About I, Capdeville J, Voigt J J, Bernard P, Michetti C, Faizon R
Service de médecine interne A, hôpital de Foix, France.
Rev Med Interne. 1994 Apr;15(4):279-81. doi: 10.1016/s0248-8663(94)80033-2.
Angiomyolipoma is a rare tumor, frequently associated with tuberous sclerosis, but not observed in its isolated form. Usually, patients with tuberous sclerosis presented with pathognomonic cutaneous and central nervous system lesions. But the thesis of "formes frustes" without typical stigmata was suggested. A case of bilateral and multifocal angiomyolipoma in a young woman without evidence of phacomatosis is reported. The association with a pulmonary lymphangiomatosis make us suspected "a forme fruste" of tuberous sclerosis.
血管平滑肌脂肪瘤是一种罕见的肿瘤，常与结节性硬化症相关，但不会以孤立形式出现。通常，结节性硬化症患者会出现具有诊断意义的皮肤和中枢神经系统病变。但也有人提出了无典型特征的“顿挫型”观点。本文报告了一例年轻女性双侧多灶性血管平滑肌脂肪瘤，该患者无色素沉着性错构瘤病证据。其与肺淋巴管瘤病的关联使我们怀疑这是结节性硬化症的“顿挫型”。
