PDF(Pubmed)
Abstract：
OBJECTIVE: This study aimed to investigate the histological characteristics and treatment efficacy of non-immunoglobulin G4-related fibrosing mediastinitis and discuss differential diagnoses for this rare entity.
METHODS: We present a case study of non-immunoglobulin G4-related fibrosing mediastinitis diagnosed on core biopsy and treated with steroids. A total of four 18-gauge core needle biopsy specimens were obtained for surgical pathology. Analysis of the patient\'s medical history, radiological characteristics of fibrosing mediastinitis, histological features, immunohistochemistry results, the differential diagnosis and treatment efficacy of different types of fibrosing mediastinitis was performed.
RESULTS: This report describes a unique presentation of fibrosing mediastinitis (syncope and weight loss) that was concerning for malignancy. Histological, laboratory and radiographical studies confirmed the diagnosis of non-immunoglobulin G4-related fibrosing mediastinitis. The patient received corticosteroid treatment which showed marked improvement after 1 month of treatment.
CONCLUSIONS: Fibrosing mediastinitis is an extremely uncommon entity with unknown pathogenesis, and it is more important to rule out malignancy and infection than to delineate between fibrosing mediastinitis and IgG4-related disease. In doing this, we may reasonably initiate a trial of corticosteroids which may prove beneficial, as in this patient. More studies on the pathogenesis of fibrosing mediastinitis are necessary to guide better directed treatments.
METHODS: We present a case study of non-immunoglobulin G4-related fibrosing mediastinitis diagnosed on core biopsy and treated with steroids. A total of four 18-gauge core needle biopsy specimens were obtained for surgical pathology. Analysis of the patient\'s medical history, radiological characteristics of fibrosing mediastinitis, histological features, immunohistochemistry results, the differential diagnosis and treatment efficacy of different types of fibrosing mediastinitis was performed.
RESULTS: This report describes a unique presentation of fibrosing mediastinitis (syncope and weight loss) that was concerning for malignancy. Histological, laboratory and radiographical studies confirmed the diagnosis of non-immunoglobulin G4-related fibrosing mediastinitis. The patient received corticosteroid treatment which showed marked improvement after 1 month of treatment.
CONCLUSIONS: Fibrosing mediastinitis is an extremely uncommon entity with unknown pathogenesis, and it is more important to rule out malignancy and infection than to delineate between fibrosing mediastinitis and IgG4-related disease. In doing this, we may reasonably initiate a trial of corticosteroids which may prove beneficial, as in this patient. More studies on the pathogenesis of fibrosing mediastinitis are necessary to guide better directed treatments.
摘要：
目的：本研究旨在探讨非免疫球蛋白G4相关性纤维化纵隔炎的组织学特征和治疗效果，并讨论这种罕见实体的鉴别诊断。
方法：我们提供了一个在核心活检中诊断并接受类固醇治疗的非免疫球蛋白G4相关性纤维化纵隔炎的案例研究。共获得4个18号芯针活检标本，用于手术病理。患者病史分析,纤维化纵隔炎的放射学特征，组织学特征，免疫组织化学结果，对不同类型的纤维性纵隔炎进行鉴别诊断和治疗。
结果：本报告描述了与恶性肿瘤有关的纤维化纵隔炎（晕厥和体重减轻）的独特表现。组织学,实验室和影像学研究证实了非免疫球蛋白G4相关性纤维化纵隔炎的诊断.患者接受皮质类固醇治疗，治疗1个月后有明显改善。
结论：纤维性纵隔炎是一种极其罕见的实体，发病机制未知，与划分纤维化纵隔炎和IgG4相关疾病相比,排除恶性肿瘤和感染更为重要.在这样做的时候，我们可以合理地启动皮质类固醇的试验，这可能被证明是有益的，就像这个病人一样。有必要对纤维化纵隔炎的发病机制进行更多的研究，以指导更好的定向治疗。
方法：我们提供了一个在核心活检中诊断并接受类固醇治疗的非免疫球蛋白G4相关性纤维化纵隔炎的案例研究。共获得4个18号芯针活检标本，用于手术病理。患者病史分析,纤维化纵隔炎的放射学特征，组织学特征，免疫组织化学结果，对不同类型的纤维性纵隔炎进行鉴别诊断和治疗。
结果：本报告描述了与恶性肿瘤有关的纤维化纵隔炎（晕厥和体重减轻）的独特表现。组织学,实验室和影像学研究证实了非免疫球蛋白G4相关性纤维化纵隔炎的诊断.患者接受皮质类固醇治疗，治疗1个月后有明显改善。
结论：纤维性纵隔炎是一种极其罕见的实体，发病机制未知，与划分纤维化纵隔炎和IgG4相关疾病相比,排除恶性肿瘤和感染更为重要.在这样做的时候，我们可以合理地启动皮质类固醇的试验，这可能被证明是有益的，就像这个病人一样。有必要对纤维化纵隔炎的发病机制进行更多的研究，以指导更好的定向治疗。
