Ashayeri Neda, Zarei Elham, Mortazavi Nafiseh, Moosazadeh Amirhesam
Department of Pediatric Hematology and Oncology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
Aliasghar Clinical Research Development Center, Department of Pediatrics, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.
J Med Case Rep. 2025 May 13;19(1):222. doi: 10.1186/s13256-025-05274-3.
Extrarenal Wilms tumor is an extremely rare condition, typically documented only in isolated case reports. Unlike classical intrarenal Wilms tumors, extrarenal Wilms tumors arise outside the kidney, often presenting a diagnostic challenge owing to its unusual location and overlapping features with other retroperitoneal tumors. Its rarity necessitates further documentation to improve recognition and management. This report presents a case of extrarenal Wilms tumor located in the retroperitoneal space of a 6-year-old girl.
A 6-year-old white Iranian girl presented with abdominal pain and swelling in the upper left abdomen. Physical examination revealed a firm, nontender, immobile mass. Ultrasound imaging identified a well-defined mass with significant necrotic and cystic areas. Abdominopelvic computed tomography scan showed a large mass on the left side of the abdomen, exerting pressure on the adjacent pancreas, spleen, and left kidney. The patient underwent laparotomy and received 19 weeks of chemotherapy, including actinomycin-D and vincristine. Post-treatment, she fully recovered and underwent monthly sonography follow-ups for 6 months after completing chemotherapy and has shown no signs of recurrence to date.
Extrarenal Wilms tumor should be considered in the differential diagnosis of abdominal pain, especially in young children, owing to its rarity and the potential for misdiagnosis as other retroperitoneal tumors. A definitive diagnosis is made through surgical intervention followed by histopathological examination.
肾外威尔姆斯瘤是一种极为罕见的病症，通常仅在个别病例报告中有记载。与经典的肾内威尔姆斯瘤不同，肾外威尔姆斯瘤起源于肾脏之外，因其不寻常的位置以及与其他腹膜后肿瘤的重叠特征，常常带来诊断挑战。其罕见性使得有必要进一步记录以提高识别和管理水平。本报告呈现了一例位于一名6岁女孩腹膜后间隙的肾外威尔姆斯瘤病例。
一名6岁的伊朗白人女孩因左上腹疼痛和肿胀前来就诊。体格检查发现一个质地坚硬、无压痛、固定不动的肿块。超声成像显示一个边界清晰的肿块，有明显的坏死和囊性区域。腹盆腔计算机断层扫描显示腹部左侧有一个大肿块，对相邻的胰腺、脾脏和左肾施加压力。该患者接受了剖腹手术，并接受了19周的化疗，包括放线菌素-D和长春新碱。治疗后，她完全康复，化疗结束后每月进行超声检查随访6个月，迄今为止未显示复发迹象。
由于肾外威尔姆斯瘤罕见且有被误诊为其他腹膜后肿瘤的可能性，在腹痛的鉴别诊断中，尤其是在幼儿中，应考虑到这种疾病。通过手术干预并随后进行组织病理学检查可做出明确诊断。
