Leptomeningeal carcinomatosis: a sequela of cholangiocarcinoma

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 suggestive of liver failure in the early postoperative period. He rapidly developed progressive, multineuroaxis symptoms and died. Routine gastrointestinal evaluation failed to demonstrate any intraabdominal pathologic process that could be responsible for his decline. A MRI of the brain was also unremarkable. Serial lumbar punctures, however, documented leptomeningeal carcinomatosis consistent with a gastrointestinal primary. The clinical presentation of leptomeningeal carcinomatosis is subtle and may masquerade as another disease state. The hallmark of this lethal process is the finding of progressive neurologic deficits at more than one level of the neuroaxis. Serial cerebrospinal fluid examination is often diagnostic. This is the first known report of leptomeningeal carcinomatosis secondary to primary cholangiocarcinoma.