背景:肺肿瘤血栓性微血管病(PTTM)是一种罕见的预后不良的实体,经常在死后被诊断出来。PTTM是由肿瘤栓塞诱导的凝血级联激活引起的,肺微脉管系统中的纤维蛋白凝块形成和纤维细胞内膜增殖。
方法:患者为65岁女性,既往有卵巢高级别浆液性癌病史,出现胸痛和呼吸急促。与当前出现前两个月的胸部CT相比,胸部计算机断层扫描(CT)显示出无数新的肺结节以及小的模糊和斑片状混浊。她出现进行性呼吸衰竭并过期。肺限制性尸检显示双侧肺大致弥漫性亚厘米结节。显微镜检查显示肺实质显示许多由多形性肿瘤细胞组成的肿瘤栓子,在肺小动脉中有不同程度的纤维蛋白沉积和纤维细胞内膜增生。小动脉,和肺泡间隔的毛细血管。免疫组织化学证实了肿瘤细胞的卵巢起源。该发现与卵巢高级别浆液性癌转移继发的PTTM一致。文献综述了卵巢癌引起的PTTM。
结论:PTTM是一种罕见的与原发性卵巢恶性肿瘤相关的致命实体。该病例研究显示PTTM与高级别浆液性癌相关的临床病理特征。这将是文献中与这种关联的PTTM的第二例。初步研究结果以摘要形式报道[1]。
BACKGROUND: Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare entity with poor prognosis, and often diagnosed postmortem. PTTM is resulting from tumor emboli induced activation of coagulation cascade, fibrin clot formation and fibrocellular intimal proliferation in pulmonary microvasculature.
METHODS: The patient was a 65-year-old female, with past medical history of ovarian high-grade serous carcinoma, presented with chest pain and shortness of breath. The chest computed tomography (CT) revealed innumerable new lung nodules as well as small hazy and patchy opacities compared to the chest CT 2 months before current presentation. She developed progressive respiratory failure and expired. A lung-restricted autopsy showed diffuse subcentimetric nodules in bilateral lungs grossly. Microscopic examination revealed the lung parenchyma demonstrated numerous tumor emboli consisting of pleomorphic tumor cells with varying degrees of fibrin deposition and fibrocellular intimal proliferation in the pulmonary arterioles, small arteries, and capillaries in the alveolar septa. Immunohistochemistry confirmed the ovarian origin of the tumor cells. The findings were consistent with PTTM secondary to metastasis of ovarian high-grade serous carcinoma. Literature review of PTTM caused by ovarian cancer was conducted.
CONCLUSIONS: PTTM is a fatal entity with rare association with primary ovarian malignancy. This case study demonstrates the clinicopathological features of PTTM associated with high-grade serous carcinoma, and it will be the second case of PTTM with this association in the literature.