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A G H, Kumar S, Singla S, Kurian N. Aggressive Inflammatory Myofibroblastic Tumor of Distal Pancreas: A Diagnostic and Surgical Challenge. Cureus 2022; 14:e22820. [PMID: 35399449 PMCID: PMC8980218 DOI: 10.7759/cureus.22820] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/03/2022] [Indexed: 11/05/2022] Open
Abstract
An inflammatory myofibroblastic tumor (IMT) is a rare soft tissue neoplasm of unknown etiology. It is a slow-growing tumor of borderline malignant potential. Distant metastases and recurrence after complete excision are rare. Establishing a preoperative diagnosis is difficult because of its nonspecific clinic-radiological features. Although the majority of cases have been reported in the lungs, it can affect any part of the body. The pancreatic inflammatory myofibroblastic tumor is very rare and only 26 cases have been reported in the medical literature. These tumors mostly arise from the head of the pancreas, whereas occurrence in the body or tail region is rather unusual. Here, we report a case of a 55-year-old male patient with a locally advanced inflammatory myofibroblastic tumor arising from the pancreatic tail. Complete excision of tumor required multi-visceral resection (distal pancreaticosplenectomy with jejunal and colonic segmental resection). The diagnosis of inflammatory myofibroblast tumor was made on the basis of histopathology and immunohistochemistry.
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Chen ZT, Lin YX, Li MX, Zhang T, Wan DL, Lin SZ. Inflammatory myofibroblastic tumor of the pancreatic neck: A case report and review of literature. World J Clin Cases 2021; 9:6418-6427. [PMID: 34435007 PMCID: PMC8362560 DOI: 10.12998/wjcc.v9.i22.6418] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/18/2021] [Revised: 03/23/2021] [Accepted: 06/01/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pancreatic inflammatory myofibroblastic tumor (IMT) is a relatively rare disease that is often confused with pancreatic cancer or pancreatic neuroendocrine tumors. The histological features of IMTs show that tissue from this type of tumor contains an intermingling of fibroblast and myofibroblast proliferation, accompanied by a varying degree of inflammatory cell infiltration.
CASE SUMMARY The management of an IMT occurring at the neck of the pancreas is presented in this paper. A 66-year-old female patient was diagnosed with a pancreatic neck mass after a series of tests. The patient underwent enucleation of the pancreatic neck tumor after a pathological diagnosis of IMT. Previous research on the clinical features, pathological diagnosis and treatment of pancreatic IMTs was reviewed. Compared with previous reports, this is a unique case of enucleation of a pancreatic IMT.
CONCLUSION The enucleation of pancreatic IMTs may be a safe and efficient surgical method for managing such tumors with a better prognosis. Further cases are required to explore surgical measures for pancreatic IMTs.
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Affiliation(s)
- Zhi-Tao Chen
- Department of Hepatobiliary and Pancreatic Surgery, Shulan (Hangzhou) Hospital Affiliated to Zhejiang Shuren University Shulan International Medical College, Hangzhou 310000, Zhejiang Province, China
- School of Medicine, Zhejiang University, Hangzhou 310000, Zhejiang Province, China
| | - Yao-Xiang Lin
- School of Medicine, Hangzhou Normal University, Hangzhou 310003, Zhejiang Province, China
| | - Meng-Xia Li
- School of Medicine, Zhejiang University, Hangzhou 310000, Zhejiang Province, China
- Department of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang Province, China
| | - Ting Zhang
- Department of Pathology, Shulan (Hangzhou) Hospital Affiliated to Zhejiang Shuren University Shulan International Medical College, Hangzhou 310000, Zhejiang Province, China
| | - Da-Long Wan
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou 310003, Zhejiang Province, China
| | - Sheng-Zhang Lin
- Department of Hepatobiliary Surgery, Shulan (Hangzhou) Hospital Affiliated to Zhejiang Shuren University Shulan International Medical College, Hangzhou 310000, Zhejiang Province, China
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A Pancreatic Inflammatory Myofibroblastic Tumor with Spontaneous Remission: A Case Report with a Literature Review. Diagnostics (Basel) 2019; 9:diagnostics9040150. [PMID: 31627359 PMCID: PMC6963339 DOI: 10.3390/diagnostics9040150] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2019] [Revised: 10/13/2019] [Accepted: 10/15/2019] [Indexed: 12/17/2022] Open
Abstract
The inflammatory myofibroblastic tumor (IMT) is a rare tumor that can develop in any systemic organ. Its features are generally benign, but it often resembles malignancies and is treated surgically. Our patient was an 82-year-old female complaining of abdominal discomfort. Computed tomography demonstrated a 5 cm, ill-enhanced mass at the pancreas head. Upper gastrointestinal endoscopy revealed a duodenal submucosal tumor with apical erosion. Endoscopic ultrasonography (EUS) demonstrated a heterogeneous, low-echoic pancreas mass without clear margins. Fine-needle aspiration biopsy (FNAB) demonstrated spindle myofibroblastic tissues with lymphoplasmacyte and eosinophil infiltration, confirming an IMT diagnosis. Surprisingly, the tumor spontaneously regressed in one month without medication. Histological diagnosis using EUS-FNAB is essential for the rare pancreatic solid tumor like IMT.
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IVIM DW-MRI of autoimmune pancreatitis: therapy monitoring and differentiation from pancreatic cancer. Eur Radiol 2015; 26:2099-106. [PMID: 26449558 DOI: 10.1007/s00330-015-4041-4] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2014] [Revised: 09/14/2015] [Accepted: 09/21/2015] [Indexed: 12/21/2022]
Abstract
OBJECTIVES To evaluate IVIM DW-MRI for changes in IVIM-derived parameters during steroid treatment of autoimmune pancreatitis (AIP) and for the differentiation from pancreatic cancer (PC). METHODS Fifteen AIP-patients, 11 healthy patients and 20 PC-patients were examined with DWI-MRI using eight b-values (50, 100, 150, 200, 300, 400, 600, 800). 12 AIP-patients underwent follow-up examinations during treatment. IVIM-parameters and ADC800-values were tested for significant differences and an ROC analysis was performed. RESULTS The perfusion fraction f was significantly lower in patients with AIP at the time of diagnosis (10.5 ± 4.3 %) than in patients without AIP (20.7 ± 4.3 %). In AIP follow-up, f increased significantly to 17.1 ± 7.0 % in the first and 21.0 ± 4.1 % in the second follow up. In PC, the f-values were lower (8.2 ± 4.0 %, n.s.) compared to initial AIP and were significantly lower compared to first and second follow-up examination. In the ROC-analysis AUC-values for f were 0.63, 0.88 and 0.98 for differentiation of PC from initial, first and second follow up AIP-examination. CONCLUSIONS The found differences in f between AIP, AIP during steroid treatment and pancreatic cancer suggest that IVIM-diffusion MRI could serve as imaging biomarker during treatment in AIP-patients and as a helpful tool for differentiation between PC and AIP. KEY POINTS • MRI is used for follow-up examinations during therapy in AIP-patients • IVIM-DWI-MRI offers parameters which reflect perfusion and true diffusion • IVIM-parameters are helpful for differentiation between AIP and pancreatic cancer • IVIM-parameters could serve as an imaging biomarker during steroid treatment.
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Possible role of TGF β1 in inflammatory pseudotumor associated with multiple neuroendocrine tumors of the small intestine. Pathol Res Pract 2015; 211:805-9. [DOI: 10.1016/j.prp.2015.06.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2014] [Revised: 05/12/2015] [Accepted: 06/01/2015] [Indexed: 12/13/2022]
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Gong S, Auer I, Duggal R, Pittaluga S, Raffeld M, Jaffe ES. Epstein-Barr virus-associated inflammatory pseudotumor presenting as a colonic mass. Hum Pathol 2015; 46:1956-61. [PMID: 26477709 DOI: 10.1016/j.humpath.2015.08.011] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2015] [Revised: 08/09/2015] [Accepted: 08/20/2015] [Indexed: 12/16/2022]
Abstract
Epstein-Barr virus (EBV)-positive inflammatory pseudotumor (IPT) commonly involves spleen and liver and has only rarely been reported in the gastrointestinal (GI) tract. The spindle cells may express myofibroblastic or follicular dendritic cell markers. We report a challenging case of EBV-positive IPT arising in the ascending colon. The lesion was composed of spindle cells positive for smooth muscle actin but negative for all follicular dendritic cell markers tested and was associated with an exuberant lymphoid proliferation containing reactive follicles, abundant plasma cells, and small lymphocytes. We further discuss pitfalls for possible misdiagnosis as ALK-positive inflammatory myofibroblastic tumor, IgG4-related disease, and peripheral T-cell lymphoma. Our case represents the first EBV-positive inflammatory pseudotumor of the GI tract in the Western literature. Awareness of this rare entity in GI tract is essential for correct diagnosis and appropriate patient management.
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Affiliation(s)
- Shunyou Gong
- Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
| | - Iwona Auer
- Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of Calgary & Calgary Laboratory Services, Calgary, AB T2N 2T9, Canada
| | - Rajan Duggal
- Department of Pathology, Medanta The Medicity, Gurgaon 122001, Delhi NCR, India
| | - Stefania Pittaluga
- Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
| | - Mark Raffeld
- Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892
| | - Elaine S Jaffe
- Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892.
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Mahajan VS, Mattoo H, Deshpande V, Pillai SS, Stone JH. IgG4-related disease. ANNUAL REVIEW OF PATHOLOGY 2014; 9:315-47. [PMID: 24111912 DOI: 10.1146/annurev-pathol-012513-104708] [Citation(s) in RCA: 248] [Impact Index Per Article: 22.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition that can affect almost any organ and is now being recognized with increasing frequency. IgG4-RD is characterized by a lymphoplasmacytic infiltrate composed of IgG4(+) plasma cells, storiform fibrosis, obliterative phlebitis, and mild to moderate eosinophilia. The diagnosis of IgG4-RD unifies many eponymous fibroinflammatory conditions that had previously been thought to be confined to single organs. IgG4-RD lesions are infiltrated by T helper cells, which likely cause progressive fibrosis and organ damage. IgG4 antibodies are generally regarded as noninflammatory. Although autoreactive IgG4 antibodies are observed in IgG4-RD, there is no evidence that they are directly pathogenic. Rituximab-induced B cell depletion in IgG4-RD leads to rapid clinical and histological improvement accompanied by swift declines in serum IgG4 concentrations. Although IgG autoantibodies against various exocrine gland antigens have been described in IgG4-RD, whether they are members of the IgG4 subclass is unknown. The contribution of autoantibodies to IgG4-RD remains unclear.
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Cohen DA, Dabbs DJ, Cooper KL, Amin M, Jones TE, Jones MW, Chivukula M, Trucco GA, Bhargava R. Interobserver agreement among pathologists for semiquantitative hormone receptor scoring in breast carcinoma. Am J Clin Pathol 2012; 138:796-802. [PMID: 23161712 DOI: 10.1309/ajcp6dkrnd5ckvdd] [Citation(s) in RCA: 69] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022] Open
Abstract
The American Society of Clinical Oncology/College of American Pathologists (ASCO/CAP) guidelines recommend reporting of hormone receptor test results in a semiquantitative manner. This study used 74 resected estrogen receptor (ER)-positive invasive breast cancers to determine reproducibility of semiquantitative scoring of hormone receptors using the H-score method. Four pathologists independently scored each slide. Agreement among observers was analyzed via Fleiss κ statistics on ER and progesterone receptor (PR) categorical scores. Intraclass correlation coefficient (ICC) was used to estimate the interobserver agreement for ER and PR H-scores on a continuous scale (0-300). There was 100% agreement for categorical ER results (κ = 1) and 97% agreement (κ = 0.823, P < .001) for categorical PR results. For quantitative H-scores, ICC agreement was 0.85 (95% confidence interval [CI] = 0.79-0.90) for ER and 0.87 (95% CI = 0.82-0.92) for PR. Because the H-score provides a continuous measure of tumor hormone receptor content, we suggest universal adoption of this method.
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Proctor RD, Rofe CJ, Bryant TJC, Hacking CN, Stedman B. Autoimmune pancreatitis: an illustrated guide to diagnosis. Clin Radiol 2012. [PMID: 23177083 DOI: 10.1016/j.crad.2012.08.016] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Autoimmune pancreatitis (AIP) remains one of the rarer forms of pancreatitis but has become increasingly well recognized and widely diagnosed as it is an important differential, particularly due to the dramatic response to appropriate therapy. It is now best considered as part of a multisystem disease and the notion of "IgG4-related systemic sclerosing disease" has become widely recognized as the number of extra-pancreatic associations of AIP grows. More recently AIP has been classified into two subtypes: lymphoplasmacytic sclerosing pancreatitis (LPSP) and idiopathic duct-centric pancreatitis (IDCP) with distinct geographical, age and sex distributions for the two subtypes, in addition to different pathological characteristics. The role of imaging is crucial in AIP and should be considered in conjunction with clinical, serological, and histopathological findings to make the diagnosis. Radiologists are uniquely placed to raise the possibility of AIP and aid the exclusion of significant differentials to allow the initiation of appropriate management and avoidance of unnecessary intervention. Radiological investigation may reveal a number of characteristic imaging findings in AIP but appearances can vary considerably and the focal form of AIP may appear as a pancreatic mass, imitating pancreatic carcinoma. This review will illustrate typical and atypical appearances of AIP on all imaging modes. Emphasis will be placed on the imaging features that are likely to prove useful in discriminating AIP from other causes prior to histopathological confirmation. In addition, examples of relevant differential diagnoses are discussed and illustrated.
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Affiliation(s)
- R D Proctor
- Department of Clinical Radiology, Royal Cornwall Hospitals NHS Trust, Truro, UK
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Zhang X, Zhang X, Li W, Jiang L, Zhang X, Guo Y, Wang X. Clinical analysis of 36 cases of autoimmune pancreatitis in China. PLoS One 2012; 7:e44808. [PMID: 23028628 PMCID: PMC3445578 DOI: 10.1371/journal.pone.0044808] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/20/2012] [Accepted: 08/14/2012] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND To improve the early identification of autoimmune pancreatitis in China by a retrospective analysis of clinical data from AIP patients. METHODOLOGY/PRINCIPAL FINDINGS The analysis included 36 patients admitted by the surgery department of our hospital from January 2003 to October 2011 whose postoperative pathological confirmations were consistent with the histological criteria of Honolulu Consensus Document. The clinical phenotypes associated with the histopathologic patterns of LPSP and IDCP were referred to as type 1 and type 2 of AIP, respectively. A retrospective analysis of clinical features, serological data, pathological findings and imageological records was performed in line with the subtypes of AIP. Type 1 showing a sex predilection (males) was commonly more dominant than type 2 in all AIP. Type 2 without a gender predilection was, on average, a decade younger than type 1. Type 1 was inferior to type 2 in ALT, ALP and γ-GT with statistical significance (P = 0.044, 0.025 and 0.013). Type 1 was inferior to type 2 in AST with difference close to statistical significance (P = 0.072). Histopathology revealed frequent lymphoplasmacytic infiltration with less frequent infiltration of neutrophils, eosinophils and fibroblasts. Diffuse and intensive interstitial fibrosis could be seen. The changes of pancreatic head were more frequently seen in type 2 than in type 1 (P = 0.05). Plasma cells staining of IgG4 at a density of over 30 or more cells per high-power field appeared to be a specific finding in China with type 1. Imageology found a diffusely or focally enlarged pancreas, most frequently a mass or enlargement in the pancreatic head, characteristic capsule-like rim, calcification or pancreatic calculus and cystic degeneration. CONCLUSIONS/SIGNIFICANCE AIP is a unique type of chronic pancreatitis and has distinctive serological, pathological and imageological characteristics, which should be used for differentiation from pancreatic cancer.
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Affiliation(s)
- Xingang Zhang
- Department of Rheumatology, Shengjing Hospital of China Medical University, Shenyang, Liaoning Province, China.
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Hassan KS, Cohen HI, Hassan FK, Hassan SK. Unusual case of pancreatic inflammatory myofibroblastic tumor associated with spontaneous splenic rupture. World J Emerg Surg 2010; 5:28. [PMID: 21092210 PMCID: PMC2995784 DOI: 10.1186/1749-7922-5-28] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2010] [Accepted: 11/22/2010] [Indexed: 12/31/2022] Open
Abstract
Background Spontaneous splenic rupture considered a relatively rare but life threatening. The three commonest causes of spontaneous splenic rupture are malignant hematological diseases, viral infections and local inflammatory and neoplastic disorders. We describe a unique and unusual case of inflammatory myofibroblastic tumor of the tail of pancreas presented with massively enlarged spleen and spontaneous splenic rupture. Case presentation A 19 years old male patient with no significant past medical history presented to emergency room with abdominal pain and fatigue. Massively enlarged spleen was detected. Hypotension and rapid reduction of hemoglobin level necessitated urgent laparatomy. About 1.75 liters of blood were found in abdominal cavity. A large tumor arising from the tail of pancreas and local rupture of an enlarged spleen adjacent to the tumor were detected. Distal pancreatectomy and splenectomy were performed. To our knowledge, we report the first case of massively enlarged spleen that was complicated with spontaneous splenic rupture as a result of splenic congestion due to mechanical obstruction caused by an inflammatory myofibroblastic tumor of the tail of pancreas. A review of the literature is also presented. Conclusion Inflammatory myofibroblastic tumor of the tail of pancreas should be included in the differential diagnosis of the etiological causes of massively enlarged spleen and spontaneous splenic rupture.
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Affiliation(s)
- Kamal S Hassan
- Clalit Health Services, Ruth and Bruce Rappaport Faculty of Medicine, Technion, Haifa, Israel.
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Schütte K, Kandulski A, Kuester D, Meyer F, Wieners G, Schulz HU, Malfertheiner P. Inflammatory Myofibroblastic Tumor of the Pancreatic Head: An Unusual Cause of Recurrent Acute Pancreatitis - Case Presentation of a Palliative Approach after Failed Resection and Review of the Literature. Case Rep Gastroenterol 2010; 4:443-451. [PMID: 21113286 PMCID: PMC2988857 DOI: 10.1159/000320953] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/31/2022] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) are a rare cause of echo-poor pancreatic head enlargement. Histologically, IMTs are characterized by spindle-shaped myofibroblasts or fibroblasts accompanied by a mixed immune cell infiltration. The most common localizations of IMTs have been reported in lung, mesentery and omentum, especially in children and young adults. IMTs show infiltrating growth, multilocular appearance and also metastasis have been reported. Curative resection is the only therapeutic option so far. In the palliative situation, evident data and clear guidelines for this rare tumor entity are missing. We report on a 44-year-old male with an unresectable IMT of the pancreatic head causing recurrent episodes of acute pancreatitis that resulted in a chronic obstructive course of the disease. The patient entered a palliative therapeutic regimen including radiation therapy and antiinflammatory medication. In a regular follow-up of 12 months, he presented with stable disease after initial progression. This case of local progressive IMT of the pancreatic head was managed with a palliative therapeutic regimen and is discussed based on the current literature.
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Affiliation(s)
- Kerstin Schütte
- Department of Gastroenterology, Hepatology and Infectious Diseases, Magdeburg, Germany
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Barth E, Savides TJ. Autoimmune pancreatitis. Expert Rev Clin Immunol 2010; 5:801-11. [PMID: 20477698 DOI: 10.1586/eci.09.54] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Autoimmune pancreatitis is becoming a more widely recognized form of pancreatitis that can mimic pancreatic or biliary malignancy. The combination of serological, histological and radiographic findings makes it unique among pancreatic diseases. The presence of autoantibodies, IgG4 and a lymphoplasmacytic infiltrate reflect its autoimmune etiology. The dramatic response to steroids is also a distinguishing feature and differentiates it from other pancreatic diseases.
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Affiliation(s)
- Erin Barth
- Department of Medicine, University of California, San Diego, CA, USA
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Bateman AC, Deheragoda MG. IgG4-related systemic sclerosing disease - an emerging and under-diagnosed condition. Histopathology 2009; 55:373-83. [DOI: 10.1111/j.1365-2559.2008.03217.x] [Citation(s) in RCA: 89] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
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Zamboni G, Capelli P, Scarpa A, Bogina G, Pesci A, Brunello E, Klöppel G. Nonneoplastic mimickers of pancreatic neoplasms. Arch Pathol Lab Med 2009; 133:439-53. [PMID: 19260749 DOI: 10.5858/133.3.439] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/08/2008] [Indexed: 11/06/2022]
Abstract
CONTEXT A variety of nonneoplastic conditions may form pancreatic masses that mimic carcinoma. Approximately 5% to 10% of pancreatectomies performed with the clinical diagnosis of pancreatic cancer prove on microscopic evaluation to be pseudotumors. OBJECTIVES To illustrate the clinical and pathologic characteristics of the 2 most frequent pseudotumoral inflammatory conditions, autoimmune pancreatitis and paraduodenal pancreatitis, and describe the criteria that may be useful in the differential diagnosis versus pancreatic carcinoma. DATA SOURCES Recent literature and the authors' experience with the clinical and pathologic characteristics of autoimmune pancreatitis and paraduodenal pancreatitis. CONCLUSIONS The knowledge of the clinical, radiologic, and pathologic findings in both autoimmune pancreatitis and paraduodenal pancreatitis is crucial in making the correct preoperative diagnosis. Autoimmune pancreatitis, which occurs in isolated or syndromic forms, is characterized by a distinctive fibroinflammatory process that can either be limited to the pancreas or extend to the biliary tree. Its correct preoperative identification on biopsy material with ancillary immunohistochemical detection of dense immunoglobulin G4-positive plasma cell infiltration is possible and crucial to prevent major surgery and to treat these patients with steroid therapy. Paraduodenal pancreatitis is a special form of chronic pancreatitis that affects young males with a history of alcohol abuse and predominantly involves the duodenal wall in the region of the minor papilla. Pathogenetically, the anatomical and/or functional obstruction of the papilla minor, resulting from an incomplete involution of the intraduodenal dorsal pancreas, associated with alcohol abuse represents the key factor. Endoscopic drainage of the papilla minor, with decompression of the intraduodenal and dorsal pancreas, might be considered in these patients.
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Affiliation(s)
- Giuseppe Zamboni
- Department of Pathology, University of Verona, Ospedale Sacro Cuore-Don Calabria, Via don Sempreboni 5, 37024 Negrar-Verona, Italy.
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Meagher C, Tang Q, Fife BT, Bour-Jordan H, Wu J, Pardoux C, Bi M, Melli K, Bluestone JA. Spontaneous development of a pancreatic exocrine disease in CD28-deficient NOD mice. THE JOURNAL OF IMMUNOLOGY 2008; 180:7793-803. [PMID: 18523243 DOI: 10.4049/jimmunol.180.12.7793] [Citation(s) in RCA: 43] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
Autoimmune pancreatitis (AIP) is a heterogeneous autoimmune disease in humans characterized by a progressive lymphocytic and plasmacytic infiltrate in the exocrine pancreas. In this study, we report that regulatory T cell-deficient NOD.CD28KO mice spontaneously develop AIP that closely resembles the human disease. NOD mouse AIP was associated with severe periductal and parenchymal inflammation of the exocrine pancreas by CD4(+) T cells, CD8(+) T cells, and B cells. Spleen CD4(+) T cells were found to be both necessary and sufficient for the development of AIP. Autoantibodies and autoreactive T cells from affected mice recognized a approximately 50-kDa protein identified as pancreatic amylase. Importantly, administration of tolerogenic amylase-coupled fixed spleen cells significantly ameliorated disease severity, suggesting that this protein functions as a key autoantigen. The establishment and characterization of this spontaneous pancreatic amylase-specific AIP in regulatory T cell-deficient NOD.CD28KO mice provides an excellent model for the study of disease pathogenesis and development of new therapies for human autoimmune pancreatitis.
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Affiliation(s)
- Craig Meagher
- Diabetes Center, University of California, San Francisco, CA 94143, USA
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Autoimmune pancreatitis: expression and cellular source of profibrotic cytokines and their receptors. Am J Surg Pathol 2008; 32:986-95. [PMID: 18460977 DOI: 10.1097/pas.0b013e31815d2583] [Citation(s) in RCA: 49] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Chronic pancreatitis is a fibrogenic disease. In autoimmune pancreatitis (AIP), a lymphoplasmacytic infiltration is followed by fibrosis. In vitro it has been shown that pancreatic stellate cells are transformed into proliferating myofibroblasts mainly by transforming growth factor beta (TGF-beta) and platelet-derived growth factor (PDGF). We studied the expression of these profibrotic cytokines, their receptors, and their cellular sources in AIP. Pancreatic tissues from 21 patients with AIP of different grades of severity were selected from a series of 52 AIP cases. Myofibroblasts (ie, activated pancreatic stellate cells), macrophages, lymphocytes, plasma cells, and the cytokines latency-associated peptide, a TGF-beta1 propeptide, TGF-beta receptor II (TGF-beta-RII), PDGF-B, and the alpha and beta isoforms of the PDGF receptor (PDGF-R alpha and PDGF-R beta) were identified immunohistochemically. Their expression and cellular distribution were related to the severity of AIP. In grade 1 and 2 AIP, macrophages and myofibroblasts expressing profibrotic cytokines and their receptors were found in periductal areas showing lymphoplasmacytic inflammation. In grade 3 AIP, there were numerous macrophages, myofibroblasts, and epithelial cells which were positive for latency-associated peptide, PDGF-B, TGF-beta-RII, PDGF-R alpha, and PDGF-R beta not only in periductal, but also in interlobular and intralobular areas. In grade 4 AIP, which is characterized by advanced fibrosis, cellularity and expression of cytokines and their receptors were greatly reduced. Our data indicate that in AIP the occurrence of myofibroblasts is intimately related to the presence of macrophages and lymphoplasmacytic cells. These cells and adjacent epithelial cells express profibrotic cytokines and their receptors, which are probably responsible for the initiation and maintenance of the fibrogenic process.
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Ma Y, Zieske AW, Fenves AZ. Bilateral infiltrating renal inflammatory pseudotumor responsive to corticosteroid therapy. Am J Kidney Dis 2008; 51:116-20. [PMID: 18155540 DOI: 10.1053/j.ajkd.2007.08.028] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2007] [Accepted: 08/07/2007] [Indexed: 01/17/2023]
Abstract
Inflammatory pseudotumor (IPT) is a quasi-neoplastic lesion that most commonly involves the lung, but has been shown to occur in nearly every tissue type. Renal involvement is very uncommon. We report the second case of IPT ever published presenting as bilateral infiltrating renal masses. Although most renal IPTs were treated with nephrectomy, our patient was managed successfully with conservative steroid treatment, thereby avoiding the alternative of dialysis or kidney transplantation.
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Affiliation(s)
- Yanjun Ma
- Department of Internal Medicine, Baylor University Medical Center, Dallas, TX 75246, USA.
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Klöppel G, Sipos B, Zamboni G, Kojima M, Morohoshi T. Autoimmune pancreatitis: histo- and immunopathological features. J Gastroenterol 2007; 42 Suppl 18:28-31. [PMID: 17520220 DOI: 10.1007/s00535-007-2048-6] [Citation(s) in RCA: 61] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
In recent years autoimmune pancreatitis (AIP) has been established as a special type of chronic pancreatitis. It is characterized by its histopathological and immunological features. The morphological hallmarks are periductal infiltration by lymphocytes and plasma cells, granulocytic epithelial lesions with focal destruction of the duct epithelium, venulitis, and diffuse sclerosis in advanced stages. AIP has therefore also been called lymphoplasmacytic sclerosing pancreatitis, duct-destructive chronic pancreatitis, or sclerosing pancreatitis. AIP most commonly involves the head of the pancreas and the distal bile duct. Occasionally it is mass-forming, and has been described as an inflammatory myofibroblastic tumor. The presence of more than 20 IgG4-positive plasma cells per high-power field is of high specificity for the tissue diagnosis of AIP.
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Affiliation(s)
- Günter Klöppel
- Department of Pathology, University of Kiel, Michaelisstr, 11, 24105, Kiel, Germany
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Kojima M, Sipos B, Klapper W, Frahm O, Knuth HC, Yanagisawa A, Zamboni G, Morohoshi T, Klöppel G. Autoimmune Pancreatitis: Frequency, IgG4 Expression, and Clonality of T and B Cells. Am J Surg Pathol 2007; 31:521-8. [PMID: 17414098 DOI: 10.1097/01.pas.0000213390.55536.47] [Citation(s) in RCA: 117] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Autoimmune pancreatitis (AIP) is a newly recognized disease. The presence of IgG4 positive plasma cells is thought to be of diagnostic help. In a surgical series of chronic pancreatitis cases, we determined the relative frequency of AIP before and after 1990, analyzed the diagnostic significance of IgG4 expression and examined the presence of oligoclonal T or B-cell populations. The histopathology of 202 surgical specimens of chronic pancreatitis removed between 1975 and 2004 was reviewed and 2 groups were distinguished, 1 of AIP cases and the other of nonautoimmune chronic pancreatitis (non-AIP CP). The intensity of infiltration of pancreatic tissue by IgG4 positive plasma cells and other immune cells was studied immunohistochemically. Finally, T and B-cell clonality was tested by polymerase chain reaction-based analysis. Except for 1 case in 1978, all cases of AIP were observed after 1990. IgG4 positive plasma cells were detected in 72.5% of AIP cases and in 63.1% of non-AIP CP cases. More than 20 cells per high power field were only seen in AIP (sensitivity 43%, specificity 100%). This finding was associated with higher age and grade. Polyclonal T and B-cell populations were found in both AIP and non-AIP CP except for 1 AIP case showing an oligoclonal IgGH-FR3 gene rearrangement. AIP seems to have increased considerably in frequency in the last 2 decades. High density infiltrates of IgG4 positive plasma cells are diagnostic for AIP, but are seen in less than half of the cases. T or B-cell oligoclonality could not be established as a feature of AIP.
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Abstract
Chronic pancreatitis is a fibroinflammatory disease of the pancreas. Etiologically, most cases are related to alcohol abuse and smoking. Recently, gene mutations have been identified as the cause of hereditary pancreatitis. Other chronic pancreatitis types that were defined in recent years are autoimmune pancreatitis (lymphoplasmacytic sclerosing pancreatitis) and paraduodenal pancreatitis ('groove pancreatitis', 'cystic dystrophy of heterotopic pancreas'). This review describes and discusses the main histological findings, the pathogenesis and the clinical features of the various types of chronic pancreatitis. In addition, pseudotumors and other tumor-like lesions are briefly mentioned.
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Affiliation(s)
- Günter Klöppel
- Department of Pathology, University of Kiel, Kiel, Schleswig-Holstein, Germany.
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Inatomi O, Andoh A, Yagi Y, Ogawa A, Hata K, Shiomi H, Tani T, Takayanagi A, Shimizu N, Fujiyama Y. Matrix metalloproteinase-3 secretion from human pancreatic periacinar myofibroblasts in response to inflammatory mediators. Pancreas 2007; 34:126-32. [PMID: 17198194 DOI: 10.1097/01.mpa.0000246662.23128.57] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/29/2023]
Abstract
OBJECTIVES Matrix metalloproteinases (MMPs) play roles in the pathophysiology of pancreatic disorders. However, the regulation of MMP-3 secretion in the pancreas remains unclear. In this study, we assessed the expression of MMP-3 in human pancreatic periacinar myofibroblasts. METHODS MMP-3 secretion and MMP-3 mRNA expression were determined by enzyme-linked immunosorbent assay and real-time polymerase chain reaction, respectively. RESULTS In human pancreatic myofibroblasts, MMP-3 secretion and mRNA expression were induced by interleukin (IL)-17, IL-1beta, and tumor necrosis factor (TNF) -alpha, respectively. The effects of IL-17 were detected as similar in extent to those induced by IL-1beta or TNF-alpha. Costimulation by IL-17 plus IL-1beta and/or IL-17 plus TNF-alpha induced a synergistic increase in MMP-3 secretion, although the costimulatory effects of these combinations were not detected in tissue inhibitor of matrix metalloproteinase-1 secretion. Adenovirus-mediated transfer of a stable form of IkappaBalpha markedly inhibited the effects of IL-17, IL-1beta, and TNF-alpha. Mitogen-activated protein kinase inhibitors (U0126, PD098059, and SB203580) also blocked MMP-3 secretion. These findings indicate a role for nuclear factor-kappaB and mitogen-activated protein kinases in cytokine-induced MMP-3 secretion. CONCLUSIONS Pancreatic periacinar myofibroblasts actively secrete MMP-3 in response to IL-17, IL-1beta, and TNF-alpha. Pancreatic myofibroblasts may play an important role in extracellular matrix turnover via MMP-3 secretion in the pancreas.
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Affiliation(s)
- Osamu Inatomi
- Department of Internal Medicine, Shiga University of Medical Science, Otsu, Japan
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Neild GH, Rodriguez-Justo M, Wall C, Connolly JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Med 2006; 4:23. [PMID: 17026742 PMCID: PMC1618394 DOI: 10.1186/1741-7015-4-23] [Citation(s) in RCA: 190] [Impact Index Per Article: 10.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2006] [Accepted: 10/06/2006] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis. METHODS We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis. RESULTS Histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment. CONCLUSION We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good.
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Affiliation(s)
- Guy H Neild
- UCL Centre for Nephrology, Royal Free Hospital, London NW3 2QG, UK
- Institute of Urology and Nephrology, Middlesex Hospital, London W1T 3AA, UK
| | - Manuel Rodriguez-Justo
- Department of Histopathology, Royal Free and University College Medical School, University College Hospital, Rockefeller Building, London WC1E 6JJ, UK
| | - Catherine Wall
- UCL Centre for Nephrology, Royal Free Hospital, London NW3 2QG, UK
| | - John O Connolly
- UCL Centre for Nephrology, Royal Free Hospital, London NW3 2QG, UK
- Institute of Urology and Nephrology, Middlesex Hospital, London W1T 3AA, UK
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Abu El-Asrar AM, Al-Kharashi SA, Missotten L, Geboes K. Expression of growth factors in the conjunctiva from patients with active trachoma. Eye (Lond) 2006; 20:362-9. [PMID: 15818386 DOI: 10.1038/sj.eye.6701884] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022] Open
Abstract
PURPOSE The blinding complications of trachoma are associated with progressive conjunctival fibrosis due to excessive accumulation of extracellular matrix (ECM) components. We studied the processes involved in the regulation of fibrosis in trachoma by investigating the expression of the fibrogenic and angiogenic connective tissue growth factor (CTGF) and basic fibroblast growth factor (bFGF), the angiogenic vascular endothelial growth factor (VEGF), the angiogenesis-associated endothelial cell marker CD105 (endoglin), and the ECM protein tenascin in the conjunctiva. METHODS Conjunctival biopsy specimens from six patients with active trachoma, and six control subjects were studied by immunohistochemical techniques using monoclonal and polyclonal antibodies directed against CTGF, bFGF, VEGF, CD105, and tenascin. RESULTS In the normal conjunctiva, weak immunoreactivity for VEGF was observed in epithelial cells. There was no immunoreactivity for the other antibodies. In all trachoma specimens, immunoreactivity for CTGF and bFGF was localized in monocytes/macrophages, positive for the CD68 marker. Strong immunoreactivity for VEGF was observed in epithelial cells and on vascular endothelial cells. CD105 immunoreactivity was observed on vascular endothelial cells. Immunoreactivity for tenascin was noted in the upper substantia propria. CONCLUSIONS These findings suggest that macrophages play an active role in conjunctival scarring, upregulated local production of CTGF, bFGF, and VEGF contributes to both fibrous tissue growth and angiogenesis, vascular endothelial cells are activated and are undergoing active angiogenesis, and deposition of tenascin reflect remodelling of the conjunctiva in trachomatous conjunctivitis.
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Affiliation(s)
- A M Abu El-Asrar
- Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
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Lopez-Tomassetti Fernandez EM, Luis HD, Malagon AM, Gonzalez IA, Pallares AC. Recurrence of inflammatory pseudotumor in the distal bile duct: Lessons learned from a single case and reported cases. World J Gastroenterol 2006; 12:3938-43. [PMID: 16804988 PMCID: PMC4087951 DOI: 10.3748/wjg.v12.i24.3938] [Citation(s) in RCA: 17] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Inflammatory myofibroblastic tumors (IMTs) or inflammatory pseudotumors (IPs) have been extensively discussed in the literature. They are usually found in the lung and upper respiratory tract. However, reporting of cases involving the biliopancreatic region has increased over recent years. Immunohistochemical study of these lesions limited to the pancreatic head or distal bile duct seems to be compatible with those observed in a new entity called autoimmune pancreatitis, but usually intense fibrotic reaction (zonation) predominates producing a mass. When this condition is limited to the pancreatic head, the common bile duct might be involved by the inflammatory process and jaundice may occur often resembling adenocarcinoma of the pancreas. We have previously reported a case of IMT arising from the bile duct associated with autoimmune pancreatitis which is an extremely rare entity. Four years after Kaush-Whipple resection, radiological examination on routine follow-up revealed a tumor mass, suggesting local recurrence. Ultrasound-guided FNA confirmed our suspicious diagnosis. This present case, as others, suggests that persistent follow-up is necessary in order to prevent irreversible liver damage at this specific location.
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Affiliation(s)
- E M Lopez-Tomassetti Fernandez
- Department of Gastrointestinal Surgery, University Hospital of Canary Islands, Ofra s/n. La Cuesta, La Laguna, Santa Cruz de Tenerife, Spain.
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Florell SR, Cessna M, Lundell RB, Boucher KM, Bowen GM, Harris RM, Petersen MJ, Zone JJ, Tripp S, Perkins SL. Usefulness (or Lack Thereof) of Immunophenotyping in Atypical Cutaneous T-Cell Infiltrates. Am J Clin Pathol 2006. [DOI: 10.1309/3jk2h6y988nuay37] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
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Mizukami H, Yajima N, Wada R, Matsumoto K, Kojima M, Klöppel G, Yagihashi S. Pancreatic malignant fibrous histiocytoma, inflammatory myofibroblastic tumor, and inflammatory pseudotumor related to autoimmune pancreatitis: characterization and differential diagnosis. Virchows Arch 2006; 448:552-60. [PMID: 16538442 DOI: 10.1007/s00428-006-0157-x] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/24/2005] [Accepted: 01/10/2006] [Indexed: 12/24/2022]
Abstract
Malignant fibrous histiocytoma (MFH) and inflammatory myofibroblastic tumor (IMT) are uncommon primary non-epithelial cell tumors of the pancreas. In addition, there are inflammatory pseudotumors (IPT) that may arise in the course of autoimmune pancreatitis (AIP). In the English language literature, only 24 cases of IMT and nine cases of MFH in the pancreas have been reported to date. We investigated three individual spindle cell tumors of the pancreas that were identified as MFH, IMT, and IPT, respectively, using immunohistochemical and molecular analysis. Both the MFH and the IMT, but not the IPT, showed nuclear p53 expression and mutations of the p53 gene. The MFH and the IMT also had higher mitotic and Ki-67 (MIB-1) indexes than the IPT. The IPT was found to be a tumor-like case of AIP. Many IgG4-positive plasma cells, which are considered to be a feature of AIP, were found in all three tumors. It is concluded that in this series of spindle cell tumors of the pancreas, apart from immunohistochemical features, the demonstration of p53 mutations may be helpful in distinguishing true neoplastic tumors from pseudotumors such as IPTs arising in the context of AIP.
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MESH Headings
- Adult
- Autoimmune Diseases/complications
- Base Sequence
- Diagnosis, Differential
- Female
- Gene Expression
- Gene Expression Profiling
- Granuloma, Plasma Cell/etiology
- Granuloma, Plasma Cell/genetics
- Granuloma, Plasma Cell/pathology
- Histiocytoma, Malignant Fibrous/genetics
- Histiocytoma, Malignant Fibrous/metabolism
- Histiocytoma, Malignant Fibrous/pathology
- Humans
- Immunohistochemistry
- Ki-67 Antigen/metabolism
- Male
- Middle Aged
- Mutation
- Neoplasms, Muscle Tissue/genetics
- Neoplasms, Muscle Tissue/metabolism
- Neoplasms, Muscle Tissue/pathology
- Pancreatectomy
- Pancreatic Neoplasms/genetics
- Pancreatic Neoplasms/metabolism
- Pancreatic Neoplasms/pathology
- Pancreatitis/complications
- Pancreatitis/pathology
- Polymerase Chain Reaction
- Tumor Suppressor Protein p53/genetics
- Tumor Suppressor Protein p53/metabolism
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Affiliation(s)
- Hiroki Mizukami
- Department of Pathology, Hirosaki University School of Medicine, and Division of Surgical Pathology, National Hirosaki Hospital, 036-8562, Japan
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Martín Malagón A, López-Tomassetti Fernández E, Arteaga González I, Carrillo Pallarés A, Díaz Luis H. Inflammatory myofibroblastic tumor of the distal bile duct associated with lymphoplasmacytic sclerosing pancreatitis. Case report and review of the literature. Pancreatology 2005; 6:145-54. [PMID: 16354963 DOI: 10.1159/000090256] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor has been described in various organs such as the liver, intestinal tract, spleen, kidney, bladder, lung, peritoneum and heart. However, its appearance in the periampullary region is uncommon and has rarely been reported in the literature. It is characterized histologically by myofibroblastic cell proliferation together with a mixed inflammatory infiltrate that clinically and radiologically mimics a malignant tumor. We report a case of IMT located in the distal common bile duct of a 51-year-old woman. She underwent Whipple resection with the initial diagnosis of cholangiocarcinoma; the pathologic diagnosis of the tumor was IMT of the distal bile duct associated with lymphoplasmacytic sclerosing pancreatitis. Referring to previously reported cases, suspected diagnosis of a malignant tumor made surgical excision the primary choice for symptom relief and in order to obtain a definitive diagnosis. IMT relationship with lymphoplasmacytic sclerosing pancreatitis is discussed.
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Affiliation(s)
- Antonio Martín Malagón
- Department of General Surgery, Hospital Universitario de Canarias, Tenerife, Canary Islands, Spain.
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29
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Adsay NV, Basturk O, Klimstra DS, Klöppel G. Pancreatic pseudotumors: non-neoplastic solid lesions of the pancreas that clinically mimic pancreas cancer. Semin Diagn Pathol 2005; 21:260-7. [PMID: 16273945 DOI: 10.1053/j.semdp.2005.07.003] [Citation(s) in RCA: 52] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
In the pancreas, a variety of non-neoplastic conditions may form solid masses that may mimic cancer. Up to 5% of pancreatectomies performed with the preoperative clinical diagnosis of carcinoma will prove to be non-neoplastic by pathologic examination, although this figure is decreasing with improved diagnostic modalities. Chronic inflammatory lesions are the leading cause of this phenomenon ("pseudotumoral pancreatitis"), and among these, autoimmune and paraduodenal pancreatitides (discussed separately in this issue) are most important. In this article, we will focus on the noninflammatory lesions that may form tumor-like lesions of the pancreas. Adenomyomatous hyperplasia of ampulla of Vater is a subtle lesion that is difficult to define; larger examples (>5 mm) have been found to be the cause of obstructive jaundice. Accessory (heterotopic) spleen may form a well-defined nodule within the tail of the pancreas and is typically mistaken for endocrine neoplasm. Lipomatous hypertrophy is the replacement of pancreatic tissue with mature adipose tissue that occasionally leads to moderate to marked enlargement of the pancreas. Hamartomas are very rare if the entity is defined strictly. They are characterized by irregularly arranged mature pancreatic elements admixed with stromal tissue. A cellular, spindle-cell variant with c-kit (CD117) expression is recognized. Pseudolymphoma forms well-defined nodules composed of hyperplastic lymphoid tissue. Rarely, foreign-body deposits, granulomatous inflammations (such as sarcoidosis or tuberculosis), and congenital lesions may form tumoral lesions. In conclusion, it is important to recognize the types of conditions that form pseudotumors in the pancreas so that they can be distinguished from ductal adenocarcinomas, especially clinically, but also pathologically. Nonspecific terms such as "inflammatory pseudotumor" ought to be avoided, and every attempt should be made to classify a "pseudotumor" into a more specific diagnostic category discussed above.
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Affiliation(s)
- N Volkan Adsay
- Karmanos Cancer Institute/Wayne State University, Detroit, Michigan, USA.
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Adsay NV, Basturk O, Thirabanjasak D. Diagnostic features and differential diagnosis of autoimmune pancreatitis. Semin Diagn Pathol 2005; 22:309-17. [PMID: 16939059 DOI: 10.1053/j.semdp.2006.04.008] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
A clinically and pathologically distinct form of chronic pancreatitis is now widely recognized and has been designated variably as lymphoplasmacytic sclerosing pancreatitis, duct-destructive (duct-centric) pancreatitis or autoimmune pancreatitis. This entity is currently defined by a constellation of clinical and pathologic findings, including the lack of both conventional risk factors for pancreatitis, such as alcohol use and gallstones, and their hallmark pattern of injury, including calcifications and pseudocysts. Histologically, it is characterized by lymphoplasmacytic inflammation with abundant IgG4-positive plasma cells that exhibit an affinity for ducts as well as venules ("peri-venulitis," with or without frank vasculitis). Inflammation is often associated with sclerosis and expansion of periductal tissue. In some cases, fibroblastic activity is prominent and resembles "inflammatory pseudotumor" or is even misdiagnosed as "inflammatory myofibroblastic tumor." In what appears to be a distinct subset of this entity, intraepithelial granulocytic infiltrates may be seen. Well-developed examples are readily recognized; however, lesser ones may be difficult to distinguish from other forms of pancreatitis based on morphology alone. This type of pancreatitis is considered an autoimmune process. In about 15% to 20% of patients, the clinical stigmata of autoimmune conditions are present at the time of diagnosis, and in many others, discovered subsequently. The usual "lymphoplasmacytic sclerotic" type tends to be associated with Sjogren, whereas the "granulocytic" subset, with inflammatory bowel disease. Most patients present with a pancreatic head mass, often with an accompanying stricture of the distal common bile duct, which thus radiologically resembles "pancreas cancer." In fact, this entity accounts for more than a third of the cases of pseudotumoral pancreatitis (mass-forming inflammatory lesions that resemble carcinoma). Elevated serum IgG4 levels are characteristic and may be very helpful in the differential diagnosis from tumors and tumor-like lesions of the pancreas which seldom result in levels above 135 mg/dL. The mean age of the patients with this condition is in the mid-50s; the subset with granulocytic intraepithelial lesions seem to be younger (mid 40s). Despite the autoimmune association, males are afflicted as commonly as (if not more than) females. Following resection, emergence of new fibro-inflammatory lesions in the remaining pancreaticobiliary tree has been noted in some cases; however, the process typically responds to steroids. It is important to recognize the distinctive clinicopathologic features of this entity, so that it can be diagnosed accurately and managed appropriately.
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Affiliation(s)
- N Volkan Adsay
- Department of Pathology, The Karmanos Cancer Institute and Wayne State University School of Medicine, Detroit, Michigan, USA.
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Pauser U, Kosmahl M, Kruslin B, Klimstra DS, Klöppel G. Pancreatic solid and cystic hamartoma in adults: characterization of a new tumorous lesion. Am J Surg Pathol 2005; 29:797-800. [PMID: 15897746 DOI: 10.1097/01.pas.0000157748.18591.d7] [Citation(s) in RCA: 59] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
Nonneoplastic tumor-like lesions ("pseudotumors") of the pancreas include cystic and noncystic varieties. We report on a solid and cystic tumor-like lesion of the pancreas that occurred in 2 adult patients. The lesions, located in the head and neck of the gland, respectively, were well demarcated and composed of cystic ductal structures embedded in focally inflamed stromal tissue. In addition, one of the lesions showed irregularly arranged but well-differentiated acini and small intralobular and interlobular ducts embedded in hypocellular, fibrotic tissue. Discrete islets were lacking, but immunohistochemical staining for chromogranin A revealed individual scattered endocrine cells evenly distributed between acinar and ductal cells. The surrounding pancreatic parenchyma did not show significant chronic pancreatitis. After tumor removal, the follow-up of the patients was uneventful. Because of the irregular arrangement of otherwise mature tissue components of the pancreas, the lesions were considered solid and cystic hamartomas. Their pathogenesis is so far unknown.
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Affiliation(s)
- Ursula Pauser
- Department of Pathology, University of Kiel, Michaelisstrasse 11, 24105 Kiel, Germany.
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Abstract
Lymphoplasmacytic sclerosing pancreatitis (LPSP), also known as autoimmune pancreatitis or nonalcoholic, duct destructive chronic pancreatitis, has been increasingly recognized in the past decade as a histologically distinctive type of pancreatitis that affects middle-aged patients who lack the typical risk factors for chronic pancreatitis (alcohol abuse in particular). LPSP is sometimes associated with other autoimmune diseases or fibroinflammatory lesions, although in some patients, pancreatic and biliary involvement represent the only known disease process. Many patients present with pancreatic masses clinically and radiographically simulating pancreatic carcinoma, and associated bile duct strictures enhance the resemblance. Elevated serum IgG4 levels have been described in patients with LPSP and have been used to distinguish LPSP from pancreatic carcinoma preoperatively. Although there is some heterogeneity of pathologic findings, resected cases of LPSP typically demonstrate dense periductal lymphoplasmacytic inflammation, periductal and parenchymal fibrosis, and obliterative venulitis; neutrophilic infiltration of the ductal epithelium ("granulocytic epithelial lesions") may also occur. Large tumor-like masses of fibroinflammatory tissue ("reactive fibroinflammatory pseudotumors") may develop and extend beyond the pancreas. Following surgical resection, a few patients suffer recurrence of fibroinflammatory lesions in the pancreatobiliary tree, or they may develop other manifestations of autoimmune disease elsewhere in the body. However, the overall prognosis is excellent. Response to steroid therapy has been noted. Current studies are focusing on identifying additional preoperative diagnostic tests and on characterizing possible variants of LPSP. This review presents the defining clinical and pathologic features of LPSP and discusses the ongoing efforts to understand the pathogenesis of this disease.
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Affiliation(s)
- David S Klimstra
- Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
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Zamboni G, Lüttges J, Capelli P, Frulloni L, Cavallini G, Pederzoli P, Leins A, Longnecker D, Klöppel G. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch 2004; 445:552-63. [PMID: 15517359 DOI: 10.1007/s00428-004-1140-z] [Citation(s) in RCA: 438] [Impact Index Per Article: 20.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2004] [Accepted: 09/21/2004] [Indexed: 12/12/2022]
Abstract
BACKGROUND AND AIMS Autoimmune pancreatitis seems to be a disease with a heterogeneous appearance. Our intention was to establish key diagnostic criteria, define grades of severity and activity, identify features of potential subtypes and evaluate the diagnostic relevance of biopsy specimens. METHODS Histopathological criteria and clinical features were recorded in pancreatic resection specimens from 53 patients who were found to have chronic pancreatitis lacking pseudocysts, calculi, irregular duct dilatations, pancreas divisum and/or duodenal wall inflammation. The severity of the chronic inflammation was graded, and the activity of the acute inflammatory component and the granulocytic epithelial lesion (GEL) were determined. Additionally, pancreatic biopsy specimens from 9 patients with suspected AIP were assessed. RESULTS Periductal lymphoplasmacytic infiltration was identified in all cases, followed in order of frequency by periductal fibrosis and venulitis. These changes were absent in 147 pancreatic specimens that showed chronic pancreatitis associated with pseudocysts, calculi, pancreas divisum and/or duodenal wall inflammation. In 90% of the cases, these chronic changes were graded as 3 or 4. In 81%, the inflammatory process resided in the head of the pancreas and involved the common bile duct. GELs were present in 42% of the patients, who had a mean age of 40.5 years, an almost equal male-female ratio and a high coincidence of ulcerative colitis or Crohn's disease. Patients without GELs were older (mean age 64 years), showed a male preponderance, commonly had Sjogren's syndrome and often developed recurrent bile-duct stenosis. Diagnostically relevant lesions were present in two of five wedge biopsy specimens and three of four fine-needle specimens. CONCLUSIONS Periductal lymphoplasmacytic infiltration and fibrosis, preferential occurrence in the pancreatic head and venulitis characterize autoimmune pancreatitis. GELs predominantly occur in a subset of patients who are younger, more commonly have ulcerative colitis and Crohn's disease and seem to have fewer recurrences than patients without GELs. Pancreatic biopsy material proved to be a very helpful adjunct for establishing the diagnosis.
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Hirano K, Komatsu Y, Yamamoto N, Nakai Y, Sasahira N, Toda N, Isayama H, Tada M, Kawabe T, Omata M. Pancreatic mass lesions associated with raised concentration of IgG4. Am J Gastroenterol 2004; 99:2038-40. [PMID: 15447769 DOI: 10.1111/j.1572-0241.2004.40215.x] [Citation(s) in RCA: 55] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Autoimmune pancreatitis (AIP) is a recognized benign disease characterized by irregular narrowing of the pancreatic duct, swelling of parenchyma, lymphoplasmacytic infiltration and fibrosis, and a favorable response to corticosteroid treatment. In this condition, the whole pancreas is diffusely affected. Recently, however, a few cases with locally affected lesions were reported, with some of them showing features similar to cancer. We reviewed 138 patients with pancreatic mass lesion, of which 17 were not initially diagnosed despite examinations. Serum IgG4 levels were elevated in seven of them. Their biopsy specimens had a similar appearance to those of AIP. We considered that they should be diagnosed as AIP or conditions related to AIP. Among the 10 patients without elevated IgG4, 4 patients were diagnosed as pancreatic cancer after follow-up, 1 presented with an islet cell tumor, 1 presented AIP with sclerosing cholangitis, and the other 4 had chronic pancreatitis.
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Affiliation(s)
- Kenji Hirano
- Department of Gastroenterology, University of Tokyo, Mitsui Memorial Hospital, Tokyo, Japan
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