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[肾血管平滑肌脂肪瘤伴局部区域淋巴结定位并与肺结节性硬化症相关:一例报告]

[Renal angiomyolipoma with locoregional lymph node localization associated with pulmonary tuberous sclerosis: a case report].

作者信息

Ambrosiani L, Bottanelli A, Marcelli G, Rovellini P, Scattoni V, Tavani E

机构信息

Divisione di Urologia, Ospedale di Rho, Milano.

出版信息

Arch Ital Urol Androl. 1995 Sep;67(4):283-7.

PMID:7581533
Abstract

The angiomyolipoma is a rare tumor of the kidney with benign clinical evolution which can be found in more than half of the female patients with tuberous sclerosis. An uncommon case of renal angiomyolipoma with extracapsular extension, inter-aortocaval lymph node involvement and pulmonary lymphangiomyomatosis with a clinical history of spontaneous recurrent pneumothorax but without the neurological signs of the tuberous sclerosis, is described. The natural history of this disease which has various clinical forms of presentations is still unclear. The role of the lymph-node dissection in the surgical treatment of this tumor is discussed.

摘要

血管平滑肌脂肪瘤是一种罕见的肾脏肿瘤,临床病程呈良性,在超过半数的结节性硬化症女性患者中可发现。本文描述了一例罕见的肾血管平滑肌脂肪瘤病例,该肿瘤具有包膜外扩展、主动脉腔间淋巴结受累及肺淋巴管平滑肌瘤病,患者有自发性复发性气胸的临床病史,但无结节性硬化症的神经学体征。这种具有多种临床表现形式的疾病的自然病史仍不清楚。本文还讨论了淋巴结清扫在该肿瘤外科治疗中的作用。

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