Department of Respiratory Disease, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
Respiration. 2024;103(2):95-99. doi: 10.1159/000535395. Epub 2024 Jan 25.
Fibrosing mediastinitis is a benign but fatal disorder characterized by the proliferation of fibrous tissue in the mediastinum, causing encasement of mediastinal organs and extrinsic compression of adjacent bronchovascular structures. FM-associated pulmonary hypertension (FM-PH) is a serious complication of FM, resulting from the external compression of lung vessels. Pathologic assessment is important for etiologic diagnosis and effective treatment of this disease.
A 59-year-old male patient presented at our hospital and was diagnosed with FM-PH. He declined surgical biopsy that is the reference standard for pathologic assessment, in consideration of the potential risks. Therefore, an endobronchial ultrasound examination was performed, which identified the subcarinal lesion. Under ultrasound guidance, four needle aspirations were carried out, followed by one cryobiopsy. Histopathological examination of transbronchial needle aspiration specimens was inconclusive, while samples from cryobiopsy suggested a diagnosis of idiopathic FM. Further immunophenotyping demonstrated the infiltration of lymphocytes, macrophages, and FOXP3-positive cells in FM-PH.
Mediastinal cryobiopsy might be a novel and safe option for FM-PH patients who are unwilling or unsuitable for surgical procedure.
纤维性纵隔炎是一种良性但致命的疾病,其特征是纵隔内纤维组织增生,导致纵隔器官被包裹,并使相邻的支气管血管结构受到外在压迫。纤维性纵隔炎相关肺动脉高压(FM-PH)是 FM 的严重并发症,源于肺血管的外在压迫。病理评估对于该病的病因诊断和有效治疗非常重要。
一名 59 岁男性患者因 FM-PH 就诊于我院。考虑到手术活检的潜在风险,患者拒绝作为病理评估金标准的手术活检。因此,我们对其进行了支气管内超声检查,发现隆突下病变。在超声引导下,对病变进行了 4 次针吸活检和 1 次冷冻活检。经支气管针吸活检标本的组织病理学检查结果不明确,而冷冻活检标本提示特发性纤维性纵隔炎的诊断。进一步免疫表型分析显示,在 FM-PH 中存在淋巴细胞、巨噬细胞和 FOXP3 阳性细胞浸润。
对于不愿意或不适合手术的 FM-PH 患者,纵隔冷冻活检可能是一种新颖且安全的选择。