A Von Hippel-Lindau Disease?Associated Microcystic Adenoma of the Ethmoid Sinus Mimicking Metastatic Clear Cell Renal Cell Carcinoma
A Von Hippel-Lindau Disease?Associated Microcystic Adenoma of the Ethmoid Sinus Mimicking Metastatic Clear Cell Renal Cell Carcinoma
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冯·希佩尔-林道病？相关筛窦微囊性腺瘤，类似转移性透明细胞肾细胞癌
DOI：
10.1097/rlu.0000000000004421
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发表时间：
2022
影响因子：
10.6
通讯作者：
Nakamoto Yuji
中科院分区：
文献类型：
--
作者：
Tsukamoto Suzune;Koyasu Sho;Sugimoto Akihiko;Matsunaga Mami;Nakamoto Yuji
一名38岁男性，患有von Hippel-Lindau（VHL）病，有肾细胞癌病史，有2个月的复发性鼻出血病史。磁振造影发现一个位于左侧筛窦的微囊状肿瘤，并有强烈的对比增强。18 F-FDG PET/CT显示病灶内FDG摄取（SUV max，4.2）。在怀疑肾细胞癌转移的情况下，患者接受了2次手术切除。然而，基于形态学和免疫组化结果，患者最终被诊断为VHL相关的筛窦微囊腺瘤，这是一种发生在VHL疾病中的极其罕见的肿瘤。
A 38-year-old man with von Hippel-Lindau (VHL) disease and a history of renal cell carcinoma presented with a 2-month history of recurrent epistaxis. MRI revealed a microcystic tumor in the left ethmoid sinus with strong contrast enhancement. 18 F-FDG PET/CT showed FDG uptake (SUV max, 4.2) in the lesion. Under the suspicion of renal cell carcinoma metastasis, the patient underwent 2 surgical resections. However, based on the morphological and immunohistochemical findings, the patient was finally diagnosed with a VHL-associated microcystic adenoma of the ethmoid sinus, which is an extremely rare tumor that occurs in VHL disease.