Cagnano M, Benharroch D, Geffen D B
Institute of Pathology, Soroka Medical Center, Beer-Sheva, Israel.
Arch Pathol Lab Med. 1991 Dec;115(12):1257-9.
We describe a 55-year-old woman with pulmonary lymphangioleiomyomatosis. The patient died of respiratory failure 9 years after diagnosis. An autopsy confirmed the diagnosis and disclosed multiple soft-tissue tumors, including a large solitary fibrous tumor of the lung, a huge cavernous hemangioma of the liver, a meningioma of the right pontocerebellar angle, and a focus of nodular stromal hyperplasia of the ovary. In addition, endocrine tumors, including a papillary carcinoma of the thyroid gland and a parathyroid adenoma, were also found. There was no evidence of tuberous sclerosis or angiomyolipoma of the kidney. The connection between pulmonary lymphangioleiomyomatosis and tuberous sclerosis is discussed.
我们描述了一名患有肺淋巴管平滑肌瘤病的55岁女性。该患者在诊断9年后死于呼吸衰竭。尸检证实了诊断，并发现了多个软组织肿瘤，包括一个巨大的肺孤立性纤维瘤、一个巨大的肝脏海绵状血管瘤、一个右桥小脑角脑膜瘤以及一个卵巢结节性间质增生灶。此外，还发现了内分泌肿瘤，包括甲状腺乳头状癌和甲状旁腺腺瘤。没有证据表明存在结节性硬化症或肾血管平滑肌脂肪瘤。本文讨论了肺淋巴管平滑肌瘤病与结节性硬化症之间的联系。
