Warth A, Herpel E, Schmähl A, Hoffmann H, Herth F J F, Schirmacher P, Dienemann H, Schnabel P A
Institute of Pathology, University of Heidelberg, Im Neuenheimer Feld 220/221, D-69120 Heidelberg, Germany.
Eur Respir J. 2008 Mar;31(3):678-80. doi: 10.1183/09031936.00021207.
Classical angiomyolipomas are benign tumours composed of various tissues, including components of fat, abnormal blood vessels and smooth muscle cells. They are often found in association with tuberous sclerosis complex (TSC). The present study reports a male patient affected by TSC with intermittent, massive chylous pleural effusions, who developed recurrent mediastinal angiomyolipomas. The tumours were characterised via histological and immunohistochemical methods. Although angiomyolipomas frequently occur in the kidneys of TSC patients, this case is the first report of mediastinal angiomyolipomas associated with TSC. Besides lymphangioleiomyomatosis, this differential diagnosis has to be taken into account in the case of chylous pleural effusions and mediastinal masses in tuberous sclerosis complex patients.
经典型血管平滑肌脂肪瘤是由多种组织组成的良性肿瘤，包括脂肪成分、异常血管和平滑肌细胞。它们常与结节性硬化症（TSC）相关。本研究报告了一名患有TSC的男性患者，伴有间歇性大量乳糜性胸腔积液，并发生了复发性纵隔血管平滑肌脂肪瘤。通过组织学和免疫组织化学方法对肿瘤进行了特征分析。虽然血管平滑肌脂肪瘤在TSC患者的肾脏中经常出现，但该病例是首例与TSC相关的纵隔血管平滑肌脂肪瘤报告。除淋巴管平滑肌瘤病外，对于结节性硬化症患者出现乳糜性胸腔积液和纵隔肿块的情况，必须考虑这种鉴别诊断。
