Acta Medica International

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

Mediastinal mass in a patient with IgG4-related disease


Kesato Iguchi1, Osamu Ishibashi1, Norio Takayashiki2, Hiroaki Satoh3,  
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

Correspondence Address:
Hiroaki Satoh
Division of Respiratory Medicine, Mito Medical Center, University of Tsukuba, Miya-machi 3-2-7, Mito, Ibaraki, 310-0015
Japan

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.



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;3:216-218


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 Oct 21 ];3:216-218
Available from: http://www.actamedicainternational.com/text.asp?2016/3/1/216/209713


Full Text



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 Introduction



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



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}{Figure 2}

 Discussion



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.

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