Inflammatory pseudotumor of the liver

Inflammatory pseudotumor of the liver (IPL) is a rare benign neoplasm. It often masquerades as a malignancy, either primary or metastatic. We describe the case of a 71-year-old man who presented with fever and malaise. Workup revealed a cecal mass, as well as a lesion in the left lobe of the liver. Pathologic examination showed a cecal neoplasm and a hepatic inflammatory pseudotumor. The patient recovered uneventfully. This case highlights the ability of IPL to masquerade as a malignant hepatic neoplasm, and emphasizes the difficulties in diagnosis.     

Inflammatory pseudotumor of the liver and spleen diagnosed by percutaneous needle biopsy

An inflammatory pseudotumor (IPT) is a relatively rare lesion characterized by chronic infiltration of inflammatory cells and areas of fibrosis. IPTs are difficult to diagnose because of the absence of specific symptoms or of characteristic hematological or radiological findings. In this study, a case of a woman aged over 70 years was reported, who presented with a general malaise lasting more than two months. A computed tomography scan demonstrated a diffusely spread lesion of the liver with a portal vein occlusion and a splenic lesion surrounded by a soft density layer. Since the percutaneous liver biopsy showed findings that suggested an IPT, although the radiological findings did not exclude the possibility of a malignancy, we performed a percutaneous spleen biopsy to enable a more definitive diagnosis. The microscopic findings from the spleen specimen lead us to a diagnosis of IPT involving the liver and spleen. Subsequent steroid pulse therapy was effective, and rapid resolution of the disease was observed.     

Inflammatory pseudotumor of the colon causing intussusception: A case report and literature review

Inflammatory pseudotumor(IPT) is a rare spaceoccupying lesion of unknown etiology that can mimic malignancy on clinic-radiological and pathological examination. We describe a rare case of ileocecal intussusception from clinically suspected malignancy of the right colon where the patient underwent righthemicolectomy. Histopathology of the resected specimen confirmed IPT of the colon. This patient was observed to have abnormally elevated total leukocyte count and platelets before and after surgery. In an adult with intussusception associated with an abdominal mass, the possibility of IPT of the colon should be considered. Considering the abnormally high total leukocyte and platelet counts and colonic IPT, it is necessary to prevent postoperative adverse effects due to these changes. Although IPT of the colon is usually a benign process, controversy regarding its management still exists. We consider hemicolectomy as a safe treatment approach for colonic IPT and review the existing literature.     

Long-term outcome of autoimmune pancreatitis

Purpose  Autoimmune pancreatitis (AIP) is a unique form of pancreatitis and can be complicated with various extrapancreatic lesions. Little is known about the long-term clinical course of AIP. Here we aimed to document the clinical course of AIP. Methods  For this study, we recruited 21 patients, averaging 66.5 years in age (range, 19–84 years) and observed them at a mean interval of 40.8 months (range, 18–130 months). Three of the patients were also diagnosed with retroperitoneal fibrosis, 3 had sialoadenitis, 2 had chronic thyroiditis, 1 had interstitial nephritis, and 1 had interstitial pneumonia. Three of the patients underwent surgical therapy, 12 patients received methylprednisolone (PSL) treatment, and the 6 remaining patients received no treatment. Results  Enlargement of the pancreas was attenuated in all the PSL-treated patients. Seven of the 21 patients showed pancreatic atrophy, of whom 2 were non-PSL-treated patients. Three patients developed chronic pancreatitis. One patient was diagnosed with pancreatic cancer after 50 months of PSL therapy. Conclusions  As with chronic pancreatitis patients, AIP patients should be observed closely for abnormality in pancreatic function.     

自身免疫性胰腺炎诊治进展

自身免疫性胰腺炎(AIP)是一种由自身免疫介导、以胰腺肿大及胰管不规则狭窄为特征的胰腺慢性炎症性疾病,病理以胰管纤维化伴有淋巴浆细胞浸润为特征,其治疗尚缺乏统一标准。本文就自身免疫性胰腺炎的流行病学、发病机制、临床特点、影像学检查、病理学特点、诊断标准、治疗及预后七个方面的相关研究进展进行综述,以加深对其认识及了解。     

自身免疫性胰腺炎的研究进展

自身免疫性胰腺炎是一种特殊类型的慢性胰腺炎,在较长时间的临床实践中没有认清其本质.近年来,随着研究的深入,发现自身免疫性胰腺炎与其他胰腺炎相比较有所不同,呈现多种临床特征,因此作为一个独立的临床疾病,受到越来越多的关注.现将近几年来关于自身免疫性胰腺炎的发病机制、临床表现、诊断和治疗进展作一综述.     

自身免疫性胰腺炎的内镜超声表现

目的 分析总结自身免疫性胰腺炎在内镜超声的特征表现.方法 回顾分析2004年8月至2007年9月确诊的11例原发性自身免疫性胰腺炎的临床资料,总结内镜超声的影像特点.结果 内镜超声下见胰腺实质回声极低,胰腺弥漫或局部增大,边缘呈波浪样改变;肝外胆管扩张,胆管壁明显增厚,呈均匀中低回声;可见胰腺周边淋巴结肿大.所有病例均未见明显胰管扩张或胰石、胰腺钙化和囊肿形成;未见周边血管受累.结论 自身免疫性胰腺炎在内镜超声下具有相对特征性的表现,有助于自身免疫性胰腺炎的临床诊断.     

Corticosteroids prevent the progression of autoimmune pancreatitis to chronic pancreatitis

Background/objectivesPatients with autoimmune pancreatitis (AIP) sometimes progress to chronic pancreatitis (CP). We evaluated the ability of corticosteroids to prevent the progression to CP.MethodsWe defined patients with definitive findings of CP (stones in the main pancreatic duct [MPD] or multiple pancreatic calcifications) as having severe calcification (SC). A total of 145 AIP patients were enrolled. We measured the duration between AIP diagnosis and SC development and retrospectively compared the time to SC development between patients with and without steroids. Multivariate analysis for factors associated with SC were performed.ResultsNineteen (13%) patients progressed to SC. Since 95 patients had pancreatic head swelling and SC was found in these patients only, our analysis focused mainly on these at-risk populations. In Kaplan-Meier analysis limited to patients with pancreatic head swelling, the incidence of SC was significantly lower in patients with steroids than in those without (hazard ratio [HR] 0.18, 95% confidence interval [CI] 0.07–0.52; p < 0.001). Multivariate testing of patients with pancreatic head swelling confirmed that steroid therapy was significantly associated with a lower incidence of SC (HR 0.11, 95% CI 0.03–0.34; p < 0.001), while MPD dilation at AIP diagnosis was related to a higher incidence of SC (HR 4.02, 95% CI 1.43–11.7; p = 0.009).ConclusionsCorticosteroids appeared to prevent progression to CP in AIP patients, especially in those with pancreatic head swelling. Patients with both pancreatic head swelling and MPD dilation at diagnosis have a higher incidence of progression to CP. Steroid therapy is suggested for these high-risk cases.     

Pyogenic liver abscess after choledochoduodenostomy for biliary obstruction caused by autoimmune pancreatitis

INTRODUCTION The differentiation of autoimmune pancreatitis (AIP) from pancreatic cancer is sometimes difficult because these diseases share many clinical features[1]. When pancreatic cancer cannot be ruled out, laparotomy or pancreatic resection may be p…     

Differences between diffuse and focal autoimmune pancreatitis

AIM: To investigate differences in clinical features between diffuse- and focal-type autoimmune pancreatitis (AIP).METHODS: Based on radiological findings by computed tomography and/or magnetic resonance imaging, we divided 67 AIP patients into diffuse type (D type) and focal type (F type). We further divided F type into head type (H type) and body and/or tail type (B/T type) according to the location of enlargement. Finally, we classified the 67 AIP patients into three groups: D type, H type and B/T type. We compared the three types of AIP in terms of clinical, laboratory, radiological, functional and histological findings and clinical course.RESULTS: There were 34 patients with D-type, 19 with H-type and 14 with B/T-type AIP. Although obstructive jaundice was frequently detected in D-type patients (88%) and H-type patients (68%), no B/T-type patients showed jaundice as an initial symptom (P < 0.001). There were no differences in frequency of abdominal pain, but acute pancreatitis was associated more frequently in B/T-type patients (36%) than in D-type patients (3%) (P = 0.017). Serum immunoglobulin G (IgG)4 levels were significantly higher in D-type patients (median 309 mg/dL) than in B/T-type patients (133.5 mg/dL) (P = 0.042). Serum amylase levels in B/T-type patients (median: 114 IU/L) were significantly greater than in H-type patients (72 IU/L) (P = 0.049). Lymphoplasmacytic sclerosing pancreatitis (LPSP) was histologically confirmed in 6 D-type, 7 H-type and 4 B/T-type patients; idiopathic duct-centric pancreatitis was observed in no patients. Marked fibrosis and abundant infiltration of CD20-positive B lymphocytes with few IgG4-positive plasma cells were detected in 2 B/T-type patients. Steroid therapy was effective in all 50 patients (31 D type, 13 H type and 6 B/T type). Although AIP relapsed during tapering or after stopping steroids in 3 D-type and 3 H-type patients, no patients relapsed in B/T type. During follow-up, radiological features of 6 B/T-type patients were not changed and 1 B/T-type patient improved naturally.CONCLUSION: Clinical features of H-type AIP were similar to those of D-type, but B/T-type differed from D and H types. B/T-type may involve diseases other than LPSP.     

Inflammatory pseudotumor of the liver with primary sclerosing cholangitis

Inflammatory pseudotumor (IPT) of the liver is a rare benign variant of hepatic masses, and its exact etiology has not been elucidated. We report a case of IPT associated with primary sclerosing cholangitis (PSC). The patient was a 50-year-old man admitted to our hospital because of jaundice. Abdominal ultrasonography (US) and computed tomography showed multiple dilations of the intrahepatic bile ducts and multiple masses in the liver. On magnetic resonance imaging, the masses were slightly hypointense on T1-weighted images and slightly hyperintense on T2-weighted images. On T1-weighted images after the bolus infusion of Gd chelate, the masses had no contrast enhancement, and they were hypointense in the arterial phase and portal venous phase. However, they were slightly enhanced and became almost isointense relative to the surrounding normal liver parenchyma in the delayed phase. Endoscopic retrograde cholangiography demonstrated multiple irregular strictures and dilations of the intrahepatic bile ducts. Angiography demonstrated no abnormal findings, but, interestingly, subsequent dynamic CO₂-enhanced US showed a strongly hyperechoic string, indicating that an artery had penetrated through the hypoechoic mass. A US-guided percutaneous needle biopsy revealed that the lesions were morphologically comparable to IPT. After cholangiography and microscopic analysis of the tumor, the final diagnosis was determined to be IPT of the liver with PSC. A number of previous reports have suggested a possible relationship between IPT and PSC, based on pathological findings. This report confirmed, based on clinical findings, that PSC is one of the causes of hepatic IPT. Received: May 12, 1999 / Accepted: October 22, 1999     

Inflammatory pseudotumor of the pancreas

Summary We describe a rare example of inflammatory pseudotumor of the pancreas in a 42-yr-old woman, which developed following chemotherapy for lymphoma of the uterine cervix. The patient had developed fatigue, weight loss abdominal pain, and anemia; abdominal CT scan showed a large mass in the pancreas. Examination of the resected specimen revealed a fleshy, well-circumscribed, 7-cm mass., Histologically, there was a hypocellular to moderately hypercellular, bland spindle-cell proliferation admixed with a prominent infiltrate of lymphocytes, histiocytes, and plasma cells. The spindle cells were vimentin positive but negative for muscle markers; electron microscopy revealed only fibroblastic cells. DNA analysis revealed a diploid population with low S-phase fraction. The patient was well at 6-mo follow-up. It is important for the pathologist to be aware of the existence of this entity in unusual locations such as the pancreas so as to avoid a mistaken diagnosis of malignancy.     

Long-term outcomes of autoimmune pancreatitis

Autoimmune pancreatitis(AIP) has been considered a favorable-prognosis disease; however,currently,there is limited information on natural course of AIP during long-term follow-up. Recently published studies regarding the long-term outcomes of AIP has demonstrated the developments of pancreatic stone formation,exocrine insufficiency,and endocrine insufficiency are observed in 5%-41%,34%-82%,and 38%-57% of patients having the disease. Furthermore,the incidence rate of developing pancreatic cancer ranges from 0% to 4.8% during the long-term followup. The event of death from AIP-related complications other than accompanying cancer is likely to be rare. During follow-up of AIP patients,careful surveillance for not only relapse of the disease but also development of complications at regular intervals is needed.     

Inflammatory pseudotumor of the liver: Radiologic diagnosis

To determine the characteristic radiologic findings of inflammatory pseudotumor of the liver, various imagings of ten patients (11 lesions) with proven diagnoses of inflammatory pseudotumor were reviewed. Radiologic examinations, i.e., computed tomography (CT; 11 lesions), ultrasonography (11 lesions), magnetic resonance imaging (MRI; 6 lesions), angiography (10 lesions), CT during arterio-portography (CTAP; 3 lesions), and gallium-67 scans (9 lesions) were analyzed for their utility in diagnosis. No inflammatory pseudotumor showed a fibrous capsule around the lesion. Ten of the 11 lesions were poorly demarcated on most of the imagings, and all 11 lesions showed delayed and/or prolonged enhancement on CT or MRI. Arterio-portal shunting was observed in 4 lesions after contrast material administration on CT or angiography. Central lesions with suspiciously high fibrotic tissue content were demonstrated in 5 lesions on CT or MRI. Major vessels coursing in the lesions were demonstrated in 4 lesions by CT, MRI, and CTAP. Inflammatory pseudotumor of the liver should be included in the differential diagnosis in patients with hepatic masses, even if the patients are asymptomatic. If radiologic examinations suggest inflammatory pseudotumor, percutaneous biopsies should be performed so that unnecessary surgery can be avoided.     

Inflammatory pseudotumor of the liver revealing gynecological Corynebacterium infection

Inflammatory pseudotumor (IPL) of the liver is a rare benign clinicopathologic entity, which has been associated with infectious conditions, including alimentary, digestive tract and mouth infections. We report a new case, where a patient developed hepatic IPL that revealed endometritis related to Corynebacterium infection; the patient had a favorable outcome of hepatic IPL after appropriate antibiotic therapy initiation. Our findings therefore confirm that antibiotic therapy may be effective in patients with hepatic IPL associated with underlying infections, avoiding unnecessary surgery. Our data also indicate that when hepatic IPL is observed, a complete clinical evaluation, including gynecological evaluation, should be systematically performed.     

肺炎性假瘤20例临床分析

目的探讨肺炎性假瘤的特点及临床处理。方法回顾分析20例肺炎性假瘤病例的临床表现,综合其临床特点。结果肺炎性假瘤患者约半数有呼吸道症状,X线及CT表现缺乏特异性,易与肺癌等相混淆。结论肺炎性假瘤临床诊断不易,对不明性质的肺部肿块,抗炎或抗痨治疗2周以上无效者,宜手术切除,以防肺癌漏诊。     

Hypothyroidism in patients with autoimmune pancreatitis

AIM To examine thyroid function and clinical features of hypothyroidism in autoimmune pancreatitis(AIP) patients.METHODS We examined thyroid function in 77 patients with type 1 AIP(50 males, 27 females; median age 68 years, range 33-85) diagnosed according to the Japanese diagnostic criteria for AIP 2011. We compared clinical and serological findings between patients with and without various categories of hypothyroidism. The change in hypothyroidism after steroid therapy was also examined. RESULTS Eight patients(10%) had hypothyroidism of 6 patients had subclinical hypothyroidism with a normal serum free thyroxine(FT4) and high thyroid stimulating hormone(TSH) level, and 2 patients had central hypothyroidism with low serum free triiodothyronine(FT3), FT4 and TSH levels. A significant goiter of the thyroid was not observed in any patient. There were no significant differences in age; male to female ratio; serum concentrations of IgG and IgG 4-related disease(IgG4-RD); presence of antithyroglobulin antibody, antinuclear antigen or rheumatoid factor; or presence of extrapancreatic lesions between the 6 patients with subclinical hypothyroidism and patients with euthyroidism. After steroid therapy, both subclinical and central hypothyroidism improved with improvement of the AIP.CONCLUSION Hypothyroidism was observed in 8(10%) of 77 AIP patients and was subclinical in 6 patients and central in 2 patients. Further studies are necessary to clarify whether this subclinical hypothyroidism is another manifestation of IgG4-RD.     

Sclerosing cholecystitis associated with autoimmune pancreatitis

AIM: To evaluate the histopathological and radiological findings of the gallbladder in patients with autoimmune pancreatitis (AIP). METHODS: The radiological findings of the gallbladder of 19 AIP patients were retrospectively reviewed. Resected gallbladders of 8 AIP patients were examined histologically and were immunostained with anti- IgG4 antibody. Controls consisted of gallbladders resected for symptomatic gallstones (n = 10) and those removed during pancreatoduodenectomy for pancreatic carcinoma (n = 10), as well as extrahepatic bile ducts and pancreases removed by pancreatoduodenectomy for pancreatic carcinoma (n = 10). RESULTS: Thickening of the gallbladder wall was detected by ultrasound and/or computed tomography in 10 patients with AIP (3 severe and 7 moderate); in these patients severe stenosis of the extrahepatic bile duct was also noted. Histologically, thickening of the gallbladder was detected in 6 of 8 (75%) patients with AIP; 4 cases had transmural lymphoplasmacytic infiltration with fibrosis, and 2 cases had mucosal-based lymphoplasmacytic infiltration. Considerable transmural thickening of the extrahepatic bile duct wall with dense fibrosis and diffuse lymphoplasmacytic infiltration was detected in 7 patients. Immunohistochemically, severe or moderate infiltration of IgG4-positive plasma cells was detected in the gallbladder, bile duct, and pancreas of all 8 patients, but was not detected in controls. CONCLUSION: Gallbladder wall thickening with fibrosis and abundant infiltration of IgG4-positive plasma cells is frequently detected in patients with AIR We propose the use of a new term, sclerosing cholecystitis, for these cases that are induced by the same mechanism as sclerosing pancreatitis or sclerosing cholangitis in AIR