IgG4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma (Klatskin tumor): a case report of a challenging disease and review of the literature

Abstract

Background:Even though IgG4-related disease has gained increased attention worldwide, the diagnosis remains challenging. IgG4-related sclerosing cholangitis (IgG4-SC) is not well described in the western hemisphere and may mimic cholangiocarcinoma (CC), especially when occurring without other symptoms such as, e.g. concurrent pancreatitis or retroperitoneal fibrosis. We present a case to add further information to the diagnosis and treatment of this challenging disease.Case report:A 60-year-old male patient presented with painless jaundice. Prior medical history showed diabetes mellitus type I, high blood pressure, and deep vein thrombosis. Diagnostic investigations were strongly suspicious of a Klatskin tumor, although biopsies were inconclusive. The tumor marker Carbohydrate Antigen 19-9 (CA 19-9) was elevated. Prior to the recommended surgery, the patient had two second opinions in two different university hospitals, both arguing for surgery as well. The patient received hilar resection with right hemihepatectomy. During the postoperative course, some major complications occurred, i.e. recurrent pleural effusion, abscess in the liver resection area, sepsis, ileus, and restricted liver metabolism. Treatment with prednisolone did not show any improvement. Approximately 3 months after surgery, the patient died in consequence of acute respiratory failure. Histology showed no signs of CC, but IgG4-SC could be diagnosed.Conclusion:In the case of preoperative signs of CC, differential diagnosis of IgG4-SC needs to be considered, in particular, in cases with missing histologic proof of malignant disease.

IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma

“…Some reports have described IgG4-SC cases associated with normal or only mildly elevated IgG4 serum levels that were misdiagnosed as cholangiocarcinoma, leading to an unnecessary partial hepatectomy [14, 15]. In our case, the patient was diagnosed with cholangiocarcinoma in 2018.…”

Isolated Type Immunoglobulin G4 Sclerosing Cholangitis: The Misdiagnosed Cholangiocarcinoma

“…Additional case reports [35][36][37][38] about IgG4-SC mimicking cholangiocarcinoma also demonstrated jaundice as the most prevalent initial presentation.…”

“…In regard to patients' demographic characteristics, most reported studies [15,[28][29][30][31][32][33][34][35][36][37][38] showed that IgG4-SC, as well as cholangiocarcinoma, affects middle aged to elderly patients (50 -82 years old), and are more common in males than in females.…”