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        west china medical publishers
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        find Keyword "Rosai-Dorfman病" 2 results
        • 肝臟原發性Rosai-Dorfman病:1例報道并文獻復習

          目的總結肝臟原發性Rosai-Dorfman(RD)病的臨床及影像學表現、病理特征、鑒別診斷、治療及預后。方法回顧性收集廣東醫科大學附屬醫院收治的1例肝臟原發性RD病的相關資料并結合國內外相關文獻報道病例進行分析。結果本例患者為一41歲女性患者,因“反復右上腹疼痛2+ 個月”就診,缺乏特異性體征和癥狀,術前誤診為肝癌。行手術完整切除肝右葉腫物,約4.0 cm×4.0 cm×3.7 cm大小,質地稍硬,邊界清;術后組織病理學檢查見腫物有大量淋巴細胞和漿細胞,其中可見胞質嗜酸、胞核稍大的梭形細胞,邊界較清;免疫組織化學染色發現,RD病組織中S-100、八聚體結合轉錄因子-2、cyclinD1、CD68、CD163等標志物呈不同程度的陽性。術后病理檢查診斷為肝臟原發性RD病,術后隨訪7個月腫瘤無復發。復習國內外文獻共收集到2例肝臟原發性RD病,術前皆誤診為肝惡性腫瘤,均行根治性手術切除,術后經組織病理學檢查才確診,術后隨訪均未見復發。結論RD病是一種罕見的非朗格漢斯細胞組織細胞增多癥,原發于肝臟的RD病更加罕見,容易誤診,需與肝細胞癌、肝內膽管細胞癌、肝腺瘤及肝轉移瘤鑒別,確診依賴于術后組織病理學檢查,手術治療效果較好,預后良好,術后需嚴密隨訪。

          Release date:2024-09-25 04:19 Export PDF Favorites Scan
        • Diagnosis and Treatment of Extranodal Rosai-Dorfman Disease in Nasal Cavity and Nasopharynx

          ObjectiveTo investigate the clinical manifestations, diagnosis, treatment and prognosis of Rosai-Dorfman disease (RDD) in nasal cavity and nasopharynx. MethodsWe retrospectively analyzed the clinical data of 4 patients with extranodal RDD admitted to the Department of Otolaryngology, Head and Neck Surgery of our hospital between November 2009 and August 2013. ResultsAll patients received complete surgical removal of the lesions. Histopathological examination showed that large histocytes had an abundant foamy cytoplasm containing ingested lymphocytes as a result of emperipolesis, and were strongly positive for S-100 and CD68 on immunohistochemical analysis, but negative for CD1a. All patients were followed up for 28 to 45 months (averaging 35 months), and no recurrence was observed during the follow-up period. ConclusionRDD is a rare disease, and the exact etiology and pathogenesis of RDD are not fully understood. There is no unified treatment plan recommended, and complete surgical removal of the lesions is an effective method. Minimally invasive surgery may be a good choice for treatment of this disease.

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