Kitaichi M, Nagai S, Nishimura K, Itoh H, Asamoto H, Izumi T, Dail D H
Laboratory of Anatomic Pathology, Kyoto University Hospital, Japan.
Eur Respir J. 1998 Jul;12(1):89-96. doi: 10.1183/09031936.98.12010089.
This investigation studied the general conditions and prognostic factors of pulmonary epithelioid haemangioendothelioma (PEH), which is a rare disease. Twenty-one patients were collected throughout Asia by a questionnaire. Age at the detection or onset of symptoms of PEH was 14-64 yrs (mean 44 yrs). Males were more likely to be detected by symptoms (4/8, 50%) than were females (1/13, 8%). Fifteen showed bilateral multiple nodular opacities. Partial spontaneous regression occurred in three asymptomatic patients (one male and two females, all with bilateral multiple nodular opacities) 5, 13 and 15 yrs after detection. Two of the three patients with pleural effusion died within 1 yr, while the 16 patients with no effusion were alive more than 1 yr later (p<0.05). Histologically, two patients with fibrinofibrous pleuritis and extrapleural proliferation of tumour cells died within 2 yrs, while only one of 14 patients lacking such manifestations died within the same period (p<0.05). All three patients without spindle tumour cells survived for 12 yrs after the diagnosis, while all four patients with such cells died during the same period (p<0.05). In conclusion, 21 patients with pulmonary epithelioid haemangioendothelioma were reported, of whom three demonstrated partial spontaneous regression, and adverse prognostic features were identified.
本研究调查了罕见疾病肺上皮样血管内皮瘤（PEH）的一般情况和预后因素。通过问卷调查在亚洲各地收集了21例患者。PEH症状检测或出现时的年龄为14 - 64岁（平均44岁）。男性比女性更易出现症状（4/8，50% 对比1/13，8%）。15例表现为双侧多发结节状阴影。3例无症状患者（1例男性和2例女性，均有双侧多发结节状阴影）在检测后5年、13年和15年出现部分自发消退。3例胸腔积液患者中有2例在1年内死亡，而16例无胸腔积液患者在1年后仍存活（p<0.05）。组织学上，2例有纤维蛋白性纤维胸膜炎和肿瘤细胞胸膜外增殖的患者在2年内死亡，而14例无此类表现的患者中同期仅1例死亡（p<0.05）。3例无梭形肿瘤细胞的患者在诊断后存活了12年，而4例有此类细胞的患者同期均死亡（p<0.05）。总之，报告了21例肺上皮样血管内皮瘤患者，其中3例出现部分自发消退，并确定了不良预后特征。
