Conrad S, Wagner B, Hamper K, Henke R P
Urologische Universitätsklinik Hamburg-Eppendorf.
Urologe A. 1994 Jan;33(1):76-9.
Lymphangiomyomatosis is an extremely rare benign disease characterized by extensive proliferation of smooth muscle cells within lymphatic vessels and lymph nodes. While 10-15% of all patients show concomitant renal angiomyolipomas, no association so far has been reported with renal cell carcinoma. We present a case with coincident lymphangiomyomatosis, renal cell carcinoma and renal angiomyolipoma. The possible underlying pathogenesis is discussed. Classifying lymphangiomyomatosis as a "forme fruste" of tuberous sclerosis allows postulation of a common pathogenesis for all three entities.
淋巴管平滑肌瘤病是一种极其罕见的良性疾病，其特征是淋巴管和淋巴结内平滑肌细胞广泛增殖。虽然所有患者中有10% - 15%伴有肾血管平滑肌脂肪瘤，但迄今为止尚未报道与肾细胞癌有关联。我们报告一例同时患有淋巴管平滑肌瘤病、肾细胞癌和肾血管平滑肌脂肪瘤的病例。并对可能的潜在发病机制进行了讨论。将淋巴管平滑肌瘤病归类为结节性硬化症的“顿挫型”，使得可以推测这三种疾病具有共同的发病机制。
