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Table of Contents
CASE REPORT
Year : 2016  |  Volume : 3  |  Issue : 1  |  Page : 216-218

Mediastinal mass in a patient with IgG4-related disease


1 Division of Surgery, Mito Medical Center, University of Tsukuba, Tsukuba, Japan
2 Division of Pathology, Mito Medical Center, University of Tsukuba, Tsukuba, Japan
3 Division of Respiratory Medicine, Mito Medical Center, University of Tsukuba, Tsukuba, Japan

Date of Web Publication5-Jul-2017

Correspondence Address:
Hiroaki Satoh
Division of Respiratory Medicine, Mito Medical Center, University of Tsukuba, Miya-machi 3-2-7, Mito, Ibaraki, 310-0015
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.5530/ami.2016.1.44

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  Abstract 


Introduction: IgG4-related disease is now considered to result from an imbalance in the regulatory immune system, and it is diagnosed on the basis of clinical and histopathologic evidence such as the presence of lymphoplasmacytic infiltrates, especially increased IgG4-positive plasma cells and fibrotic sclerosing tissue.
Case report: We report herein a 45-year-old male patient with mediastinal mass with IgG4-related disease. IgG4-related disease can involve any organ but the most frequently involved sites include pancreato-hepatobiliary tract, salivary glands, and kidneys, generally manifesting as a mass in one or more sites mimicking a neoplasia. In the thoracic region, IgG4-related disease manifests as interstitial lung disease and fibrosing mediastinitis. Mediastinal lesion in our patient was quite resemble to mediastinal tumor derived from anterior mediastinum. This is the first case with mediastinal mass in a patient with this disease.
Conclusion: Although very rare, we should rule out this disease as differential diagnosis in patients with anterior homogeneous density mediastinal mass simulating mediastinal tumor.

Keywords: Mediastinal mass, IgG4-related disease, Mediastinal tumor


How to cite this article:
Iguchi K, Ishibashi O, Takayashiki N, Satoh H. Mediastinal mass in a patient with IgG4-related disease. Acta Med Int 2016;3:216-8

How to cite this URL:
Iguchi K, Ishibashi O, Takayashiki N, Satoh H. Mediastinal mass in a patient with IgG4-related disease. Acta Med Int [serial online] 2016 [cited 2019 Jul 21];3:216-8. Available from: http://www.actamedicainternational.com/text.asp?2016/3/1/216/209713






  Introduction Top


IgG4-related disease can involve multiple organs, particularly exocrine organs such as pancreas, salivary glands, and biliary tract.[1],[2] When it affects the thorax, it commonly manifests interstitial lung disease and fibrosing mediastinitis.[3],[4],[5],[6],[7],[8],[9],[10],[11] We present herein a case of IgG4-related disease mimicking as mediastinal tumor in the anterior mediastinum.


  Case Report Top


A 45-year-old-man was admitted to our hospital due to incidentally detected mediastinal mass, which was not found in chest radiograph a year before. The patient had 5-year history of bronchial asthma. His serum immunoglobulin level, including igG4, was slightly elevated (114 mg/dL); normal range, 4.8-105 mg/d). The results of other biochemical tests were normal. Chest CT scan revealed a well-defined homogenous anterior mediastinal mass [Figure 1]a. As the mass was located at anterior mediastinum, we suspected thymoma, germ cell tumor, or lymphoma. Although the patient had no symptom, video-assisted thoracic surgery (VATS) was planned extended thymectomy with combined resection of the surrounding involved structures. After carrying out thoracotomy, we found a hen's egg-sized mass with smooth surface in anterior mediastinum. Adhesion of the mass to the surrounding tissue was not observed, but left internal thoracic artery and vessel were involved. The tumor was completely excised [Figure 1]b. Pathological specimen obtained from VATS showed storiform pattern of fibrosis and immunohistochemical staining for IgG4 positive cells [Figure 2]a and [Figure 2]b. Pathological diagnosis was IgG4-related disease. Postoperative course was not remarkable. Thereafter, oral prednisolone was prescribed for 18 months (tapered off from an initial dose of 40mg/day). The patient was followed up for 2 years and had no mass lesion including mediastinum.
Figure 1: Chest CT scan revealed a well-defined homogenous anterior mediastinal mass (a), Macroscopic view of completely resected a hen's egg-sized mass with smooth surface in anterior mediastinum (b)

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Figure 2: Pathological specimen obtained from video-assisted thoracic surgery showed storiform pattern of fibrosis (a) and immunohistochemical staining for IgG4 positive cells (b)

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  Discussion Top


Although the precise mechanisms are still unclear, IgG4-related disease is now considered to result from an imbalance in the regulatory immune system.[12],[13] IgG4-related disease develops predominantly in males.[2],[9] In majority of patients, IgG4-related disease is diagnosed on the basisof clinical and histopathologic evidence such as the presence of lymphoplasmacytic infiltrates, especially increased IgG4-positive plasma cells and fibrotic sclerosing tissue.[2],[9] As for treatment, IgG4-related disease has been shown to respond favorably to corticosteroid.[14] Azathioprine or rituximab is now useful in cases of relapse or failure to wean off corticosteroid.[2],[9]

IgG4-related disease can involve virtually any organ but the most frequently involved sites include the pancreas, bile ducts and gallbladder, liver, salivary glands, and kidneys generally manifesting as a mass in one or more sites mimicking a neoplasia.[2],[9] In the thoracic region, IgG4-related disease manifests as interstitial lung disease and fibrosing mediastinitis.[9],[10],[11] There have been three large prospective cohort of patients with IgG4-related disease. In a Japanese study of 114 patients with IgG4-related disease, 26 (22.8%) of them had pulmonary involvement and only 1 (0.9%) of them had mediastinal fibrosis.[10] In a French study of 25 patients with IgG4-related disease, 3 (12%) of them had pulmonary involvement and none of them had mediastinal fibrosis.[11] In a recent Chinese study of 118 patients with IgG4-related disease, 32 (2%) of them had pulmonary involvement and only 4 (3.4%) of them had mediastinal fibrosis.[9] To our best knowledge, however, mediastinal mass associated with this disease at mediastinum has not been previously reported. Therefore, this is the first case with mediastinal mass in patient with this disease.

Mediastinal lesion in our patient was quite resemble to mediastinal tumor derived from anterior mediastinum. Differential diagnosis of anterior mediastinal mass includes thymoma, germ cell tumor, and lymphoma.[15],[16] Neurogenic tumors are atypical with regard to location. Thymoma appears as homogeneous mass and germ cell tumor usually appears as heterogenous mass with calcification in enhanced CT scan. Taking the location and density of the mass into consideration, we preoperatively evaluated it was thymoma derived from anterior mediastinum.

We present herein a case of IgG4-related disease mimicking as mediastinal tumor in the anterior mediastinum. Although very rare, we should rule out this disease as differential diagnosis in patients with anterior homogenous density mediastinal mass, mimicking mediastinal tumor.



 
  References Top

1.
Brito-Zeron P, Ramos-Casals M, Bosch X, Stone JH. The clinical spectrum of IgG4-related disease. Autoimmun Rev 2014; 13: 1203–10.  Back to cited text no. 1
    
2.
Islam AD, Selmi C, Datta-Mitra A, et al. The changing faces of IgG4-related disease: Clinical manifestations and pathogenesis. Autoimmun Rev 2015 Jun 23. pii: S1568-9972(15)00134–2.  Back to cited text no. 2
    
3.
Shigemitsu H, Koss MN. IgG4-related interstitial lung disease: a new and evolving concept. Curr Opin Pulm Med 2009; 15: 513–6.  Back to cited text no. 3
    
4.
Miyashita T, Yoshioka K, Nakamura T, et al. A case oflymphomatoid granulomatosis-like lung lesions with abundant infiltrating IgG4- positive plasma cells whose serum IgG4 levels became high following the start ofcorticosteroid therapy. Intern Med 2010; 49: 2007–11.  Back to cited text no. 4
    
5.
Dias OM, Kawassaki Ade M, Haga H, Cukier A, Carvalho CR. Immunoglobulin G4-related systemic sclerosing disease in a patient with sclerosing cholangitis, inflammatory pseudotumors of the lung and multiple radiological patterns: a case report. Clinics (Sao Paulo) 2011; 66: 1983–6.  Back to cited text no. 5
    
6.
Yi ES, Sekiguchi H, Peikert T, Ryu JH, Colby TV. Pathologic manifestations ofimmunoglobulin(Ig)G4-related lung disease. Semin Diagn Pathol 2012; 29: 219–25.  Back to cited text no. 6
    
7.
Yamashita H, Takahashi Y, Ishiura H, Kano T, Kaneko H, Mimori A. Hypertrophicpachymeningitis and tracheobronchial stenosis in IgG4-related disease: casepresentation and literature review. Intern Med 2012; 51: 935–41.  Back to cited text no. 7
    
8.
Kitada M, Matuda Y, Hayashi S, et al. IgG4-related lung disease showing high standardized uptake values on FDG-PET: reportof two cases. J Cardiothorac Surg 2013; 8: 160.  Back to cited text no. 8
    
9.
Lin W, Lu S, Chen H, et al. Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients. Rheumatology (Oxford) 2015 Jun 22. pii: kev203  Back to cited text no. 9
    
10.
Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectionalstudy of 114 cases. Am J Surg Pathol2010; 34: 1812–9.  Back to cited text no. 10
    
11.
Ebbo M, Daniel L, Pavic M, et al. IgG4-related systemicdisease: features and treatment response in a Frenchcohort: results of a multicenter registry. Medicine2012; 91: 49–56.  Back to cited text no. 11
    
12.
Umehara H, Nakajima A, Nakamura T, et al. IgG4-related disease and its pathogenesis-cross-talk between innate and acquired immunity. Int Immunol 2014; 26: 585–95.  Back to cited text no. 12
    
13.
Smit W, Barnes E. The emerging mysteries of IgG4-related disease. Clin Med 2014; 14 Suppl 6: s56–60.  Back to cited text no. 13
    
14.
Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifestations of IgG4-related disease. Ann Am Thorac Soc 2014; 11: 1466–75.  Back to cited text no. 14
    
15.
Carter BW, Okumura M, Detterbeck FC, Marom EM. Approaching the patient with an anterior mediastinal mass: a guide for radiologists. J Thorac Oncol 2014; 9(9 Suppl 2): S110–18.  Back to cited text no. 15
    
16.
Motus IY, Bazhenov AV, Massard G. Surgery for huge mediastinal tumors. Asian Cardiovasc Thorac Ann 2015; 23: 846–50.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2]


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