Cicioni Gaia, Iannone Immacolata, Crocetti Daniele, Padua Cristina DE, Coppola Alessandro, Petramala Luigi, Sapienza Paolo, Letizia Claudio
Department of Surgery, "Sapienza" University, Rome, Italy.
Department of Surgery, "Sapienza" University, Rome, Italy;
In Vivo. 2025 Jul-Aug;39(4):2154-2164. doi: 10.21873/invivo.14011.
BACKGROUND/AIM: Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated condition characterized by fibroinflammatory lesions affecting multiple organs. When localized in the retroperitoneum, it may mimic malignancy and often leads to surgical intervention. We report the case of a 21-year-old male with retroperitoneal IgG4-RD and review the relevant literature, emphasizing the diagnostic challenges and importance of differential diagnosis.
A case report of a 21-year-old man with retroperitoneal IgG4-RD is presented. A literature review was conducted Scopus, Embase, and Medline through December 2024, selecting studies with immunohistochemically confirmed retroperitoneal IgG4-RD. Data were independently extracted and analyzed using SPSS software.
Of 74 articles retrieved, 22 met the inclusion criteria, totaling 23 patients including our case. The mean age was 62 years, with a male predominance (65%). The left ureter was most commonly involved (52%). Frequent symptoms included localized abdominal pain (61%) and hydronephrosis (96%). Imaging often suggested malignancy, leading 74% of patients to undergo surgery. Histopathology confirmed IgG4-RD in all cases, though only 30% showed IgG4 immunohistochemical positivity.
Retroperitoneal IgG4-RD closely mimics malignancy, posing significant diagnostic challenges. Elevated serum IgG4 levels and tissue biopsy are critical for accurate diagnosis. IgG4-RD should be considered in patients with unexplained retroperitoneal masses to avoid unnecessary surgery and ensure appropriate treatment.
背景/目的：免疫球蛋白G4相关性疾病（IgG4-RD）是一种全身性免疫介导疾病，其特征为影响多个器官的纤维炎性病变。当病变局限于腹膜后时，可能类似恶性肿瘤，常导致手术干预。我们报告一例21岁男性腹膜后IgG4-RD病例，并回顾相关文献，强调诊断挑战和鉴别诊断的重要性。
报告一例21岁男性腹膜后IgG4-RD病例。通过检索Scopus、Embase和Medline数据库至2024年12月，选择经免疫组化确诊的腹膜后IgG4-RD研究。数据独立提取并使用SPSS软件进行分析。
检索到的74篇文章中，22篇符合纳入标准，包括我们的病例在内共23例患者。平均年龄为62岁，男性占优势（65%）。左侧输尿管最常受累（52%）。常见症状包括局部腹痛（61%）和肾积水（96%）。影像学检查常提示恶性肿瘤，导致74%的患者接受手术。所有病例组织病理学均确诊为IgG4-RD，但仅30%显示IgG4免疫组化阳性。
腹膜后IgG4-RD酷似恶性肿瘤，带来重大诊断挑战。血清IgG4水平升高和组织活检对准确诊断至关重要。对于原因不明的腹膜后肿块患者，应考虑IgG4-RD，以避免不必要的手术并确保适当治疗。
