Incidence, clinical presentation and pathological features of benign sclerosing cholangitis of unknown origin masquerading as biliary carcinoma.
BACKGROUND
Benign obstructions of the liver hilum are occasionally encountered in surgically resected cases. Some of these cases are pathologically classified as benign sclerosing cholangitis and are not clearly categorized. This study aims to elucidate the clinicopathological features of benign sclerosing cholangitis of unknown origin.


METHODS
Patients who had undergone surgery of the proximal bile duct from 1993 to 2008 on suspicion of proximal bile carcinoma were evaluated, then their clinical presentation, histopathological and immunohistochemical findings were retrospectively analyzed.


RESULTS
One hundred seventy-six patients underwent surgery during this period. Of these, five patients were diagnosed with benign sclerosing cholangitis of unknown origin. All five patients were middle-aged with no history of autoimmune disease. One patient died of recurrent cholangitis after surgery. Histopathologically, lymphoplasmacytic infiltrations were observed in all five cases. Among them, the clinicopathological findings of one patient were compatible with IgG4-related sclerosing cholangitis, and the other two patients had unusual histopathological findings with prominent lymph follicular formation.


CONCLUSIONS
Although patients with benign sclerosing cholangitis of unknown origin comprise a heterogeneous population, they can be further classified into subgroups. The response to immunosuppressive therapy, long-term prognosis and histological morphology of the disease should be considered in order to classify these cases of sclerosing cholangitis.
Fujita
Takeo
T
Division of Digestive Surgery, National Cancer Center Hospital East, 6-5-1 Kashiwanoha, Kashiwa, Chiba 277-8577, Japan. takfujit@east.ncc.go.jp
Kojima
Motohiro
M
Gotohda
Naoto
N
Takahashi
Shinichiro
S
Nakagohri
Toshio
T
Konishi
Masaru
M
Ochiai
Atsushi
A
Kinoshita
Taira
T
D000328:Adult; D000368:Aged; D001650:Bile Duct Neoplasms / Q000175:diagnosis*; D001652:Bile Ducts / Q000378:metabolism / Q000473:pathology*; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D002763:Cholecystectomy; D002779:Cholestasis / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007150:Immunohistochemistry; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
D007074:Immunoglobulin G