Sclerosing hepatic carcinoma: relationship to hypercalcemia

Abstract

A series of 30 patients is reported whose primary hepatic tumors had a distinctive histologic pattern that we have called "sclerosing hepatic carcinoma" (SHC). Sixty-nine percent of those tested had hypercalcemia and low levels of serum phosphate. As comparison, 38 control patients who had either classical peripheral cholangiocarcinoma or typical hepatocellular carcinoma were studied. In the control group, only two patients, who also had bone metastases, had hypercalcemia. Sclerosing hepatic carcinoma is characterized by intense fibrosis in which the tubular neoplastic structures are embedded. Although the tumor in each patient superficially resembled peripheral cholangiocarcinoma, on close inspection 63% were found to be of apparent hepatocyte origin, 20% were apparently ductal, and 13% were mixed or not distinguishable. One patient's tumor had the pattern of the rare cholangiolocellular carcinoma. The difficulty of histological diagnosis was well illustrated by the fact that none of premortem biopsies in 16 patients was correctly interpreted unequivocally as primary carcinoma of the liver. Many were misinterpreted as metastatic adenocarcinomas, most frequently of pancreatic origin. We believe that, by describing clinicopathological features, more attention will be drawn to this unique carcinoma of liver origin.