Servicio de Anatomía Patológica del Hospital Álvaro Cunqueiro (CHUVI), Vigo, Spain.
Servicio de Anatomía Patológica del Hospital Álvaro Cunqueiro (CHUVI), Vigo, Spain.
Rev Esp Patol. 2022 Sep;55 Suppl 1:S2-S6. doi: 10.1016/j.patol.2020.10.003. Epub 2020 Dec 11.
IgG4 related disease is a term used to describe a fibroinflammatory condition characterized by storiform fibrosis, inflammation with a dense lymphoplasmocytic infiltrate rich in plasma cells expressing IgG4, and often, if not always, raised serum levels of IgG4. We report a case of a patient with a past history of retroperitoneal fibrosis presenting with a swollen left testicle, who underwent an orchidectomy due to suspicion of malignancy. The surgical specimen revealed a paratesticular pseudotumor with histopathological and immunohistochemical characteristics of IgG4 related disease. To the best of our knowledge, just nine such cases have previously been reported, of which only three were manifestations of systemic disease, as in the present case. Whilst it is important to recognize the clinical and radiological features of this entity, histopathology is often essential in order to confirm the diagnosis.
IgG4 相关疾病是一种用于描述纤维炎症性疾病的术语，其特征为席纹状纤维化、炎症伴密集的淋巴浆细胞浸润，富含表达 IgG4 的浆细胞，且通常（如果并非总是）血清 IgG4 水平升高。我们报告了一例既往有腹膜后纤维化病史的患者，表现为左侧睾丸肿胀，因怀疑恶性肿瘤而行睾丸切除术。手术标本显示为副睾丸假性肿瘤，具有 IgG4 相关疾病的组织病理学和免疫组织化学特征。据我们所知，此前仅报道过 9 例此类病例，其中只有 3 例表现为全身性疾病，与本例相似。虽然认识到这种疾病的临床和影像学特征很重要，但组织病理学通常是确诊所必需的。
