Recurrent distal cholangiocarcinoma and humoral hypercalcemia of malignancy: report of a rare case and literature review

Abstract

A 61-year-old Japanese woman presented with epigastric pain and jaundice. Imaging showed the presence of primary distal cholangiocarcinoma (DCC). A subtotal stomach-preserving pancreaticoduodenectomy was performed, followed by chemotherapy using S-1. However, second-line chemotherapy with gemcitabine and cis-diamminedichloroplatinum was required for the treatment of hepatic metastasis of the DCC 3 months following the surgery. Nine months after the surgery, the serum calcium and parathyroid hormone-related peptide concentrations were high, at 16.5 mg/dL and 28.7 pmol/L, respectively, which suggested the presence of humoral hypercalcemia of malignancy (HHM) secondary to the DCC. Moreover, marked leukocytosis, with a white blood cell count of 40,400/μL, was also present. The patient died 11 months after the diagnosis of DCC. Because hypercalcemia of malignancy is associated with a poor prognosis, and HHM and leukocytosis caused by DCC are very rare, we have presented the present case in detail and provide a review of the existing literature.

A Case of Fibroblast Growth Factor Receptor Fusion-Positive Intrahepatic Cholangiocarcinoma With Humoral Hypercalcemia of Malignancy

“…Literature suggests that elevated PTHrP might be an indicator of aggressive disease and reflects poor prognosis, especially in patients less than 65 years of age [13]. A recent case report and literature review of HHM in cholangiocarcinoma by Ito et al highlighted similar findings in regard to PTHrP and associated poor prognosis [5].…”

“…HHM is mostly seen in squamous cell carcinomas such as the lung, head and neck, esophagus, and cervix [ 3 , 4 ]. HHM in cases of cholangiocarcinoma is rare and, to our knowledge, has only been reported in a few case reports [ 5 - 9 ].…”

“…Cholangiocarcinoma has been anatomically classified into intrahepatic, distal, and perihilar with intrahepatic being 10-20% of all cases [ 10 ]. HHM has been reported predominantly in cases of intrahepatic cholangiocarcinoma [ 5 ]. Unresectable cholangiocarcinoma carries a dismal prognosis in general [ 10 ].…”

“…The demographic characteristics and anatomical classification of this case are in line with what has been previously reported in the literature. Literature suggests a median survival from diagnosis of hypercalcemia to be 3-5 months [ 5 , 11 ]. However, our patient lived for approximately 10 months after the initial diagnosis of hypercalcemia.…”