Inflammatory pseudotumors mimicking intrahepatic cholangiocarcinoma of the liver; IgG4-positivity and its clinical significance

Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, Keimyung University Dongsan Medical Center, 56 Dalseong-ro, Jung-gu, Daegu 700-712, Republic of Korea.
Journal of Hepato-Biliary-Pancreatic Sciences (Impact Factor: 2.99). 09/2011; 19(4):405-12. DOI: 10.1007/s00534-011-0436-z
Source: PubMed


Inflammatory pseudotumor (IPT) of the liver is a benign condition with a good prognosis. However, it is difficult to distinguish clinical and radiological findings of IPT from those of malignancies. The aims of this study are to determine the clinical, radiological, and pathological characteristics, particularly relating to the role of the autoimmune mechanism in the development of inflammatory pseudotumours (IPTs) of the liver, and to suggest appropriate diagnostic and therapeutic strategy.
The clinical, diagnostic, and pathological characteristics including IgG4 immunohistochemical stain and follow-up data of 22 IPT patients were retrospectively analyzed.
The patients were 16 men and 6 women with a mean age of 59 years. Fifteen patients (68.2%) had associated biliary diseases. Of the 16 patients treated conservatively, the masses completely resolved in 10 patients, and reduced in size in 5 patients within the first 6 months. The remaining 6 patients were treated by surgical resection. IgG4 staining of 17 tissue samples from 22 patients were negative, most of the infiltrate being of fibrohistiocytic type, whereas 4 of 5 lymphoplasmacytic cells of dominant tumors show positive staining of IgG4. Although IgG4-related sclerosing cholangitis was mostly of lymphoplasmacytic type, other histological and clinical characteristics were similar in both types of IPTs.
IPTs of the liver can be diagnosed based on radiological and pathological findings by needle biopsy. Although the lymphoplasmacytic type of IPTs seems to correspond to IgG4-related disease, as assessed by IgG4 immunohistochemical stain, its clinical significance is unknown. Although most IPTs can be resolved with conservative therapy, surgical resection should be considered in cases of uncertain biopsy result, presumed malignant lesion, combination with other pathology, or lack of response to conservative management.

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    • "The etiology of IPT is unclear, but predominantly inflammatory pattern of pathology and the associated laboratory findings suggest an underlying infection via hepatobiliary tract in several studies.2,5 Previous studies reported that patients had hepatobiliary tract disease such as biliary stones, history of liver resection, cholangitis, liver abscess, hepatobiliary malignancy in 68% to 80%,17,20-23 and abdominal pain, fever, elevated inflammatory markers including ESR, CRP, leukocyte count were common in cases with IPTs of liver.2,17,20,21 These findings support the underlying infectious condition as etiology of IPTs. "
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    ABSTRACT: Inflammatory pseudotumor (IPT) of the liver is a rare disease characterized by chronic infiltration of inflammatory cells. However, the clinical characteristics and outcomes of IPT remain uncertain. Clinical features, image findings, and outcomes of 55 patients with histologically proven IPT were evaluated. They consisted of 26 men and 19 women with median age of 65 years. Serum carcinoembryonal antigen and carbohydrate antigen 19-9 levels were normal in 42 patients (93.3%). Enhanced CT scans indicated poorly defined peripheral enhancement (82.5%) at the arterial phase and poorly defined hyperattenuating lesions with internal hypoattenuating areas at the equilibrium phase (77.0%). Gadolinium-enhancement MRI revealed poorly defined peripheral rim-like enhancement (77.8%). Ten patients underwent surgical resection and 35 were treated conservatively with or without antibiotics. No recurrence was noted after surgical resection during follow-up (1 to 48 months). In all patients who received conservative treatment, complete resolution or size reduction was noted during follow-up (1 to 192 months). CT and MRI provide clues to the diagnosis of IPT in patients with liver masses and normal tumor markers. However, due to the lack of pathognomonic findings, the clinician's suspicion and histological diagnosis are necessary to make an accurate diagnosis of IPT.
    Gut and Liver 01/2014; 8(1):58-63. DOI:10.5009/gnl.2014.8.1.58 · 1.81 Impact Factor
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    • "Recently, there are several reports that hepatic inflammatory pseudotumor is associated with IgG4-related SC, in which extrahepatic manifestations were reported including lymph node swelling, sialoadenitits, and pancreatitis.5,11-13 However, cholecystitis mimicking GB cancer has never been reported like in this case. "
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    ABSTRACT: Immunoglobulin G4 (IgG4)-related disease is a novel disease entity that can involve diverse organs, causing specific diseases, including autoimmune pancreatitis, sclerosing cholangitis, cholecystitis, inflammatory aortic aneurysm, and inflammatory pseudotumor. IgG4-related disease is characterized by elevated serum IgG4 concentrations, abundant IgG4 lymphoplasmacytic infiltration, and dramatic steroid responses. It is clinically important to differentiate this rare disease from primary sclerosing cholangitis and cholangiocarcinoma, because the treatment and prognosis of these two diseases are completely different. However, the preoperative diagnosis is challenging, and the disease is frequently misdiagnosed. If the serum level of IgG4 is within the normal range, the diagnosis of IgG4-related disease is more difficult. This article reports on a 59-year-old man with IgG4-related disease mimicking unresectable gallbladder cancer with normal serum IgG4 concentrations.
    Gut and liver 09/2013; 7(5):616-20. DOI:10.5009/gnl.2013.7.5.616 · 1.81 Impact Factor
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    ABSTRACT: Inflammatory pseudotumours are rare benign tumours that can present as solid and potentially malignant tumours on diagnostic images. A 59-year-old male presented at the emergency department with continuous pain in his right upper abdomen, attacks of colic and intermittent fever. CT scans of the abdomen revealed a solid laesion in the liver. Histopathological analysis of a biopsy showed an inflammatory pseudotumour. Since the pain persisted and a malignancy could not be excluded with certainty, surgical resection of the tumour was performed. The specimen showed an inflammatory pseudotumour with an increase in IgG4 positive plasma cells as well as a purulent infection around aggregates of Actinomyces. It is important to realise that a pseudotumour caused by an inflammatory reaction can mimic a malignancy.
    Nederlands tijdschrift voor geneeskunde 02/2013; 157(7):A5277.
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