From the Department of Radiology and Center for Imaging Science (M.J.C., K.S.L., H.S.H., T.J.K., T.S.K.), Department of Nuclear Medicine (B.T.K.), Department of Pathology (Y.H.K.), and Department of Thoracic Surgery (Y.M.S.), Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-Dong, Kangnam-Ku, Seoul 135-710, Korea.
Radiology. 2016 Aug;280(2):602-10. doi: 10.1148/radiol.2016151706. Epub 2016 Mar 4.
Purpose To describe clinical, computed tomographic (CT), and positron emission tomographic (PET) features, correlation of CT and pathologic results, and survival of patients with pulmonary intravascular lymphomatosis. Materials and Methods The institutional review board approved this retrospective study with waiver of patient consent. Forty-two patients with pulmonary intravascular lymphomatosis were identified, 11 (26%) of whom showed lung involvement. CT features were correlated with histopathologic results. Clinical and survival outcomes were compared between patients with and those without pulmonary involvement by adopting the χ(2), Student t, or Kaplan-Meier analysis with log-rank tests. Results At clinical presentation, all 11 patients showed B symptoms (systemic symptoms of fever, night sweats, and weight loss), 10 had respiratory and four had neurologic symptoms, and two had skin lesions. Patients received cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with (n = 5) or without (n = 6) rituximab, and seven (64%) patients died. Patients with lung involvement showed reduced overall and recurrence-free survival (median; 10.8 and 18.9 months, respectively) compared with those without lung involvement (median, 18.4 and 31.0 months, respectively) (P = .338 and .065, respectively). The most common CT abnormality was bilateral ground-glass opacity (GGO, n = 10), with increased fluorodeoxyglucose uptake at PET/CT (seven of seven patients). GGO correlated histopathologically with the expanded alveolar septal vasculatures and perivascular spaces filled with neoplastic lymphoid cells. Conclusion Pulmonary intravascular lymphomatosis appeared as bilateral GGO on CT images, with increased fluorodeoxyglucose uptake on PET/CT images. GGO on CT images correlated with the area of expanded alveolar septae because of distended vessels filled with neoplastic lymphoid cells. (©) RSNA, 2016 Online supplemental material is available for this article.
目的 描述肺血管内淋巴瘤的临床、计算机断层扫描（CT）和正电子发射断层扫描（PET）表现、CT 与病理结果的相关性以及患者的生存情况。
材料与方法 本回顾性研究经机构审查委员会批准，患者无需同意。共确定 42 例肺血管内淋巴瘤患者，其中 11 例（26%）有肺部受累。CT 特征与组织病理学结果相关。采用 χ(2)检验、Student t 检验或 Kaplan-Meier 分析和对数秩检验比较有和无肺部受累患者的临床和生存结局。
结果 在临床表现时，11 例患者均有 B 症状（发热、盗汗和体重减轻等全身症状），10 例有呼吸系统症状，4 例有神经系统症状，2 例有皮肤损伤。患者接受环磷酰胺、多柔比星、长春新碱和泼尼松化疗（n = 5）或联合（n = 6）利妥昔单抗，7 例（64%）患者死亡。有肺部受累的患者总生存和无复发生存时间均缩短（中位时间分别为 10.8 个月和 18.9 个月），而无肺部受累的患者分别为 18.4 个月和 31.0 个月（P =.338 和.065）。最常见的 CT 异常是双侧磨玻璃影（GGO，n = 10），7 例患者的 PET/CT 均有氟脱氧葡萄糖摄取增加。GGO 与组织病理学上扩大的肺泡间隔血管和充满肿瘤性淋巴样细胞的血管周围间隙相对应。
结论 CT 图像上肺血管内淋巴瘤表现为双侧 GGO，PET/CT 图像上氟脱氧葡萄糖摄取增加。CT 图像上的 GGO 与因充满肿瘤性淋巴样细胞而扩张的血管导致的扩大的肺泡间隔相对应。
