Eichler A, Ukena D, Wilkens H, Reitnauer K, Girmann M, Remberger K, Kramann B, Sybrecht G W
Medizinische Universitätsklinik V, Pathologisches Institut der Universität des Saarlandes.
Pneumologie. 2000 Jun;54(6):243-8. doi: 10.1055/s-2000-8994.
A diffuse angiomatosis affecting thoracal organs is a rare, frequently fatal disorder occurring mainly in childhood. The definite diagnosis is usually made by autopsy. A specific treatment does not exist. In the case report presented here we describe the course of an 37-year old patient presenting initially with dyspnea and hemoptysis. Radiological investigations showed a mass lesion in the anterior superior mediastinum, bihilar masses and a periesophageal mass with soft tissue density and a pericardial effusion. Bilateral interstitial infiltrations were also present. CT-guided mediastinal biopsies and bronchial mucosal biopsies were nonspecific. Open-lung biopsy revealed the diagnosis of an extensive diffuse angiomatosis affecting mediastinum, pericardium and pleura. During the 18-month monotherapy with interferon-alpha 2a the patient showed significant improvements of clinical as well as laboratory and radiological parameters. The presented case confirms the experience of several authors, that the diagnosis of a diffuse thoracal authors can only be made by open lung biopsy or autopsy. The presented case also underlines the possibility of a palliative therapy of aggressive angiomatous diseases with interferon-alpha 2a.
一种累及胸部器官的弥漫性血管瘤病是一种罕见的、通常致命的疾病，主要发生在儿童期。确切诊断通常通过尸检做出。不存在特异性治疗方法。在本文介绍的病例报告中，我们描述了一名37岁患者的病程，该患者最初表现为呼吸困难和咯血。影像学检查显示前上纵隔有肿块病变、双侧肺门肿块、食管周围有软组织密度肿块及心包积液。还存在双侧间质浸润。CT引导下的纵隔活检和支气管黏膜活检均无特异性。开胸肺活检确诊为累及纵隔、心包和胸膜的广泛性弥漫性血管瘤病。在使用α-2a干扰素进行18个月的单一治疗期间，患者的临床、实验室及影像学参数均有显著改善。该病例证实了几位作者的经验，即弥漫性胸部血管瘤病的诊断只能通过开胸肺活检或尸检做出。该病例还强调了用α-2a干扰素对侵袭性血管瘤病进行姑息治疗的可能性。
