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01-04-2009 | original article

Fibroinflammatory Biliary Stricture: A Rare Bile Duct Lesion Masquerading as Cholangiocarcinoma

Authors: T. Clark Gamblin, A. M. Krasinskas, A. S. Slivka, M. E. Tublin, Jake Demetris, Eveline Shue, Susan Caro, J. Wallis Marsh, A. James Moser

Published in: Journal of Gastrointestinal Surgery | Issue 4/2009

Abstract

Introduction

Fibroinflammatory biliary stricture (FIBS) is a rare benign tumor-like process of the extrahepatic bile duct that masquerades as cholangiocarcinoma.

Methods

In order to distinguish this unusual entity from cancer, we performed a systematic analysis of 11 patients with FIBS. All patients presented with jaundice; six patients had coexisting autoimmune disease. Preoperative evaluation included computed tomography scan and endoscopic retrograde cholangiopancreatography with benign brush cytology. Surgical treatment included nine bile duct resections with five concurrent liver resections and two incisional biopsies. Light microscopy demonstrated fibrous lesions admixed with chronic inflammation.

Results and discussion

Immunohistochemistry demonstrated smooth muscle actin expression in all lesions except one; five tumors exhibited IgG4 positive plasma cells. The lesions were negative for cytokeratin, ALK1, CD21, S100, Ki67, and p53. Six patients received postoperative immunosuppression. At 41 month median follow-up (range 15–58 months), there was no evidence of recurrent FIBS in ten patients, while one was lost to follow-up.

Conclusion

FIBS is a rare myofibroblastic lesion with an immunohistochemical profile distinct from other epithelial and stromal neoplasms of the extrahepatic bile duct. A subset of these cases appear to represent IgG4-related sclerosing cholangitis. Because preoperative cytology is not diagnostic of FIBS, surgical resection remains the mainstay of diagnosis and treatment, while immunosuppression may reduce the risk of recurrence.

Chen YK, Pleskow DK. SpyGlass single-operator peroral cholangiopancreatoscopy system for the diagnosis and therapy of bile-duct disorders: a clinical feasibility study (with video). Gastrointest Endosc 2007;65(6):832–841. doi:10.1016/j.gie.2007.01.025.PubMedCrossRef

Nishiyama Y, Yamamoto Y et al. Comparison of early and delayed FDG PET for evaluation of biliary stricture. Nucl Med Commun 2007;28(12):914–919.PubMedCrossRef

Kim JY, Lee JM et al. Contrast-enhanced MRI combined with MR cholangiopancreatography for the evaluation of patients with biliary strictures: differentiation of malignant from benign bile duct strictures. J Magn Reson Imaging 2007;26(2):304–312.PubMedCrossRef

Khalid A, Pal R, Sasatomi E, Swalsky P, Slivka A, Whitcomb D, Finkelstein S. Use of microsatellite marker loss of heterozygosity in accurate diagnosis of pancreaticobiliary malignancy from brush cytology samples. GUT 2004;53:1860–1865.PubMedCrossRef

Hadjis NS, Collier NA, Blumgart LH. Malignant masquerade at the hilum of the liver. Br J Surg 1985;72(8):659–661. Aug.PubMedCrossRef

Corvera CU, Blumgart LH, Darvishian F, Klimstra DS, DeMatteo R, Fong Y, D’Angelica M, Jarnagin WR. Clinical and pathologic features of proximal biliary strictures masquerading as hilar cholangiocarcinoma. J Am Coll Surg 2005;201(6):862–869. Epub 2005 Oct 13, Dec.PubMedCrossRef

Verbeek PC, van Leeuwen DJ, de Wit LT, Reeders JWAJ, Smits NJ, Bosma A, Huibregtse K, van der Heyde MN. Benign fibrosing disease at the hepatic confluence mimicking Klatskin tumors. Surgery 1992;112:866–870.PubMed

Wetter LA, Ring EJ, Pellegrini CA, Way LW. Differential diagnosis of Sclerosing cholangiocarcinomas of the common hepatic duct (Klatskin tumors). Am J Surg 1991;161:57–63.PubMedCrossRef

Standfield NJ, Salisbury JR, Howard ER. Benign non-traumatic inflammatory strictures of the extrahepatic biliary system. Br J Surg 1989;76:849–852.PubMedCrossRef

10.

Kram MT, May LD, Cooperman A et al. Lymphoplasmacytic sclerosing pancreatitis and cholangitis. Gastrointest Endosc 2002;55:588–590.PubMedCrossRef

11.

Hawegawa K, Kubota K, Komatsu Y et al. Mass-forming inflammatory periductal fibrosis mimicking hilar bile duct carcinoma. Hepatogastroenterology 2000;47:1230–1233.

12.

Graham SM, Barwick K, Cahow CE, Baker CC. Cholangitis glandularis proliferans. A histologic variant of primary sclerosing cholangitis with distinctive clinical and pathological features. J Clin Gastroenterol 1988;10:579–583.PubMedCrossRef

13.

Krukowski ZH, McPhie JL et al. Proliferative cholangitis (cholangitis glandularis proliferans). Br J Surg 1983;70(3):166–171.PubMedCrossRef

14.

Ozeki Y, Matsubara N et al. [Inflammatory tumor of the hepatic hilus mimicking bile duct cancer–report of a case]. Nippon Geka Gakkai Zasshi 1993;94(9):1064–1067.PubMed

15.

Hamdi I, Marzouk I et al. [Inflammatory pseudotumor of the spleen and radiopathologic correlation]. J Radiol 2006;87(12 Pt 1):1894–1896.PubMedCrossRef

16.

Locke JE, Choti MA et al. Inflammatory pseudotumor of the liver. J Hepatobiliary Pancreat Surg 2005;12(4):314–316.PubMedCrossRef

17.

Dehner LP. Extrapulmonary inflammatory myofibroblastic tumor: the inflammatory pseudotumor as another expression of the fibrohistiocytic complex (Abstract). Lab Invest 1986;54:15. [typically, abstracts are not cited] [a better older reference might be: Dehner LP.

18.

Zhang L, Notohara K, Levy MJ, Chari ST, Smyrk TC. IgG4-positive plasma cell infiltration in the diagnosis of autoimmune pancreatitis. Mod Pathol 2006, Sept 15.

19.

Palazzo JP, Chang CD. Inflammatory pseudotumor of the pancreas. Histopathology 1993;23:86–91.CrossRef

20.

Jepsen P, Vilstrup H et al. Incidence rates of intra- and extrahepatic cholangiocarcinomas in Denmark from 1978 through 2002. J Natl Cancer Inst 2007;99(11):895–897.PubMedCrossRef

21.

Qin XL, Wang ZR et al. Utility of serum CA19-9 in diagnosis of cholangiocarcinoma: in comparison with CEA. World J Gastroenterol 2004;10(3):427–432.PubMed

22.

Are C, Gonen M et al. Differential diagnosis of proximal biliary obstruction. Surgery 2006;140(5):756–763.PubMedCrossRef

23.

Nonmura A, Minato H, Shimizu K, Kadoya M, Matsui O. Hepatic hilar inflammatory pseudotumor mimicking cholangiocarcinoma with cholangitis and phlebitis: a variant of primary sclerosing cholangitis? Pathol Res Pract 1997;193:519–525.

24.

Bahadori M, Liebow AA. Plasma cell granuloma of the lung. Cancer 1973;31:191–208.PubMedCrossRef

25.

Gonzalez-Crussi F, deMello DE, Sotelo-Avilla C. Omental mesenteric myxoid hamartomas: infantile lesions simulating malignant tumors. Am J Surg Pathol 1983;7(6):567–578.PubMed

26.

Pisciotto PT, Gray GF, Miller DR. Abdominal plasma cell pseudotumor. J Pediatr 1978;93:628–630.PubMedCrossRef

27.

Tang TT, Segura AD, Oechler MD et al. Inflammatory myofibrohistiocytic proliferation simulating sarcoma in children. Cancer 1990;65:1626–1634.PubMedCrossRef

28.

Umiker WO, Iverson L. Post inflammatory tumors of the lung: report of four cases simulating xanthoma, fibroma or plasma cell tumor. J Thorac Surg 1954;28:55–62.PubMed

29.

Shek TWH, Ho FCS, Ng IOC et al. Follicular dendritic cell tumor of the liver. Evidence for an Ebstein-Barr virus related proliferation of follicular dendritic cells. Am J Surg Pathol 1996;20(3):313–324.PubMedCrossRef

30.

Fukushima N, Suzuki M et al. A case of inflammatory pseudotumour of the common bile duct. Virchows Arch 1997;431(3):219–224.PubMedCrossRef

31.

Meis JM, Enzinger FM. Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor. Am J Surg Pathol 1991;15:1146–1156.PubMedCrossRef

32.

Coffin Cm, Watterson J, Priest JR et al. Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathological and immunohistochemical study of 84 cases. Am J Surg Pathol 1995;19(8):859–872.PubMed

33.

Donner L, Tompler R, White R. Progression of inflammatory myofibroblastic tumor (inflammatory pseudotumor) of soft tissue into sarcoma after several recurrences. Hum Pathol 1996;27:1096–1098.CrossRef

34.

Spencer H. The pulmonary plasma cell/histiocytoma complex. Histopathology 1984;8:90316.

35.

Treissman SP, Gillis DA, Lee CLY et al. Omental–mesenteric inflammatory pseudotumor. Cytogenetic demonstration of genetic changes and monclonality in one tumor. Cancer 1994;73:1433–1437.PubMedCrossRef

36.

Zavaglia C, Barberis M, Gelosa F. Inflammatory pseudotumor of the liver with malignant transformation. Report of two cases. Ital J Gastroenterol 1996;28(3):152–159.PubMed

37.

Chiarle R, Voena C et al. The anaplastic lymphoma kinase in the pathogenesis of cancer. Nat Rev Cancer 2008;8(1):11–23.PubMedCrossRef

38.

Zen Y, Harada K, Sasaki M, Sato Y, Tsuneyama K, Haratake J, Kurumaya H, Katayanagi K, Masuda S, Niwa H, Morimoto H, Miwa A, Uchiyama A, Portmann BC, Nakanuma Y. IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis. Am J Surg Pathol 2004;28(9):1193–1203. Sep.PubMedCrossRef

39.

Hamano H, Kawa S, Uehara T, Ochi Y, Takayama M, Komatsu K, Muraki T, Umino J, Kiyosawa K, Miyagawa S. Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis. Gastrointest Endosc 2005;62(1):152–157. Jul.PubMedCrossRef

40.

Kamisawa T, Nakajima H, Egawa N, Funata N, Tsuruta K, Okamoto A. IgG4-related sclerosing disease incorporating sclerosing pancreatitis, cholangitis, sialadenitis and retroperitoneal fibrosis with lymphadenopathy. Pancreatology 2006;6(1–2):132–137.PubMedCrossRef

41.

Stathopoulos G, Nourmand AD, Blackstone M, Anderson D, Baker AL. Rapidly progressive sclerosing cholangitis following surgical treatment of pancreatic pseudotumor. J Clin Gastroenterol 1995;21(2):143–148.PubMedCrossRef

42.

Weisner RH, Grambsch PM, Dickson ER et al. Primary sclerosing cholangitis: natural history, prognostic factors and survival analysis. Hepatology 1989;10:430–436.CrossRef

43.

Knox T, Kaplan M. A double-blind controlled trial of oral-pulse methotrexate therapy in the treatment of primary sclerosing cholangitis. Gastroenterology 1994;106:494–499.PubMed

44.

Zen Y, Fujii T et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology 2007;45(6):1538–1546.PubMedCrossRef

45.

Arber A, Kamel OW, Van de Rijn M et al. Frequent presence of Ebstein–Barr virus in inflammatory pseudotumor. Hum Pathol 1995;26:1093–1098.PubMedCrossRef

46.

Selves J, Meggetto F, Bousset P et al. Inflammatory pseudotumor of the liver. Evidence for follicular dendritic reticulum cell proliferation associated with clonal Epstein–Barr virus. Am J Surg Pathol 1996;20:747–753.PubMedCrossRef

47.

Borkham-Kamphorst E, Stoll D et al. Inhibitory effect of soluble PDGF-beta receptor in culture-activated hepatic stellate cells. Biochem Biophys Res Commun 2004;317(2):451–462.PubMedCrossRef

48.

Wakabayashi T, Kawaura Y et al. Long-term prognosis of duct-narrowing chronic pancreatitis: strategy for steroid treatment. Pancreas 2005;30(1):31–39.PubMed

49.

Nishino T, Toki F et al. Long-term outcome of autoimmune pancreatitis after oral prednisolone therapy. Intern Med 2006;45(8):497–501.PubMedCrossRef

Title: Fibroinflammatory Biliary Stricture: A Rare Bile Duct Lesion Masquerading as Cholangiocarcinoma
Authors: T. Clark Gamblin
A. M. Krasinskas
A. S. Slivka
M. E. Tublin
Jake Demetris
Eveline Shue
Susan Caro
J. Wallis Marsh
A. James Moser
Publication date: 01-04-2009
Publisher: Springer-Verlag
Published in: Journal of Gastrointestinal Surgery / Issue 4/2009
Print ISSN: 1091-255X
Electronic ISSN: 1873-4626
DOI: https://doi.org/10.1007/s11605-008-0750-1

At a glance: The STEP trials

Springer Medicine

Fibroinflammatory Biliary Stricture: A Rare Bile Duct Lesion Masquerading as Cholangiocarcinoma

Abstract

Introduction

Methods

Results and discussion

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Introduction

Methods

Results and discussion

Conclusion

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