IgG4相关的附睾疾病,类似睾丸恶性肿瘤-一种罕见的实体

L. Krishna, S. Krishnamoorthy, H. Sekar, S. Murali, R. Swaminathan, N. Kumaresan
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引用次数: 1

摘要

免疫球蛋白G4相关疾病(IgG4 RD)是一种全身性纤维炎症性疾病,通常表现为多器官受累。我们报告一例罕见的54岁男性附睾IgG4 RD。患者表现为左睾丸进行性肿胀。临床诊断为肺结核。阴囊超声显示一个相对异回声的肿块,累及左侧睾丸附近的左侧附睾。有局灶性梭形细胞增殖,浆细胞和瘢痕疙瘩样胶原数量增加。免疫组化检测波形蛋白和IgG4阳性,CD34阴性。IgG4的血清水平升高(165 mg/dL)。腹部和胸部的计算机断层扫描没有显示任何全身受累。HE被用于切除附睾肿块。术中发现肿块与左睾丸紧密粘连,且与之不可分离,因此需要切除左睾丸。血清IgG4水平升高的组织病理学和免疫组织化学证实了附睾IgG4 RD的诊断。据我们所知,这种情况是一种极为罕见的疾病,医学文献中只有极少数孤立的附睾IgG4 RD病例报道,没有其他全身表现。
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IgG4 Related Disease of Epididymis, Mimicking Testicular Malignancy – A Rare Entity
Immunoglobulin G4 related disease (IgG4-RD) is a systemic fibro inflammatory condition that usually presents with multiorgan involvement. We present a rare case of 54 year old male with an isolated IgG4-RD of epididymis. The patient presented with a progressive swelling of the left testicle. A clinical diagnosis of tuberculosis was made. Ultrasound scrotum showed a relatively hetero-echoic mass lesion involving the left epididymis in close proximity to the left testis. There was a focal spindle cell proliferation and an increase in number of plasma cells and keloid like collagen. Immunohistochemistry was positive for vimentin and IgG4 and negative for CD34. Serum level of IgG4 was elevated (165 mg per dL). Computed tomography of abdomen and thorax did not show any systemic involvement. HE was posted for excision of the epididymal mass. Intraoperatively, the mass was found to be densely adherent to left testicle and inseparable from it, necessitating left total orchiectomy. Histopathology and immunohistochemistry with elevated serum IgG4 levels confirmed the diagnosis of IgG4-RD of the epididymis. To the best of our knowledge, this condition is an extremely rare entity, with only very few cases of isolated IgG4-RD of epididymis reported in medical literature, with no other systemic manifestations.
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