Karimi S, Mohammadi F, Pejhan S, Bakhshayeshkaram M, Akhavan-Azaril P, Bahadori M
Department of Pathology, National Institute of Tuberculosis and Lung Disease (NRITLD), Shahid Beheshti University of Medical Science (SBUMS), Tehran, Iran.
Monaldi Arch Chest Dis. 2006 Sep;65(3):172-4. doi: 10.4081/monaldi.2006.565.
Angiomatosis is a rare, benign but clinically extensive and serious vascular lesion of soft tissue. Hereby, we report a case of diffuse angiomatosis of mediastinum, presenting with hemomediastinum and bilateral massive hemothorax in a 19-year old boy. On medical imaging, mediastinal widening along with enhanced small vessels and capillaries were detected. On operation, hemorrhagic sponge-like vascular tissue patches were seen extensively in pericardium, pleura, lymph node and thymus as well. The lesion was debulked. The pathologic evaluation revealed characteristic features of soft tissue angiomatosis involving thymus, lymph node, pleura, pericardium and its fibrofatty tissue. Diffuse angiomatosis should be considered for differential diagnosis of vascular lesions of anterior mediastinum.
血管瘤病是一种罕见的、良性但临床上广泛且严重的软组织血管病变。在此，我们报告一例纵隔弥漫性血管瘤病，一名19岁男孩表现为纵隔积血和双侧大量血胸。在医学影像上，检测到纵隔增宽以及小血管和毛细血管强化。手术中，在心包、胸膜、淋巴结和胸腺中也广泛可见出血性海绵状血管组织斑块。对病变进行了减瘤手术。病理评估显示软组织血管瘤病累及胸腺、淋巴结、胸膜、心包及其纤维脂肪组织的特征性表现。对于前纵隔血管病变的鉴别诊断应考虑弥漫性血管瘤病。
