{"title":"模仿肾盂肿瘤的IgG4相关腹膜后纤维化:病例报告和文献综述","authors":"Sahin Gokhan, Dundar Mehmet, Senturk Taskin","doi":"10.1186/s12301-024-00408-3","DOIUrl":null,"url":null,"abstract":"Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of a fibroinflammatory mass in the retroperitoneum. Immunoglobulin-G4 related RPF was suggested as a secondary form of RPF and thought to be part of the spectrum of Immunoglobulin-G4 related diseases (IgG4-RD). Patients often present to the clinic because of flank pain. Ranging from mild to end-stage renal failure can be observed. The main purpose of treatment is to preserve renal function. As it is a rare condition, there is no definite treatment strategy. We report a case of 39-year-old man with left flank pain and diagnosis of IgG4-related RPF mimicking a renal pelvis tumor. A 39-year-old male patient presented with left flank pain. MRI suggested solid retroperitoneal mass associated with hydronephrosis in the left kidney collecting system. Upon identifying the retroperitoneal origin of the mass during nephroureterectomy, the procedure was concluded following the acquisition of frozen section and routine pathological samples from the lesion. In the histopathological examination, inflammatory cells were observed and specific immunohistochemistry for IgG-4 was detected focally positive. Following the placement of a DJ stent, immunosuppressive therapy was initiated with Prednol and Azathioprine. After a one-year follow-up period, during which the patient received immunosuppressive treatment and underwent tri-monthly DJ stent replacements, the DJ stent was subsequently removed, revealing complete regression of hydronephrosis. With the correct diagnosis and treatment of IgG4-related RPF, it is possible to prevent irreversible complications of the disease. Because it is a rare disease, case reports in the literature will be useful for treatment.","PeriodicalId":7432,"journal":{"name":"African Journal of Urology","volume":"94 1","pages":""},"PeriodicalIF":0.5000,"publicationDate":"2024-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"IgG4-related retroperitoneal fibrosis mimicking renal pelvis tumor: a case report and literature review\",\"authors\":\"Sahin Gokhan, Dundar Mehmet, Senturk Taskin\",\"doi\":\"10.1186/s12301-024-00408-3\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of a fibroinflammatory mass in the retroperitoneum. Immunoglobulin-G4 related RPF was suggested as a secondary form of RPF and thought to be part of the spectrum of Immunoglobulin-G4 related diseases (IgG4-RD). Patients often present to the clinic because of flank pain. Ranging from mild to end-stage renal failure can be observed. The main purpose of treatment is to preserve renal function. As it is a rare condition, there is no definite treatment strategy. We report a case of 39-year-old man with left flank pain and diagnosis of IgG4-related RPF mimicking a renal pelvis tumor. A 39-year-old male patient presented with left flank pain. MRI suggested solid retroperitoneal mass associated with hydronephrosis in the left kidney collecting system. Upon identifying the retroperitoneal origin of the mass during nephroureterectomy, the procedure was concluded following the acquisition of frozen section and routine pathological samples from the lesion. In the histopathological examination, inflammatory cells were observed and specific immunohistochemistry for IgG-4 was detected focally positive. Following the placement of a DJ stent, immunosuppressive therapy was initiated with Prednol and Azathioprine. After a one-year follow-up period, during which the patient received immunosuppressive treatment and underwent tri-monthly DJ stent replacements, the DJ stent was subsequently removed, revealing complete regression of hydronephrosis. With the correct diagnosis and treatment of IgG4-related RPF, it is possible to prevent irreversible complications of the disease. Because it is a rare disease, case reports in the literature will be useful for treatment.\",\"PeriodicalId\":7432,\"journal\":{\"name\":\"African Journal of Urology\",\"volume\":\"94 1\",\"pages\":\"\"},\"PeriodicalIF\":0.5000,\"publicationDate\":\"2024-02-13\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"African Journal of Urology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1186/s12301-024-00408-3\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"UROLOGY & NEPHROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"African Journal of Urology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1186/s12301-024-00408-3","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"UROLOGY & NEPHROLOGY","Score":null,"Total":0}
引用次数: 0
摘要
腹膜后纤维化(RPF)是一种罕见疾病,其特征是腹膜后出现纤维炎性肿块。免疫球蛋白-G4相关的腹膜后纤维化被认为是腹膜后纤维化的一种继发形式,也被认为是免疫球蛋白-G4相关疾病(IgG4-RD)的一部分。患者通常因腹部疼痛而就诊。可观察到从轻度到终末期的肾衰竭。治疗的主要目的是保护肾功能。由于该病十分罕见,目前尚无明确的治疗策略。我们报告了一例 39 岁男性左侧腹痛病例,诊断为 IgG4 相关 RPF(模仿肾盂肿瘤)。一名 39 岁男性患者因左侧腹痛就诊。核磁共振成像提示腹膜后实性肿块伴有左肾集合系统肾积水。在肾切除术中确定肿块来源于腹膜后后,在获取病变部位的冰冻切片和常规病理样本后,手术结束。在组织病理学检查中,观察到炎性细胞,IgG-4 的特异性免疫组化检测呈灶性阳性。植入 DJ 支架后,开始使用泼尼松和硫唑嘌呤进行免疫抑制治疗。在为期一年的随访期间,患者接受了免疫抑制治疗,并每三个月更换一次 DJ 支架,随后拆除了 DJ 支架,发现肾积水完全消退。通过对 IgG4 相关 RPF 的正确诊断和治疗,有可能避免该病出现不可逆转的并发症。由于这是一种罕见疾病,文献中的病例报告将有助于治疗。
IgG4-related retroperitoneal fibrosis mimicking renal pelvis tumor: a case report and literature review
Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of a fibroinflammatory mass in the retroperitoneum. Immunoglobulin-G4 related RPF was suggested as a secondary form of RPF and thought to be part of the spectrum of Immunoglobulin-G4 related diseases (IgG4-RD). Patients often present to the clinic because of flank pain. Ranging from mild to end-stage renal failure can be observed. The main purpose of treatment is to preserve renal function. As it is a rare condition, there is no definite treatment strategy. We report a case of 39-year-old man with left flank pain and diagnosis of IgG4-related RPF mimicking a renal pelvis tumor. A 39-year-old male patient presented with left flank pain. MRI suggested solid retroperitoneal mass associated with hydronephrosis in the left kidney collecting system. Upon identifying the retroperitoneal origin of the mass during nephroureterectomy, the procedure was concluded following the acquisition of frozen section and routine pathological samples from the lesion. In the histopathological examination, inflammatory cells were observed and specific immunohistochemistry for IgG-4 was detected focally positive. Following the placement of a DJ stent, immunosuppressive therapy was initiated with Prednol and Azathioprine. After a one-year follow-up period, during which the patient received immunosuppressive treatment and underwent tri-monthly DJ stent replacements, the DJ stent was subsequently removed, revealing complete regression of hydronephrosis. With the correct diagnosis and treatment of IgG4-related RPF, it is possible to prevent irreversible complications of the disease. Because it is a rare disease, case reports in the literature will be useful for treatment.