Kreisman H, Robitaille Y, Dionne G P, Palayew M J
Cancer. 1978 Jul;42(1):364-72. doi: 10.1002/1097-0142(197807)42:1<364::aid-cncr2820420155>3.0.co;2-m.
A 48-year-old woman presented with the classical clinicopathological features of the lymphangiomyomatosis syndrome. After a three year stable period, there was the onset of a rapidly progressive downhill course unresponsive to dietary, bronchodilator and corticosteroid therapy. Pathological findings were characterized by widespread pulmonary, thoracic duct and lymph node involvement. There was a mediastinal lymphangiomyoma growing within the distal thoracic duct, and a similar lesion within the left kidney which could clinically mimic an angiomyolipoma. Comments are also made on the finding of a parathyroid adenoma. The physiopathology and possible resemblance to "formes frustes" of tuberous sclerosis are discussed.
一名48岁女性表现出淋巴管肌瘤病综合征的典型临床病理特征。在经历了三年的稳定期后，病情开始迅速进展，呈下坡趋势，对饮食、支气管扩张剂和皮质类固醇治疗均无反应。病理检查结果显示肺部、胸导管和淋巴结广泛受累。在胸导管远端有一个纵隔淋巴管肌瘤生长，左肾内也有一个类似病变，临床上可酷似血管平滑肌脂肪瘤。文中还对甲状旁腺腺瘤的发现进行了评论。讨论了其生理病理学以及与结节性硬化症“顿挫型”可能的相似之处。
