Pulmonary Epithelioid Hemangioendothelioma with an Initial Presentation of Consolidation: Use of Endobronchial Ultrasonography for Diagnosis-A Case Report and Literature Review

肺上皮樣血管內皮瘤是一個低惡性度的少見疾病。由於僅使用氣管鏡來診斷周邊肺部病灶的正確率偏低，此類血管腫瘤診斷的方法通常是由開胸做肺切片或手術切除得到。然而、隨著氣管內超音波術的進步，已幫助周邊肺部病灶的正確定位與提升診斷率。此處，我們報告了一位五十四歲女性，起初表現肺堅實，伴隨咳嗽、氣促及胸部X光發現右下肺葉實質化與右肋膜積水。藉由支氣管超音波導引肺切片得到檢體並藉免疫組織染色診斷爲肺上皮樣血管內皮瘤。其氣管內超音波圖像呈現不均勻的斑點狀低回音散佈在病灶周圍，並伴有難以界定的邊緣、但無散在性線狀的空氣支氣管造影像。這特殊呈像與病理組織的周邊血管靜脈竇結構一致。藉此病例，我們回顧了罕見的肺上皮樣血管內皮瘤與氣管內超音波鑑別診斷圖像特徵。

關鍵字

肺上皮樣血管內皮瘤；肺堅實；支氣管鏡超音波

並列摘要

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare disease of borderline malignancy. The diagnosis of the vascular tumor is usually established by open lung biopsy or surgical removal of the tumor, since the diagnostic yield is limited in peripheral lung abnormalities using bronchoscopy alone. Nonetheless, advances in miniature probes and endobronchial ultrasonography (EBUS) have facilitated the accurate localization of peripheral lung lesions and the diagnostic yield. Herein, we describe a 54-year-old woman with symptoms of cough and shortness of breath, as well as right lower lobe consolidation and right pleural effusion, as evidenced on chest radiographs. The immunohistochemical diagnosis of PEH was reached by EBUS-guided transbronchial lung biopsy (EBUS-TBLB). EBUS revealed a specific pattern of heterogenous mottled hypoechoic densities with the presence of an ill-defined margin, but an absence of linear discrete air-bronchograms, which consisted of a structure of peripheral vascular sinusoids on histology. The literature concerning PEH and the characteristics of EBUS in differentiating benign from malignant lung lesions are reviewed.

並列關鍵字

pulmonary epithelioid hemangioendothelioma PEH ； consolidation ； endobronchial ultrasonography EBUS

國際替代計量

Pulmonary Epithelioid Hemangioendothelioma with an Initial Presentation of Consolidation: Use of Endobronchial Ultrasonography for Diagnosis-A Case Report and Literature Review

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