Intrahepatic cholestasis as a paraneoplastic syndrome associated with pheochromocytoma

Abstract

Pheochromocytoma is a rare tumor of chromaffin cells that secrete catecholamines and several cytokines. The clinical manifestations are protean and may include hypertension, weight loss, sweating, palpitation, headache, anxiety, tremor, nausea, vomiting, and hypercalcemia. The tumor can mimic many unrelated diseases, leading to significant delay and difficulty in diagnosis. We report a case of a 37-yr-old male admitted with jaundice, dark urine, fever, and signs of a systemic inflammatory response. Abdominal computed tomography revealed a heterogeneously enhancing tumor between the pancreatic tail and left kidney. There was no evidence of obstruction to bile flow, neoplastic involvement of the liver or bile ducts, or infectious etiology. The tumor was removed and found to be a pheochromocytoma. Immunohistochemical analysis revealed the presence of interleukin-1beta in the tumor cells. After surgery, the jaundice resolved without further treatment, leading us to the conclusion that it was a paraneoplastic phenomenon possibly related to interleukin-1beta production. We suggest that occult pheochromocytoma should be added to the differential diagnosis of unexplained intrahepatic cholestasis.