and