Leptomeningeal carcinomatosis is a rare and deceptive presentation of
systemic cancer. We report the treatment of a patient with a Klatskin'
s tumor by surgical resection. The patient presented with symptoms sug
gestive of liver failure in the early postoperative period. He rapidly
developed progressive, multineuroaxis symptoms and died. Routine gast
rointestinal evaluation failed to demonstrate any intraabdominal patho
logic process that could be responsible for his decline. A MRI of the
brain was also unremarkable. Serial lumbar punctures, however, documen
ted leptomeningeal carcinomatosis consistent with a gastrointestinal p
rimary. The clinical presentation of leptomeningeal carcinomatosis is
subtle and may masquerade as another disease state. The hallmark of th
is lethal process is the finding of progressive neurologic deficits at
more than one level of the neuroaxis. Serial cerebrospinal fluid exam
ination is often diagnostic. This is the first known report of leptome
ningeal carcinomatosis secondary to primary cholangiocarcinoma.