我们介绍了一个与IgG4硬化性疾病相关的腹膜后肿瘤性纤维炎性病变的独特病例;它是IgG4相关疾病的罕见表现,位于腹膜后通常会导致弥漫性纤维化,而不是像肿块一样的病变.一名49岁的男子出现在急诊科,抱怨腹痛和呕吐。随后用腹部超声检查,CT,MRI显示有不明来源的大腹膜后肿块,异质,具有在MRI中最佳可视化的同心圆图案。病灶被切除,组织学和免疫组织化学研究显示腹膜后的IgG4相关的纤维化性病变。
We present a unique case of a retroperitoneal tumefactive fibroinflammatory lesion related to IgG4-sclerosing disease; it is a rare manifestation of the IgG4-related disease, which usually causes diffuse fibrosis when located in the retroperitoneum, rather than mass-like lesions. A 49-year-old man presented to the emergency department complaining of abdominal pain and vomiting. Subsequent testing with abdominal ultrasound, CT, and MRI revealed a large retroperitoneal mass of unknown origin, heterogenous, with a concentric circles pattern best visualized in MRI. The lesion was resected, and the histological and immunohistochemical studies revealed an IgG4-related tumefactive fibroinflammatory lesion of the retroperitoneum.