Moritani Suzuko, Ichihara Shu, Seki Yukio, Kataoka Masato, Yokoi Toyoharu
Department of Pathology and Clinical Laboratories, National Hospital Organization, Nagoya Medical Center, Nagoya, Japan.
Pathol Int. 2006 Jun;56(6):350-7. doi: 10.1111/j.1440-1827.2006.01971.x.
Pulmonary capillary hemangiomatosis is a rare vascular proliferative disease of unknown etiology. The common clinical features are slowly progressive and finally fatal pulmonary hypertension. The clinical diagnosis is usually difficult. Because most reported cases are of autopsy, little is known about its incipient lesion and natural history. Presented herein is a case of pulmonary capillary hemangiomatosis incidentally detected in a surgically resected lung for a metastatic colon cancer. The patient was a 60-year-old Japanese woman with a history of sigmoid colon cancer 3 years previously. The patient had undergone a right lower lobectomy for a metastatic tumor in the hilar region and a thoracoscopic tumorectomy of the peripheral area of the left upper lobe. Except for an episode of hemoptysis 2 weeks prior to the lung surgery, there were no other clinical symptoms characteristic of pulmonary capillary hemangiomatosis. The non-tumor area of right lower lobe showed multiple foci of capillary proliferation affecting alveolar walls, interlobular septa and pleura associated with patchy hemorrhage. There was a minor degree of vascular and bronchial involvement by capillary proliferation. It is suggested this particular case is an incidentally detected clinically incipient stage of pulmonary capillary hemangiomatosis. Passive congestion secondary to metastatic colon cancer in the hilar region may have contributed to the pathogenesis of this lesion.
肺毛细血管瘤病是一种病因不明的罕见血管增生性疾病。常见临床特征为缓慢进展并最终导致致命的肺动脉高压。临床诊断通常较为困难。由于大多数报道的病例是尸检病例，对其早期病变和自然病史了解甚少。本文报告一例在因转移性结肠癌接受手术切除的肺中偶然发现的肺毛细血管瘤病病例。患者为一名60岁的日本女性，3年前有乙状结肠癌病史。患者因肺门区转移瘤接受了右下叶切除术，并对左上叶周边区域进行了胸腔镜肿瘤切除术。除了肺手术前2周出现一次咯血外，没有其他肺毛细血管瘤病的临床特征性症状。右下叶非肿瘤区域显示出多个毛细血管增生灶，累及肺泡壁、小叶间隔和胸膜，并伴有片状出血。毛细血管增生对血管和支气管有轻度累及。提示该特殊病例是偶然发现的肺毛细血管瘤病临床早期阶段。肺门区转移性结肠癌继发的被动性充血可能促成了该病变的发病机制。
