Mixed hepato/cholangiocarcinoma with paraneoplastic hypercalcemia

Malignant hypercalcemia without bone metastasis may result from the abnormal secretion of parathyroid hormone (PTH) or PTH-related protein (PTH-rP). We present a case of possible PTH-rP-secreting mixed hepato/cholangiocarcinoma. In July 2000, a 52-year-old woman presented with right hypochondrial pain and weight loss. Imaging procedures revealed a 10-cm lesion involving the right hepatic lobe and the hilum. Retrohepatic inferior vena caval invasion was detected by abdominal angiography. Serum calcium was elevated, while intact PTH was undetectable. The rising level of hypercalcemia rapidly became life-threatening. Medical treatment with diphosphonate and octreotide was ineffective. Chemoembolization of the right branch of the hepatic artery, preceded by right portal vein embolization, helped regain normocalcemia and improve the patient's general condition. Extended right hepatectomy with prosthetic replacement of the vena cava and resection of the left portal vein was performed. Histopathology revealed mixed hepato/cholangiocarcinoma. Hypocalcemia appeared postoperatively but was successfully corrected. Recurrence of diffuse hepatic nodules resulted in recurrent hypercalcemia and death 3 and a half years after the surgery. This is the third case to be reported in the English-language literature. Paraneoplastic PTH-rP-induced hypercalcemia, which may be a fatal complication, can respond to arterial chemoembolization. Extensive hepatic resection may offer a good chance for survival.