每日一题_2023.03.03

A 62-year-old man undergoing abdominal ultrasonography for the evaluation of gallstones was found to have a retroperitoneal mass. A physical examination and the results of routine laboratory studies were normal. A computed tomographic (CT) urogram was completed (left), and a subsequent positron-emission tomography–CT showed no hypermetabolic activity. He underwent stent placement in both ureters, and a core-biopsy of the perinephric soft tissue was obtained. Hematoxylin and eosin staining of the specimen is shown (right). What is the diagnosis?

一名62岁男性在接受腹部超声检查评估胆囊结石时发现腹膜后肿块。体格检查和常规实验室检查结果正常。完成了计算机断层扫描(CT)尿路造影(左)，随后的正电子发射断层扫描- CT显示无高代谢活性。患者的双侧输尿管均放置了支架，并对肾周软组织进行了粗针活检。苏木精-伊红染色显示标本(右)。诊断是什么?

A. Erdheim–Chester disease 

B. IgG4-related disease 

C. Langerhans cell histiocytosis 朗格汉斯细胞组织细胞增生症

D. Lymphoma 淋巴瘤

E. Sarcoidosis 结节病

答案解析：

正确的答案是Erdheim -Chester疾病。 Erdheim – Chester疾病是一种非胰岛细胞的组织细胞增多症，通常在长骨中表现出硬化病变。 它还与骨外部的发现有关，包括渗透腹膜腹膜隔膜组织，导致横截面成像上的“毛茸茸的肾脏”外观，如该患者所示。 在对活检标本的组织病理学检查中，注意到弥漫性浸润呈淡染色的组织形成细胞（箭头）。

正确答案：A